Ears Clicking? PM or TTS?

You need to be aware of Tensor Tympani Syndrome. It is when a small muscle in the ear has a myoclonus. Read this.


Middle Ear Myoclonus Terrence E. Zipfel a1Srinivas R. Kaza a1 and J. Scott Greene a1
a1 Department of Otolaryngology – Head and Neck Surgery, Penn State Geisinger Medical Centre, USA

Abstract

 

Tinnitus produced by repetitive contraction of the middle-ear muscles is a rare condition. We present an interesting case of bilateral middle-ear myoclonus causing incapacitating tinnitus in a patient with multiple sclerosis. Otological examination demonstrated rhythmic involuntary movement of the tympanic membrane. These movements correlated with a rhythmic ‘rushing wind’ noise perceived by the patient. Oropharyngeal examination showed no evidence of palatal myoclonus. Impedance audiometry confirmed rhythmic change in the middle-ear volume. Medical management was unsuccessful. The patient’s tinnitus was subsequently cured with bilateral sectioning of the tensor tympani and stapedial tendons.

Management of middle ear myoclonus.

Royal National Throat, Nose and Ear Hospital, London.

Tinnitus produced by synchronous repetitive contraction of the middle ear muscles (middle ear myoclonus) is a rare condition. We present six cases of middle ear myoclonus in whom different management regimes were successful. In two patients, the tinnitus was controlled by conservative measures. In one patient, whose tinnitus was associated with blepharospasm, significant improvement occurred following botulinum toxin injection into the ipsilateral orbicularis oculi. Three patients were cured by tympanotomy with stapedial and tensor tympani tendon section. The aetiology of this type of myoclonus remains unclear. The diagnosis is based on the history of involuntary and rhythmic clicking or buzzing tinnitus which is invariably unilateral. The primary differential diagnosis is palatal myoclonus whilst other local aural pathologies must be excluded by careful clinical assessment. Surgical section of these muscles via tympanotomy brings guaranteed relief when conservative measures fail.

One of the best articles I’ve found on the neurology of Palatal Myoclonus.

This article might explain why FCR works for PM. I’ll explain at the end.

Palatal myoclonus secondary to hypertrophic olivary degeneration

by Enrique Palacios, Ewa Wasilewska, Jorge E. Alvernia, Ramon E. Figuero.

A 64-year-old woman with a history of hypertension and stroke presented with palatal tremor and myoclonus 11 months after experiencing a vascular insult in the brainstem. The myoclonus was characterized by rhythmic involuntary movement of the soft palate, uvula, pharynx, and larynx. Magnetic resonance imaging (MRI) detected the presence of an old pontine vascular insult associated with a prominence and abnormal hyperintensity in the area of the right inferior olivary nucleus (figures 1-3). These findings were consistent with hypertrophic olivary degeneration. (1,2)

MRI is the most sensitive modality for the diagnosis of hypertrophic olivary degeneration because of its high diagnostic sensitivity in the area of the brainstem. On imaging, hypertrophic olivary degeneration occurs in three characteristic stages (3-5):

* In the first 4 to 6 months, no significant morphologic change in the olive or signal intensity is seen. This period corresponds with the initial phase of gliosis, demyelination, and vacuolization.

* From 6 months through 4 years, hypertrophy and an abnormal signal intensity of the olive are seen. These findings are related to neuronal and astrocytic hypertrophy (figures 1 and 2).

* After 4 years, the hypertrophy resolves, and abnormal signal intensity can be observed.

No evidence of contrast enhancement is seen during any of these stages.

The differential diagnosis of an abnormal localized signal intensity in the olive and pontomedullary area includes ischemic infarction, demyelination, tumor, an inflammatory process (including sarcoidosis), and focal rhomboencephalitis. A focal, hyperintense, nonenhancing lesion and enlargement of the olivary nucleus with a coexisting pontine lesion suggest an injury of the dentato-rubro-olivary pathway, findings that are consistent with hypertrophic olivary degeneration). (3-5)

[FIGURE 2 OMITTED]

Hypertrophic olivary degeneration is a form of trans-synaptic degeneration caused by an insult to the neuronal connections of the dentato-rubro-olivary pathway (i.e., the triangle of Guillain and Mollaret) by a primary brainstem inury. (1,2) Disruption of this neuronal pathway affects the reflex arc that controls fine voluntary movements, resulting in signs and symptoms such as palatal myoclonus and dentatorubral tremor. (2,3) This type of astrocytic degeneration has been reported in both children and adults; there is no predilection to either sex. (4)

At any given time following a primary brainstem injury, there may be focal enlargement rather than atrophy of the inferior olivary nucleus. This finding is characteristically identified on MRI. However, failure to properly identify the enlargement may result in a misdiagnosis (e.g, a tumor or multiple sclerosis). (1,5,6)

[FIGURE 3 OMITTED]

The Guillain-Mollaret triangle is composed of the dentate nucleus, the red nucleus, and the inferior olivary nucleus. The red nucleus and the ipsilateral inferior olivary nucleus are connected via the central tegmental tract, and the dentate nucleus connects to the contralateral red nucleus through the superior cerebellar peduncle. There are no direct connections between the inferior olivary nucleus and the contralateral dentate nucleus. (1-4) While hypertrophic olivary degeneration can occur with any focal lesion that involves the dentato-rubro-olivary pathway, it is typically associated with lesions that involve the superior cerebellar peduncle (dentatorubral tract), the dentate nucleus, or the central tegmental tract. (2,4)

There are three patterns of hypertrophic olivary degeneration as they relate to the location of the primary lesion. When the lesion is in the dentate nucleus or superior cerebellar peduncle, the olivary degeneration is contralateral. When the primary lesion is in the central tegmental tract, olivary degeneration is ipsilateral, as occurred in our patient. When the lesion involves both pathways, the olivary degeneration is bilatera. (1,6)

Hypertrophy of the olivary nucleus following a primary brainstem injury is believed to represent a combination of cell body enlargement, vacuolation of the cytoplasm, astrocytic proliferation, demyelination, and fibrillary gliosis. (2,6) The degree of the hypertrophic olivary nucleus is variable, depending on the time interval from the insult to the Guillain-Mollaret triangle. As seen on imaging, olivary hypertrophy develops 4 to 6 months after the primary insult, and it may resolve 10 to 16 months later. However, abnormal hyperintensity of the inferior olivary nucleus on T2-weighted MRI may continue for years despite the persistence of the signs and symptoms of palatal myoclonus and dentatorubral tremor. (2-5)

The clinical manifestations of hypertrophic olivary degeneration probably reflect a loss of inhibitory control as a result of disruption of the dentato-rubroolivary pathway. (1-3) The most common symptom is palatal myoclonus, which is characterized by rhythmic involuntary movement of the soft palate, uvula, pharynx, and larynx. (2,6) Other clinical signs and symptoms include dentatorubral tremor, severe myoclonus of the cervical muscles and diaphragm, and symptoms of cerebellar or brainstem dysfunction. (2) The central tegmental tract has several connections to the nucleus ambiguous, which gives rise to efferent motor fibers of the vagus nerve that innervates the muscles that control palatal movement. (2,5) Our patient exhibited the classic signs and symptoms of palatal myoclonus, which correlated with imaging findings involving the central tegmental tract.

Not all patients with hypertrophic olivary degeneration develop the classic symptoms, but virtually all patients who develop palatal myoclonus after a brainstem insult will develop hypertrophic olivary degeneration. (1,2) Hypertrophic olivary degeneration is a sequela of a brainstem injury of various etiologies, and its clinical presentation is variable. Identification of the characteristic MRI findings of the olivary nucleus indicating an associated lesion involving the dentatorubro-olivary pathway allows for correct diagnosis. Therefore, it is important to understand the potential clinical pattern that may lead to recognition of the MRI findings of hypertrophic olivary degeneration.

References

(1.) Salamon-Murayama N, Russell EJ, Rabin BM. Diagnosis please. Case 17: Hypertrophic olivary degeneration secondary to pontine hemorrhage. Radiology 1999;213(3):814-17.

(2.) Krings T, Foltys H, Meister IG, Reul J. Hypertrophic olivary degeneration following pontine haemorrhage: Hypertensive crisis or cavernous haemangioma bleeding? J Neurol Neurosurg Psychiatry 2003;74(6):797-9.

(3.) Hirono N, Kameyama M, Kohayashi Y, et al. MR demonstration of a unilateral olivary hypertrophy caused by pontine tegmental hematoma. Neuroradiology 1990;32(4):340-2.

(4.) Uchino A, Hasuo K, Uchida K, et al. Olivary degeneration after cerebellar or brain stem haemorrhage: MRI. Neuroradiology 1993;35(5):335-8.

(5.) Osborn AG, Blaser SI, Salzman KL. Toxic/metabolic/degenerative disorders, acquired. In: Osborn AG, Blaser SI, Salzman KL. Diagnostic Imaging: Brain. Salt Lake City: Amirsys; 2004.

(6.) Kitajima M, Korogi Y, Shimomura O, et al. Hypertrophic olivary degeneration: MR imaging and pathologic findings. Radiology 1994;192(2):539-43.

Enrique Palacios, MD, FACR; Ewa Wasilewska, MD; Jorge E. Alvernia, MD; Ramon E. Figueroa, MD, FACR

From the Department of Radiology (Dr. Palacios and Dr. Wasilewska) and the Department of Neurosurgery (Dr. Alvernia), Tulane University Hospital and Clinic, New Orleans; and the Department of Radiology, Medical College of Georgia, Augusta (Dr. Figueroa).

COPYRIGHT 2009 Vendome Group LLC
COPYRIGHT 2010 Gale, Cengage Learning
Comment:
In this article they mention the central tegmental tract has several connections to the nucleus ambiguous, which gives rise to efferent motor fibers of the vagus nerve that innervates the muscles that control palatal movement.

Read the following definition of the Nucleous Ambiguus.

The nucleus ambiguus (literally “ambiguous nucleus”) is a region of histologically disparate cells located just dorsal (posterior) to the inferior olivary nucleus in the lateral portion of the upper (rostralmedulla. It receives upper motor neuron innervation directly via the corticobulbar tract.

This nucleus gives rise to the branchial efferent motor fibers of the vagus nerve (CN X) terminating in the laryngealpharyngeal muscles, and musculus uvulae[citation needed]; as well as to the efferent motor fibers of theglossopharyngeal nerve (CN IX) terminating in the stylopharyngeus.

Now read this Definition of the corticobulbar tract.
The corticobulbar (or corticonucleartract is a white matter pathway connecting the cerebral cortex to the brainstem. The ‘bulb’ is an archaic term for the medulla oblongata; in modern clinical usage, it sometimes includes the pons as well. The word ‘bulbar’ therefore refers to the nerves and tracts connected to the medulla, and also by association to the muscles thus innervated, those of the tongue, pharynx and larynx.
Something to keep in mind is the innervation of the face and nasal passages is from the trigeminal nucleus. When I perform FCR I’m creating a stimulation through these very pathways! Your nerve cells need 3 things to be healthy. Oxygen, Glucose and activation. Activation is stimulation and if your brain isn’t stimulated it is programed to start dyeing off. To stabilize the area’s of your brain that cause PM you need to look at healthy area’s that can be activated that will support the unhealthy area’s. This is called augmenting neurology. Stabilizing these area’s of the brain as well as improving activation will be the answer to overcoming PM.
Your’s in Health,
John Lieurance, DC, ND

Clinical trial of piracetam in patients with myoclonus: nationwide multiinstitution study in Japan. The Myoclonus/Piracetam Study Group.

Clinical trial of piracetam in patients with myoclonus: nationwide multiinstitution study in Japan. The Myoclonus/Piracetam Study Group.

Ikeda A, Shibasaki H, Tashiro K, Mizuno Y, Kimura J.

Department of Brain Pathophysiology,
Kyoto University School of Medicine, Japan.
Mov Disord 1996 Nov;11(6):691-700

Abstract

Sixty patients with disabling myoclonus excluding mainly spinal myoclonus were treated by piracetam as an open-labeled study, and myoclonus score, neurological symptoms, functional disability, and intensity of myoclonus were scored before and after treatment, including a blinded video inspection.  Electrophysiological correlation also was investigated before and after treatment.  Piracetam was effective in myoclonus, especially that of cortical origin, in both monotherapy and polytherapy. Piracetam also had positive benefits on gait ataxia and convulsions but not on dysarthria, and feeding and hand writing improved much more significantly.  Psychologically significant improvement was seen in decreased motivation, sleep disturbance, attention deficit, and depression, all of which might be possibly secondary benefits associated with improvement of myoclonus.  There was no positive correlation between clinical and electrophysiological improvement.  Tolerance was good, and side effects were transient.  However, hematological abnormalities observed in at least two patients in the present study should be kept in mind when relatively large doses of piracetam are administered, especially in combination with other anti-myoclonic drugs.

Various Studies on Piracetam and Myoclonus

Progressive Myoclonic Epilepsies.

Uthman BM, Reichl A.

University of Florida College of Medicine,
Department of Neurology and Neurosciences,
Malcom Randall Veterans Affairs Medical Center,
Neurology Service (127),
1601 SW Archer Road, Gainesville, FL 32608   
Curr Treat Options Neurol
 2002 Jan;4(1):3-17

Abstract

The treatment of progressive myoclonus epilepsy (PME) remains a major therapeutic challenge in neurology. Generalized convulsive seizures are often well controlled through classic antiepileptic drugs (AEDs) like valproate and clonazepam, whereas myoclonus, the main symptom that is affecting patients most in their daily life, is usually refractory to standard AEDs. Alternative therapy concepts have been and still are investigated. Among the new drugs, zonisamide and piracetam have shown the most promising results as add-on treatments. Other therapeutic approaches, like the use of antioxidants, 5-hydroxy tryptophan (5-HTP), and baclofen should also be taken into consideration for the treatment of intractable cases of PME. Non pharmacologic treatment options such as diet and physical therapy should always be considered, because they may save costs and side effects. In some instances, the occasional use of alcohol has shown beneficial effects.

 

Long-term efficacy and safety of piracetam in the treatment of progressive myoclonus epilepsy. 

Fedi M, Reutens D, Dubeau F, Andermann E,
D’Agostino D, Andermann F. FRCP(C)

Montreal Neurological Institute and Hospital,
3801 University St, Room 127,
Montreal, Quebec, Canada H3A 2B4.
Arch Neurol 2001 May;58(5):781-6

Abstract

BACKGROUND: Piracetam has been proven to be effective and well tolerated in the treatment of myoclonus in short-term studies. 

OBJECTIVE: To assess its long-term clinical efficacy, 11 patients with disabling myoclonus due to progressive myoclonus epilepsy were treated with piracetam in an open-label study.

METHODS: Neurologic outcome (at the 1st, 6th, 12th, and 18th month of treatment) was assessed by an adjusted sum score of the following 3 indices: motor impairment, functional disability, and global assessment of disability due to myoclonus. Severity of other neurologic symptoms (seizure frequency and severity, dysarthria, and gait ataxia) also was assessed. Treatment with piracetam was initiated at a dose of 3.2 g/d that was gradually increased until stable benefit was noted (maximal dose in the trial was 20 g/d). Concomitant antiepileptic drugs were maintained at their previous dose.

RESULTS: Statistically significant improvement in the total rating score was observed after introduction of piracetam at the 1st, 6th, and 12th month of treatment. Severity of other neurologic symptom scores did not improve significantly. Two patients reported drowsiness during the first 2 weeks of treatment.

CONCLUSIONS: Piracetam given as add-on therapy seems to be an effective, sustained, and well-tolerated treatment of myoclonus. In patients with progressive myoclonus epilepsy, the efficacy of the drug increased during the first 12 months of treatment and then stabilized.

A pharmacological profile of piracetam (Myocalm), a drug for myoclonus 

Tajima K, Nanri M.

Taiho Pharmaceutical Co.,
Ltd., Tokyo, Japan.
Nippon Yakurigaku Zasshi 2000 Oct;116(4):209-14

Abstract

Myoclonus is defined as shock-like, brief involuntary abnormal movements in muscle jerking caused by external stimuli; and it arises from progressive myoclonus epilepsy, post-anoxic encephalopathy and Alzheimer’s disease, causing disabling symptoms.  It is a rare syndrome but very difficult to control. Piracetam (2-oxo-1-pyrrolidineacetamide, Myocalm) was developed more than 30 years ago as a cyclic derivative of gamma-amino butyric acid (GABA); it has been used in European countries for the treatment of memory loss and other cognitive defects in patients. Some reports have suggested that piracetam has anti-myoclonus activities, but the mechanisms of myoclonus are not well-identified, and thus there have been few preclinical studies on piracetam for the treatment of myoclonus. We investigated the effect ofpiracetam and clonazepam, an anti-epileptic drug, on high dosage urea-induced myoclonus using an electromyogram in rats. The incidence of myoclonus induced by urea 4.5 g! /k! g (i.p.) was significantly reduced by piracetam at 300 mg/kg (i.p.) and by clonazepam at 0.3 mg/kg (p.o.).  The co-administration of piracetam 100 mg/kg (i.p.) and clonazepam at 0.03-0.1 mg/kg (p.o.) significantly reduced the incidence of myoclonus, although separate administration was not effective.  After oral administration of piracetam, it is rapidly and completely absorbed and excreted almost unchanged in the urine; however, it does show a little binding to human serum protein.  Repeated oral administration of piracetam for 7 days in phase-I trials did not show any accumulation of the drug. In the placebo-controlled double-blind crossover trial ofpiracetam conducted in the UK, there was a significant improvement in cortical myoclonus. In phase-II trials,piracetam inhibited myoclonus and showed an improvement in the quality of life (QOL) of the patients. These results show that piracetam has a beneficial use in clinics for severe myoclonus patients when it is combined with anti-epileptic drugs, demonstrating an improvement in the myoclonus and QOL of patients.

Effectiveness of piracetam in cortical myoclonus.

Brown P, Steiger MJ, Thompson PD, Rothwell JC,
Day BL, Salama M, Waegemans T, Marsden CD.

MRC Human Movement and Balance Unit,
Institute of Neurology, London, England.
Mov Disord 1993;8(1):63-8

Abstract

Twenty-one patients with disabling spontaneous, reflex, or action myoclonus due to various causes, who had shown apparent clinical improvement on introduction of piracetam, entered a placebo-controlled double-blind crossover trial of piracetam (2.4-16.8 g daily). All but one patient had electrophysiological evidence of cortical myoclonus.  Patients were randomly allocated to a 14-day course of piracetam followed by identical placebo, or placebo followed by piracetam.  Nineteen patients received piracetam/placebo in addition to their routine antimyoclonic treatment (carbamazepine, clonazepam, phenytoin, primidone, sodium valproate, or tryptophan plus isocarboxazid, alone or in combination) and two received piracetam/placebo as monotherapy.  All patients were rated at the end of each treatment phase using stimulus sensitivity, motor, writing, functional disability, global assessment, and visual analogue scales. Ten of the 21 patients had to be rescued from the placebo phase of the trial because of a severe and intolerable exacerbation of their myoclonus.  No patients required rescue from the piracetam phase of the double-blind trial.  When the 21 patients were considered together, there was a significant improvement in motor, writing, functional disability, global assessment, and visual analogue scores during treatment with piracetam compared with placebo. The total rating score also improved significantly with piracetam, by a median of 22%.  Piracetam, usually in combination with other antimyoclonic drugs, is a useful treatment for myoclonus of cortical origin.

 

Beneficial effect of piracetam monotherapy on post-ischaemic palatal pmyoclonus

Karacostas D, Doskas T, Artemis N, Vadicolias K, Milonas I. B.

Department of Neurology,
Aristotelian University School of Medicine,
AHEPA Hospital, Thessaloniki, Greece.
J Int Med Res 1999 Jul-Aug;27(4):201-5

Abstract

A 70-year-old hypertensive woman suffered a subarachnoid haemorrhage followed by delayed vasospasm in the basal cerebral arteries. This resulted in multiple ischaemic lesions in the right middle cerebral artery region and contralateral post-ischaemic palatal myoclonus. In this setting, piracetamadministered in high doses (24-36 g/day), abolished the myoclonus observed in this patient. Although there is evidence from case reports and clinical trials of the therapeutic efficacy of piracetam in patients with skeletal myoclonus of various causes, to our knowledge this is the first report indicating the beneficial effect of piracetammonotherapy on post-ischaemic palatal myoclonus. 

 

 

Clinical trial of piracetam in patients with myoclonus: nationwide multiinstitution study in Japan. The Myoclonus/Piracetam Study Group.


Clinical trial of piracetam in patients with myoclonus: nationwide multiinstitution study in Japan. The Myoclonus/Piracetam Study Group.

Ikeda A, Shibasaki H, Tashiro K, Mizuno Y, Kimura J.

Department of Brain Pathophysiology,
Kyoto University School of Medicine, Japan.
Mov Disord 1996 Nov;11(6):691-700

Abstract

Sixty patients with disabling myoclonus excluding mainly spinal myoclonus were treated by piracetam as an open-labeled study, and myoclonus score, neurological symptoms, functional disability, and intensity of myoclonus were scored before and after treatment, including a blinded video inspection.  Electrophysiological correlation also was investigated before and after treatment.  Piracetam was effective in myoclonus, especially that of cortical origin, in both monotherapy and polytherapy. Piracetam also had positive benefits on gait ataxia and convulsions but not on dysarthria, and feeding and hand writing improved much more significantly.  Psychologically significant improvement was seen in decreased motivation, sleep disturbance, attention deficit, and depression, all of which might be possibly secondary benefits associated with improvement of myoclonus.  There was no positive correlation between clinical and electrophysiological improvement.  Tolerance was good, and side effects were transient.  However, hematological abnormalities observed in at least two patients in the present study should be kept in mind when relatively large doses of piracetam are administered, especially in combination with other anti-myoclonic drugs.

 I found this online. I haven’t tried this with any PM patients but I plan on doing so and will post results.

John Lieurance, DC, ND

Therapies for Palatal Myoclonus, treatment for palatal myoclonus

 

optokinetic

[op′tōkinet′ik]

Etymology: Gk, optikos, sight, kinesis, motion
pertaining to movement of the eyeballs in response to the movement of objects across the visual field, such as in optokinetic nystagmus. Also called opticokinetic.
Mosby’s Medical Dictionary, 8th edition. © 2009, Elsevier.

nystagmus [nis-tag´mus]

involuntary, rapid, rhythmic movement (horizontal, vertical, rotatory, or mixed, i.e., of two types) of the eyeball. adj., adj nystag´mic.
amaurotic nystagmus nystagmus in the blind or in those with defects of central vision.
amblyopic nystagmus nystagmus due to any lesion interfering with central vision.
aural nystagmus labyrinthine nystagmus.
caloric nystagmus rotatory nystagmus in response to caloric stimuli in the ear, seen during the caloric test.
Cheyne’s nystagmus a peculiar rhythmical eye movement resembling Cheyne-Stokes respiration in rhythm.
congenital nystagmus (congenital hereditary nystagmus) nystagmus usually present at birth, usually horizontal and pendular, but occasionally jerky and pendular; the nystagmus may be caused by or associated with optic atrophy, coloboma, albinism, bilateral macular lesions, congenital cataract, severe astigmatism, and glaucoma.
dissociated nystagmus that in which the movements in the two eyes are dissimilar.
end-position nystagmus that occurring only at extremes of gaze.
fixation nystagmus that occurring only on gazing fixedly at an object.
gaze nystagmus nystagmus made apparent by looking to the right or to the left.
labyrinthine nystagmus vestibular nystagmus due to labyrinthine disturbance.
latent nystagmus that occurring only when one eye is covered.
lateral nystagmus involuntary horizontal movement of the eyes.
optokinetic nystagmus nystagmus induced by looking at objects moving across the visual field.
pendular nystagmus nystagmus in which the oscillations of the eyes have an equal rate, amplitude, direction, and type of movement.
positional nystagmus that which occurs, or is altered in form or intensity, on assumption of certain positions of the head.
retraction nystagmus (nystagmus retracto´rius) a spasmodic backward movement of the eyeball occurring on attempts to move the eye; a sign of midbrain disease.
rotatory nystagmus involuntary rotation of the eyes about the visual axis.
secondary nystagmus nystagmus occurring after the abrupt cessation of rotation of the head, caused by the labyrinthine fluid continuing to move.
spontaneous nystagmus that occurring without specific stimulation of the vestibular system.
vertical nystagmus involuntary up-and-down movement of the eyes.
vestibular nystagmus nystagmus due to disturbance of the labyrinth or of the vestibular nuclei; the movements are usually jerky.
Comment:
Take a look at an eye phone application called OPTIK. Try and see if your myoclonus changes with the direction, speed and even whether the phone is in your periphery in various feilds.
I find many PM patients have an effect. Usually it will speed up the myoclonus.
John Lieurance, DC

Research on PM

Latest citations:

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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University of Munich, Grosshadern, Munich, Germany.
PURPOSE: The objective of this clinical study was to investigate the history and clinical findings in 10 patients having an essential palatal tremor. Furthermore, a botulinum toxin A (BTA) therapy in 5 cases was carried out, and the outcome was analyzed. MATERIALS AND METHODS: Seven adult and 3 pediatric patients with essential palatal tremor were examined at presentation, before and after start of treatment, and every 3 months or when symptoms recurred. Findings were documented by endoscopic video recordings, electromyography, tympanometry, and ear canal microphone recording. The BTA injections were performed in local or general anesthesia, under elecromyographic guidance. RESULTS: The BTA therapy in all 5 patients was successful. Surprisingly, 2 of these patients, aged 10 and 6 years, remained in remission for several years after a single successful injection. CONCLUSION: Botulinum toxin therapy is a safe and effective treatment of essential palatal tremor and seems to be especially useful in pediatric patients. The long lasting effect in children hints toward a pathophysiologic difference between pediatric and adult essential palatal tremor.
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        Victoria C. Chang, MD Neurological Institute, Columbia University Medical Center, 710 West 168th Street, 3rd Floor, New York, NY 10032, USA. vchang@neuro.columbia.edu.
Myoclonus is a hyperkinetic movement disorder characterized by quick, involuntary jerks. It encompasses a vast range of etiologies and widespread anatomic locations. Treatment frequently requires multiple agents and is often only partially beneficial. These patients pose a considerable challenge for the clinician, further complicated by the fact that many of the treatment choices lack evidence-based support. In the past few years, publications regarding therapy have been largely observational case reports or series. Although the literature on treatment of cortical myoclonus appears to be growing, evidence regarding myoclonus of noncortical origin is less well established. Investigation of more satisfactory treatments is needed, as this condition can be disturbing, debilitating, and sometimes harmful for patients. Continuing investigations are using various animal models (mostly of posthypoxic myoclonus), electrophysiologic studies, new imaging techniques such as diffusion tensor imaging, and genetic studies. Meanwhile, the clinical approach to diagnosing and classifying myoclonus remains largely unchanged. This review updates readers on current investigations and suggests guidelines for diagnosing and treating myoclonus.
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        Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.
We report the outcome of botulinum toxin injection for essential palatal myoclonus, given on two occasions over a period of one year, in an eight-year-old boy, the youngest patient treated with botulinum toxin to date. Though there was significant relief of ear clicks each time after the injection, he developed severe palatal palsy following the second injection, which persisted for a month. We suggest that appropriate caution needs to be exercised when repeating botulinum toxin injections for palatal myoclonus in children.(c) 2007 Movement Disorder Society.

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        Integrated Centre for Research and Treatment of Vertigo, Balance and Ocular Motor Disorders, Ludwig-Maximilians University Munich, Marchioninistr. 15, 81377 Munich, Germany.
Previous studies have used low-frequency tones to modulate distortion product otoacoustic emissions (DPOAEs). The cubic DPOAE (CDPOAE) is mostly chosen because amplitudes sufficient for modulation can be evoked with moderate sound pressure levels. Quadratic DPOAEs (QDPOAEs) however, are more sensitive to minute changes of the cochlear operating point (OP) and are better suited to assess changes of the cochlear OP. Here, we compare the properties of low-frequency (30 Hz, 80-120 dB SPL) modulated CDPOAE and QDPOAEs evoked with f(2) = 2 and 5 kHz in human subjects with normal hearing. The modulation depth was quantified with the modulation index (MI), a measure which considers both amplitude and phase. Modulated CDPOAEs evoked with f(2) = 2 kHz have amplitude maxima at the zero crossings and amplitude minima at the extremes of the biasing tone (BT) which correlate positively with the BT level. CDPOAEs evoked with f(2) = 5 kHz were recorded during biasing in exactly the same way as described before. At the highest BT levels used (120 dB SPL), very little modulation could be detected. Not only the depth, but also the shape of the QDPOAE modulation pattern is correlated with the BT level. At moderate BT levels (about 90-100 dB SPL) QDPOAEs evoked with f(2) = 5 kHz show one amplitude notch around the zero crossing of the positive going flank of the BT (a single modulation pattern). At and above a BT level of about 105 dB SPL, the pattern reverses and shows a double modulation pattern. At the highest BT level used (120 dB SPL), quadratic MIs exceed cubic MIs (2.0 ± 0.5 and 0.97 ± 0.06, respectively). Patterns of low-frequency modulated QDPOAEs in humans are similar to the modulation seen in animal studies and as predicted by mathematical models. Human low-frequency modulated QDPOAEs are ideally suited to estimate cochlear OP shifts because of their high sensitivity to the OP shift.
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        Department of Otorhinolaryngology Head and Neck Surgery, Interdisciplinary Centre for Vertigo and Balance Disorders (IFB-LMU), Grosshadern Medical Centre, University of Munich, Munich, Germany.
OBJECTIVES: The aim of this study was to assess whether gadolinium-based contrast agent influences short-term hearing function in patients with Ménière disease undergoing intratympanically enhanced inner-ear magnetic resonance imaging. DESIGN: This is a prospective cohort study. SETTING: This study was conducted a tertiary referral university hospital, ENT department. PARTICIPANTS: In this study, 21 adult patients with definite, unilateral Ménière disease were included. According to the criteria of the Committee on Hearing and Equilibrium, all patients were in stage 1 or 2 of the disease, with largely preserved hearing function. OUTCOMES: All patients underwent clinical and audiologic testing before and 24 hours after intratympanic application of gadolinium-based contrast agent. The effects of the contrast medium on the hearing function were assessed by analysis of frequency thresholds, pure-tone average from 500 Hz to 3 kHz, and speech audiometry. RESULTS: Pure-tone average and single-frequency thresholds in audiometry showed no statistically significant difference after the application of intratympanic gadolinium-based contrast agent. Furthermore, speech audiometry scores remained stable after the application of the contrast agent. CONCLUSIONS: This study did not demonstrate clinically significant short-term effects of intratympanic application of gadolinium-based contrast agent on hearing function in patients with Ménière disease in initial stages.
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        Department of Otorhinolaryngology Head and Neck Surgery, Grosshadern Medical Centre, University of Munich, Marchioninistr. 15, 81377, Munich, Germany, rguerkov@med.uni-muenchen.de.
Our objective is to determine whether the degree of endolymphatic hydrops as it is detected in vivo in patients with definite Meniere’s disease correlates with audiovestibular function. In this prospective study, 37 patients with definite Meniere’s disease according to AAO-HNS criteria were included. Intratympanic contrast enhanced temporal bone MRI was performed using a 3D FLAIR protocol. The degree of endolymphatic hydrops in the cochlea and the vestibulum was graded on a Likert scale (0-3). The degree of hydrops was then analyzed with respect to its correlation with audiometric hearing levels, electrocochleographic SP/AP ratios, interaural amplitude ratios of vestibular evoked myogenic potentials and degree of horizontal semicircular canal paresis on caloric irrigation. There was a significant correlation between the degree of hydrops on the one hand and the averaged hearing level at 0.25-1 and 0.5-3 kHz and the vestibular evoked myogenic potential interaural amplitude ratio on the other hand. A trend toward a correlation was noticed between the hydrops and the caloric response, no correlation was noticed between the hydrops and the SP/AP ratio. The degree of endolymphatic hydrops correlates with a progressive loss of auditory and sacculus function in patients with Meniere`s disease.
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PURPOSE: Botulinum neurotoxin A (BTA) is a promising therapeutic option in the treatment of idiopathic rhinitis (IR), a disease characterized by nasal obstruction and hydrous rhinorrhea. The conventional localization for the injection of BTA in IR is the nasal turbinates. In our own clinical experience, submucoperichondrial injection of BTA in the nasal septum is an alternative that is easy to perform for the therapist and also well tolerated by the patient. MATERIAL AND METHODS: Five patients received an injection of in total 80 mouse units Dysport (Ipsen Pharma, Ettlingen, Germany) in the nasal septum. The unpleasantness of the nasal injection of BTA was measured on a visual analogue scale. Over the course of 14 days, nasal symptoms (rhinorrhea, nasal obstruction, urge to sneeze, nasal pruritus), the number of facial tissues used daily, and possible complications were evaluated. RESULTS: The unpleasantness of the injection of BTA into the nasal septum after local anesthesia was rated low (visual analogue scale, 0.76 on average). A good subjective symptom control was achieved in 3 patients concerning rhinorrhea and in all patients concerning nasal obstruction. The number of facial tissues used daily as a parameter for rhinorrhea was on average 21.0 before the injection of BTA, decreased in 4 patients over the course of time, and was on average 5.8 after 14 days. No patient reported any adverse effects after the injection of BTA. CONCLUSIONS: This pilot study demonstrates that septal injection of BTA in patients with IR can achieve good symptom control and patient comfort and should be compared in further studies to the conventional turbinal injection technique.
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        Department of Otorhinolaryngology, Head and Neck Surgery, Ludwig Maximilian University, Marchioninistrasse 15, 81377, Munich, Germany. thomas.braun@med.uni-muenchen.de
The objective of the study was to evaluate patient benefit and health-related quality of life after use of botulinum neurotoxin (BoNT) A for various otorhinolaryngological, functional (non-cosmetic) indications. The design consisted of a survey study of a patient cohort (n = 40) treated with BoNT A for functional indications. Patients were asked to answer the Glasgow Benefit Inventory (GBI), a retrospective questionnaire well validated for measuring the effect of otorhinolaryngological interventions on the health-related quality of life. GBI scores can range from -100 (maximal adverse effect), through 0 (no effect), to 100 (maximal positive effect). A total of 29 patients (72.5%) returned a valid questionnaire. Mean total GBI scores for the particular indications were 1.2 (sialorrhea, n = 7), 22.6 (gustatory sweating, n = 8), 20.6 (palatal tremor, n = 5), 15.0 (postlaryngectomy voice disorders due to pharyngoesophageal spasm, n = 5), 38.9 (adductor spasmodic dysphonia, n = 2) and 27.8 (oromandibular dystonia, n = 2), showing a mean overall positive effect of BoNT A treatment on the health-related quality of life, respectively. A varying percentage of patients reported an increase in their health-related quality of life, indicated by positive total GBI scores: sialorrhea 28.6%, gustatory sweating 87.5%, palatal tremor 60%, postlaryngectomy voice disorders 60%, spasmodic dysphonia 100% and oromandibular dystonia 100%. Use of BoNT A can be considered an effective therapeutic option for all the indications investigated. However, the possibility of raising patients’ health-related quality of life with this kind of therapy varies significantly for different indications. Further studies are needed to analyze the patients who will benefit most from a treatment with BoNT A.
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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University, Munich, Germany. Eike.Krause@med.uni-muenchen.de
HASH(0x2b1f0a4e6040)
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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University of Munich, Grosshadern, Munich, Germany.
PURPOSE: The objective of this clinical study was to investigate the history and clinical findings in 10 patients having an essential palatal tremor. Furthermore, a botulinum toxin A (BTA) therapy in 5 cases was carried out, and the outcome was analyzed. MATERIALS AND METHODS: Seven adult and 3 pediatric patients with essential palatal tremor were examined at presentation, before and after start of treatment, and every 3 months or when symptoms recurred. Findings were documented by endoscopic video recordings, electromyography, tympanometry, and ear canal microphone recording. The BTA injections were performed in local or general anesthesia, under elecromyographic guidance. RESULTS: The BTA therapy in all 5 patients was successful. Surprisingly, 2 of these patients, aged 10 and 6 years, remained in remission for several years after a single successful injection. CONCLUSION: Botulinum toxin therapy is a safe and effective treatment of essential palatal tremor and seems to be especially useful in pediatric patients. The long lasting effect in children hints toward a pathophysiologic difference between pediatric and adult essential palatal tremor.
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Tardive dyskinesia syndromes in the head and neck region may appear as adverse effects of psychopharmacotherapy. They are caused by blockage of central dopamine receptors. Oftentimes, these disorders persist even after discontinuation of the antipsychotic medication, and they are disabling the patients functionally and psychosocially. Medical therapeutic efforts with different psychopharmaceuticals, benzodiazepines or vitamin-E-preparations are frequently unsuccessful. Local application of botulinum neurotoxin A offers a new treatment modality, which can target the overshooting dysfunction directly at the peripheral muscle.
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        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University Munich, Munich, Germany. eike.krause@med.uni-muenchen.de
PURPOSE: Laryngectomized patients with pharyngoesophageal spasm frequently have poor voice quality and dysphagia. Local botulinum toxin A (BTA) injection can relieve muscular hypertonicity and improve symptoms. This procedure should also prolong the functional life span of the tracheoesophageal voice prosthesis. MATERIALS AND METHODS: This study evaluates 33 BTA treatments in 11 laryngectomees. All patients were having poor voice quality; 6 patients had additional dysphagia. In 10 patients, the BTA injection has been carried out during rigid pharyngoscopy under general anesthesia. One patient was treated in local anesthesia. RESULTS: A subjective improvement of voice quality was reported in 94%. This lasted on average for 20 weeks. The swallowing function improved moderately. For the first time, the functional life span of voice prostheses was examined. After treatment of pharyngoesophageal spasm, their durability was almost tripled. The BTA therapy has a significant effect. CONCLUSIONS: The BTA treatment improves voice quality and prolongs functional durability of voice prostheses in laryngectomees with pharyngoesophageal spasm. The success of treatment is of limited duration but can be repeated in the long-term.
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        Department of Neurology, Ludwig-Maximilians University, Klinikum Grosshadern, Marchioninistrasse 15, D-81366 Munich, Germany. judith.wagner@med.uni-muenchen.de

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        Department of Pediatrics, Section of Pediatric Otolaryngology, Inova Fairfax Hospital for Children, Falls Church, VA, USA. Rhs738@aol.com
An 8-year-old boy was seen by his primary care pediatrician with a chief complaint of “intermittent rapid vibrations of the epiglottis” that began several weeks prior. Intraoral examination revealed rapid, symmetrical bilateral contractions of the soft palate muscles (velum), accompanied by clicking sounds audible to physician (objective tinnitus) and patient. The patient was able to volitionally control the initiation and cessation of the palatal movements. The child’s mother stated that there had been no clicking noises heard while the boy was sound asleep. Palatal “clonus” was tentatively diagnosed as the cause of the problem. A normal magnetic resonance imaging study with contrast enhancement confirmed that there was no anatomical basis for the localized movement disorder.Palatal myoclonus is an uncommon localized intraoral movement disorder. There are 2 distinct types, and our patient was diagnosed with the essential palatal myoclonus type. This type is characteristically associated with clicking tinnitus, heard by the affected person as well as those in close proximity. The clicking noise is not continuous, ceases during sleep, and is not lifelong.
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        Servicio Otorrinolaringología, Hospital Clínico Universitario, Valencia, España.
Objective tinnitus can have many different etiologies, palatal myoclonus being one of the less frequent. This type of tinnitus is generated by involuntary rhythmic contraction of the soft palate, which generates an audible click for the patient and for the explorer. Botulinum toxin achieves temporary muscle paralysis through presynaptic inhibition of the acetylcholine level at the neuromuscular union. We present a patient with long-term objective tinnitus, along with this patient’s response to botulinum toxin injection.
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        Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London, UK.
Myoclonus is a sudden, brief, involuntary muscle jerk. It is caused by abrupt muscle contraction, in the case of positive myoclonus, or by sudden cessation of ongoing muscular activity, in the case of negative myoclonus (NM). Myoclonus may be classified in a number of ways, although classification based on the underlying physiology is the most useful from the therapeutic viewpoint. Given the large number of possible causes of myoclonus, it is essential to take a good history, to clinically characterize myoclonus and to look for additional findings on examination in order to limit the list of possible investigations. With regards to the history, the age of onset, the character of myoclonus, precipitating or alleviating factors, family history and associated symptoms and signs are important. On examination, it is important to see whether the myoclonus appears at rest, on keeping posture or during action, to note the distribution of jerks and to look for the stimulus sensitivity. Electrophysiological tests are very helpful in determining whether myoclonus is cortical, subcortical or spinal. A single pharmacological agent rarely control myoclonus and therefore polytherapy with a combination of drugs, often in large dosages, is usually needed. Generally, antiepileptic drugs such as valproate, levetiracetam and piracetam are effective in cortical myoclonus, but less effective in other forms of myoclonus. Clonazepam may be helpful with all types of myoclonus. Focal and segmental myoclonus, irrespective of its origin, may be treated with botulinum toxin injections, with variable success.
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        Department of Colorectal Surgery, Cleveland Clinic Florida, Weston, Florida.
Paradoxical puborectalis contraction and increased perineal descent are two forms of functional constipation presenting as challenging diagnostic and treatment dilemmas to the clinician. In the evaluation of these disorders, the clinician should take special care to exclude anatomic disorders leading to constipation. Physical examination is supplemented by additional diagnostic modalities such as cinedefecography, electromyography, manometry, and pudendal nerve tefninal motor latency. Generally, these investigations should be used in combination with the two playing the more relied upon techniques. Treatment is typically conservative with biofeedback playing a principal role with favorable results when patient compliance is emphasized. When considering paradoxical puborectalis contraction, failure of biofeedback is usually augmented with botulinum toxin injection. Increased perineal descent is generally treated with biofeedback and perineal support maneuvers. Surgery has little or no role in these conditions. The patient who insists on surgical intervention for either of these two conditions should be offered a stoma.
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        Fondation Adolphe de Rothschild, Unité d’Otologie-Otoneurologie, Paris, France.
Palatal tremor is a rare neurotological disorder responsible for objective tinnitus in children. Palatal tremor may be symptomatic of an underlying neurological disease or essential when a cause cannot be identified. We report a case of an essential palatal tremor in a 10-year-old girl complaining of clicking tinnitus. No treatment was undergone as she was not obviously bothered by the ear-clicking sound. Different treatment modalities have been used for distressing tinnitus related to palatal myoclonus. Recently several publications reported satisfactory results with botulinum toxin injection, which seems to be the treatment of choice.
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        Department of Thoracic Surgery, Glenfield Hospital, University Hospitals of Leicester, Leicester, United Kingdom.
Myoclonus as a sequel to thoracotomy has been reported, and its treatment can be challenging to both the patient and the surgeon. We describe a 43-year-old patient with chest wall pain and latissimus dorsi muscle contractions (myoclonus) after video-assisted thoracoscopic lung volume reduction. His symptoms remained refractory to benzodiazepines, nerve blockage, and botulinum toxin injection due to either poor compliance or lack of response to therapy. These symptoms started to resolve spontaneously 18 months after the procedure.
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        Assistant Professor of General Surgery, Department of Surgery, Catholic School of Medicine, University Hospital ‘Agostino Gemelli’, Largo Agostino Gemelli 8, 00168 Rome, Italy. gbrisin@tin.it , Research Fellow of General Surgery, Department of Surgery,’Tor Vergata’ University Hospital, Viale Oxford 81, 00133 Rome, Italy. fede.cadeddu@libero.it , Resident in General Surgery, Department of Surgery, Catholic School of Medicine, University Hospital ‘Agostino Gemelli’, Largo Agostino Gemelli 8, 00168 Rome, Italy. pacomaz81@yahoo.it , Assistant Professor of General Surgery, Department of Surgery, Catholic School of Medicine, University Hospital ‘Agostino Gemelli’, Largo Agostino Gemelli 8, 00168 Rome, Italy. giorgio.maria@rm.unicatt.it.
Since its introduction for the treatment of strabismus, botulinum toxin (BoNT) has been increasingly used in the treatment of several disorders with excessive or inappropriate muscle contractions. The therapeutic effects of BoNT occur through the temporary chemodenervation caused by the injection into the local target muscle or skin. Modulation of muscle relaxation may be achieved by varying the dose of BoNT solution injected; most adverse effects are transient. Indeed, botulinum neurotoxin has been used to selectively weaken the internal anal sphincter as a treatment for chronic anal fissure in several randomized, controlled trials and open-label studies. The use of botulinum neurotoxin seems to be an effective and safe approach for the treatment of chronic anal fissure, particularly in patients at high risk for incontinence.
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        Service d’ORL Pédiatrique, CHU Timone, Marseille, France.
Palatal myoclonus is an uncommon, rhythmic,”shock-like” involuntary movement of the muscles of the soft palate, throat, and other structures derived from the branchial arcs. Objective tinnitus is frequently neglected in review articles about childhood tinnitus. Our aim was to present the case of a 7-year-old girl with bilateral objective tinnitus due to palatal myoclonus without hearing impairment (normal hearing thresholds between 250 Hz and 8 kHz) but with otherwise normal hearing thresholds (250 Hz-8 kHz) and no evidence of intracerebral or systemic disorders. No treatment was useful.
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We report a 30-year-old man with moving ear syndrome caused by focal myoclonic jerks of the right temporal muscle. This focal myoclonus would disappear while the patient was sleeping, swallowing, or speaking. He was treated with botulinum toxin type A with a favorable outcome. Previous reports of this condition and possible therapeutic approaches are discussed.(c) 2007 Movement Disorder Society.
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        Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.

trihexyphenidyl palatal myoclonus

  1. The drugs used for treatment of palatal myoclonus are clonazepam, sodium valproate, tetrabenazine, haloperidol, trihexyphenidyl HCL and carbamazepine. Other Pharmacologic Agents: Trihexyphenidyl. Types of myoclonus in which trihexyphenidyl may be useful. Palatal; Mechanism of action. Acetylcholine-receptor antagonist, exerts a direct . Two patients are reported with palatal myoclonus, progressive ataxia, and dysarthria, unresponsive to treatment with trihexyphenidyl or L-5-hydroxytryptophan. Palatal myoclonus is an uncommon, rhythmic, . clonazepam, carbamazepine, valproic acid, antiarrhythmics, and trihexyphenidyl. W.: Movement Disorders, 1987; trihexyphenidyl palatal myoclonus 2(2): 93-98; Effectiveness of Trihexyphenidyl Against Pendular Nystagmus and Palatal Myoclonus: Evidence of Cholinergic Effectiveness of trihexyphenidyl against pendular nystagmus and palatal myoclonus: Evidence of cholinergic dysfunction. Movement Disorders 2(2): 98-98, 1987. Anticholinergic drugs, such as benztropine or trihexyphenidyl, may be useful. . Palatal myoclonus secondary to hypertrophic olivary degeneration.(IMAGING. Effectiveness of trihexyphenidyl against pendular nystagmus and palatal myoclonus: evidence of cholinergic dysfunction. Mov Disord 1987; 2: 93 – 98. . Scherokman B., Gunderson C.H., McBurney J.W., McClintock W. (1987) Effectiveness of trihexyphenidyl against pendular nystagmus and palatal myoclonus . [Palato-pharyngo-laryngeal myoclonus]. [Article in Japanese] . is palate-pharyngo-laryngeal type or only palatal type. . Clonazepam, trihexyphenidyl, carbamazepine, 5HTP and caeruletin . “Effectiveness of trihexyphenidyl against pendular nystagmus and palatal myoclonus: evidence of cholinergic

    trihexyphenidyl palatal myoclonus

    dysfunction.” Mov Disord 2(2): 93-8. vodopa, trihexyphenidyl, valproic acid, propranolol without effect. A mild improvement of . tients who develop palatal myoclonus after a brain insult will have HOD, not all patients . trihexyphenidyl palatal myoclonus The treatment of choice for essential palatal myoclonus is the administration of medications, including clonazepam, sodium valproate, tetrabenazine, haloperidol, trihexyphenidyl . Pharmacotherapy was attempted with clonazepan, levodopa, trihexyphenidyl, valproic . Although virtually all patients who develop palatal myoclonus after a brain insult will . Herishanu Y, Louzoun Z. Trihexyphenidyl treatment of vertical pendular nystagmus. . in degenerative disorders of the nervous system, in persons with palatal myoclonus, in .

Myoclonus Fact Sheet from NINDS Publication

What is myoclonus?

Myoclonus describes a symptom and not a diagnosis of a disease. It refers to sudden, involuntary jerking of a muscle or group of muscles. Myoclonic twitches or jerks usually are caused by sudden muscle contractions, called positive myoclonus, or by muscle relaxation, called negative myoclonus. Myoclonic jerks may occur alone or in sequence, in a pattern or without pattern. They may occur infrequently or many times each minute. Myoclonus sometimes occurs in response to an external event or when a person attempts to make a movement. The twitching cannot be controlled by the person experiencing it.

In its simplest form, myoclonus consists of a muscle twitch followed by relaxation. A hiccup is an example of this type of myoclonus. Other familiar examples of myoclonus are the jerks or “sleep starts” that some people experience while drifting off to sleep. These simple forms of myoclonus occur in normal, healthy persons and cause no difficulties. When more widespread, myoclonus may involve persistent, shock-like contractions in a group of muscles. In some cases, myoclonus begins in one region of the body and spreads to muscles in other areas. More severe cases of myoclonus can distort movement and severely limit a person’s ability to eat, talk, or walk. These types of myoclonus may indicate an underlying disorder in the brain or nerves.

What are the causes of myoclonus?

Myoclonus may develop in response to infection, head or spinal cord injury, stroke, brain tumors, kidney or liver failure, lipid storage disease, chemical or drug poisoning, or other disorders. Prolonged oxygen deprivation to the brain, called hypoxia, may result in posthypoxic myoclonus. Myoclonus can occur by itself, but most often it is one of several symptoms associated with a wide variety of nervous system disorders. For example, myoclonic jerking may develop in patients with multiple sclerosis, Parkinson’s disease, Alzheimer’s disease, or Creutzfeldt-Jakob disease. Myoclonic jerks commonly occur in persons with epilepsy, a disorder in which the electrical activity in the brain becomes disordered leading to seizures.

What are the types of myoclonus?

Classifying the many different forms of myoclonus is difficult because the causes, effects, and responses to therapy vary widely. Listed below are the types most commonly described.

  • Action myoclonusis characterized by muscular jerking triggered or intensified by voluntary movement or even the intention to move. It may be made worse by attempts at precise, coordinated movements. Action myoclonus is the most disabling form of myoclonus and can affect the arms, legs, face, and even the voice. This type of myoclonus often is caused by brain damage that results from a lack of oxygen and blood flow to the brain when breathing or heartbeat is temporarily stopped.
  • Cortical reflex myoclonusis thought to be a type of epilepsy that originates in the cerebral cortex – the outer layer, or “gray matter,” of the brain, responsible for much of the information processing that takes place in the brain. In this type of myoclonus, jerks usually involve only a few muscles in one part of the body, but jerks involving many muscles also may occur. Cortical reflex myoclonus can be intensified when individuals attempt to move in a certain way (action myoclonus) or perceive a particular sensation.
  • Essential myoclonusoccurs in the absence of epilepsy or other apparent abnormalities in the brain or nerves. It can occur randomly in people with no family history, but it also can appear among members of the same family, indicating that it sometimes may be an inherited disorder. Essential myoclonus tends to be stable without increasing in severity over time. In some families, there is an association of essential myoclonus, essential tremor, and even a form of dystonia, called myoclonus dystonia.  Another form of essential myoclonus may be a type of epilepsy with no known cause.
  • Palatal myoclonusis a regular, rhythmic contraction of one or both sides of the rear of the roof of the mouth, called the soft palate. These contractions may be accompanied by myoclonus in other muscles, including those in the face, tongue, throat, and diaphragm. The contractions are very rapid, occurring as often as 150 times a minute, and may persist during sleep. The condition usually appears in adults and can last indefinitely. Some people with palatal myoclonus regard it as a minor problem, although some occasionally complain of a “clicking” sound in the ear, a noise made as the muscles in the soft palate contract.  The disorder can cause discomfort and severe pain in some individuals.
  • Progressive myoclonus epilepsy (PME) is a group of diseases characterized by myoclonus, epileptic seizures, and other serious symptoms such as trouble walking or speaking. These rare disorders often get worse over time and sometimes are fatal. Studies have identified many forms of PME. Lafora body disease is inherited as an autosomal recessive disorder, meaning that the disease occurs only when a child inherits two copies of a defective gene, one from each parent. Lafora body disease is characterized by myoclonus, epileptic seizures, and dementia (progressive loss of memory and other intellectual functions). A second group of PME diseases belonging to the class of cerebral storage diseases usually involves myoclonus, visual problems, dementia, and dystonia (sustained muscle contractions that cause twisting movements or abnormal postures). Another group of PME disorders in the class of system degenerationsoften is accompanied by action myoclonus, seizures, and problems with balance and walking. Many of these PME diseases begin in childhood or adolescence.
  • Reticular reflex myoclonusis thought to be a type of generalized epilepsy that originates in the brain stem, the part of the brain that connects to the spinal cord and controls vital functions such as breathing and heartbeat. Myoclonic jerks usually affect the whole body, with muscles on both sides of the body affected simultaneously. In some people, myoclonic jerks occur in only a part of the body, such as the legs, with all the muscles in that part being involved in each jerk. Reticular reflex myoclonus can be triggered by either a voluntary movement or an external stimulus.
  • Stimulus-sensitive myoclonusis triggered by a variety of external events, including noise, movement, and light. Surprise may increase the sensitivity of the individual.
  • Sleep myoclonus occurs during the initial phases of sleep, especially at the moment of dropping off to sleep. Some forms appear to be stimulus-sensitive. Some persons with sleep myoclonus are rarely troubled by, or need treatment for, the condition. However, myoclonus may be a symptom in more complex and disturbing sleep disorders, such as restless legs syndrome, and may require treatment by a doctor.

What do scientists know about myoclonus?

Although rare cases of myoclonus are caused by an injury to the peripheral nerves (defined as the nerves outside the brain and spinal cord, or the central nervous system), most myoclonus is caused by a disturbance of the central nervous system. Studies suggest that several locations in the brain are involved in myoclonus. One such location, for example, is in the brain stem close to structures that are responsible for the startle response, an automatic reaction to an unexpected stimulus involving rapid muscle contraction.

The specific mechanisms underlying myoclonus are not yet fully understood. Scientists believe that some types of stimulus-sensitive myoclonus may involve overexcitability of the parts of the brain that control movement. These parts are interconnected in a series of feedback loops called motor pathways. These pathways facilitate and modulate communication between the brain and muscles. Key elements of this communication are chemicals known as neurotransmitters, which carry messages from one nerve cell, or neuron, to another. Neurotransmitters are released by neurons and attach themselves to receptors on parts of neighboring cells. Some neurotransmitters may make the receiving cell more sensitive, while others tend to make the receiving cell less sensitive. Laboratory studies suggest that an imbalance between these chemicals may underlie myoclonus.

Some researchers speculate that abnormalities or deficiencies in the receptors for certain neurotransmitters may contribute to some forms of myoclonus. Receptors that appear to be related to myoclonus include those for two important inhibitory neurotransmitters: serotonin and gamma-aminobutyric acid (GABA). Other receptors with links to myoclonus include those for opiates and glycine, the latter an inhibitory neurotransmitter that is important for the control of motor and sensory functions in the spinal cord. More research is needed to determine how these receptor abnormalities cause or contribute to myoclonus.

How is myoclonus treated?

Treatment of myoclonus focuses on medications that may help reduce symptoms. The drug of first choice to treat myoclonus, especially certain types of action myoclonus, is clonazepam, a type of tranquilizer. Dosages of clonazepam usually are increased gradually until the individual improves or side effects become harmful. Drowsiness and loss of coordination are common side effects. The beneficial effects of clonazepam may diminish over time if the individual develops a tolerance for the drug.

Many of the drugs used for myoclonus, such as barbiturates, levetiracetam,phenytoin, and primidone, are also used to treat epilepsy. Barbiturates slow down the central nervous system and cause tranquilizing or antiseizure effects. Phenytoin, levetiracetam, and primidone are effective antiepileptic drugs, although phenytoin can cause liver failure or have other harmful long-term effects in individuals with PME. Sodium valproate is an alternative therapy for myoclonus and can be used either alone or in combination with clonazepam. Although clonazepam and/or sodium valproate are effective in the majority of people with myoclonus, some people have adverse reactions to these drugs.

Some studies have shown that doses of 5-hydroxytryptophan (5-HTP), a building block of serotonin, leads to improvement in people with some types of action myoclonus and PME. However, other studies indicate that 5-HTP therapy is not effective in all people with myoclonus, and, in fact, may worsen the condition in some individuals. These differences in the effect of 5-HTP on individuals with myoclonus have not yet been explained, but they may offer important clues to underlying abnormalities in serotonin receptors.

The complex origins of myoclonus may require the use of multiple drugs for effective treatment. Although some drugs have a limited effect when used individually, they may have a greater effect when used with drugs that act on different pathways or mechanisms in the brain. By combining several of these drugs, scientists hope to achieve greater control of myoclonic symptoms. Some drugs currently being studied in different combinations include clonazepam, sodium valproate, levetiracetam, and primidone. Hormonal therapy also may improve responses to antimyoclonic drugs in some people.

What research is being done?

Within the Federal government, the National Institute of Neurological Disorders and Stroke (NINDS), a component of the National Institutes of Health (NIH), has primary responsibility for research on the brain and nervous system. As part of its mission, the NINDS supports research on myoclonus at its laboratories in Bethesda, Maryland, and through grants to universities and major medical institutions across the country.

Scientists are seeking to understand the underlying biochemical basis of involuntary movements and to find the most effective treatment for myoclonus and other movement disorders.

Investigators are evaluating the role of neurotransmitters and receptors in myoclonus. If abnormalities in neurotransmitters or receptors are found to play a causative role in myoclonus, future research can focus on determining the extent to which genetic alterations are responsible for these abnormalities and on identifying the nature of those alterations. Scientists also may be able to develop drug treatments that target specific changes in the receptors to reverse abnormalities, such as the loss of inhibition, and to enhance mechanisms that compensate for these abnormalities. Identifying receptor abnormalities also may help researchers develop diagnostic tests for myoclonus. NINDS-supported scientists at research institutions throughout the country are studying various aspects of PME, including the basic mechanisms and genes involved in this group of diseases.