I had a felow travel to Sarasota this week for FCR. I have been working hard on the neurology for PM and feel I am making progress in further perfecting the treatment series. I have some demonstrations in this video of some of the functional neurology I am using with these cases. Each patient is very different and it is unpredictable what therapies will work for each case. A detailed neurological exam in needed to determine how the myoclonus can be augmented. The FCR is performed to take the pressure off the cranium and to allow better oxygen in the central nervous system.

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

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.

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.

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. 

 

 

Latest citations:

Am J Otolaryngol. 2009 Mar 26;:   20015723

Difference in outcome of botulinum toxin treatment of essential palatal tremor in children and adults.

[My paper]Eike Krause, Florian Heinen, Robert Gürkov

        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.

Curr Treat Options Neurol. 2008 May ;10 (3):222-9  18579026 Cit:1

Myoclonus.

[My paper]Victoria C Chang, Steven J Frucht

        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.

Mov Disord. 2007 May 21;:   17516476

Efficacy and complication of botulinum toxin injection in palatal myoclonus: Experience from a patient.

[My paper]Pramod Kumar Pal, Ponnathpur Satish Lakshmi, Muninarayanappa Nirmala

        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.

Other papers by authors:

Hear Res. 2012 Mar 23;:   22465462

Low-frequency modulated quadratic and cubic distortion product otoacoustic emissions in humans.

[My paper]Markus Drexl, Robert Gürkov, Eike Krause

        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.

Am J Otolaryngol. 2012 Feb 6;:   22317837

Short-term audiologic effect of intratympanic gadolinium contrast agent application in patients with Ménière disease.

[My paper]Julia Palo Rodrigues Louza, Wilhelm Flatz, Eike Krause, Robert Gürkov

        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.

Eur Arch Otorhinolaryngol. 2011 Mar 23;:   21431434

In vivo visualization of endolyphatic hydrops in patients with Meniere’s disease: correlation with audiovestibular function.

[My paper]Robert Gürkov, Wilhem Flatz, Julia Louza, Michael Strupp, Eike Krause

        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.

Am J Otolaryngol. 2011 Mar 16;:   21419514

Septal injection of botulinum neurotoxin A for idiopathic rhinitis: a pilot study.

[My paper]Thomas Braun, Robert Gürkov, Matthias F Kramer, Eike Krause

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.

Eur Arch Otorhinolaryngol. 2010 Dec ;267 (12):1963-7  20563590

Patient benefit from treatment with botulinum neurotoxin A for functional indications in otorhinolaryngology.

[My paper]Thomas Braun, Robert Gürkov, John Martin Hempel, Alexander Berghaus, Eike Krause

        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.

Otolaryngol Head Neck Surg. 2010 Jun ;142 (6):809-13  20493350

Influence of cochlear implantation on peripheral vestibular receptor function.

[My paper]Eike Krause, Julia P R Louza, Juliane Wechtenbruch, Robert Gürkov

        Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Ludwig-Maximilians-University, Munich, Germany. Eike.Krause@med.uni-muenchen.de

HASH(0x2b1f0a4e6040)

Am J Otolaryngol. 2009 Mar 26;:   20015723

Difference in outcome of botulinum toxin treatment of essential palatal tremor in children and adults.

[My paper]Eike Krause, Florian Heinen, Robert Gürkov

        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.

Laryngorhinootologie. 2009 Aug ;88 (8):508-10  19885971

[Local botulinum neurotoxin A therapy in tardive lingual dyskinesia]

[My paper]Eike Krause, Robert Gürkov

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.

Am J Otolaryngol. ;30 (6):371-5  19880024

Botulinum toxin A prolongs functional durability of voice prostheses in laryngectomees with pharyngoesophageal spasm.

[My paper]Eike Krause, John-Martin Hempel, Robert Gürkov

        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.

Arch Neurol. 2009 Aug ;66 (8):1038-9  19667230

Cerebrospinal fluid rhinorrhea with spontaneous sphenoid sinus fistula.

[My paper]Judith Wagner, Christoph Schankin, Thomas Klopstock, Klaus Seelos, Andreas Leunig, Andreas Straube

        Department of Neurology, Ludwig-Maximilians University, Klinikum Grosshadern, Marchioninistrasse 15, D-81366 Munich, Germany. judith.wagner@med.uni-muenchen.de

Latest similar papers:

Pediatr Emerg Care. 2012 Feb ;28 (2):158-9  22307183

Loud clicking sounds associated with rapid soft palate muscle contractions.

[My paper]Richard H Schwartz, Robert S Bahadori, John S Myseros

        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.

Acta Otorrinolaringol Esp. 2011 Apr 20;:   21513907

Objective tinnitus from palatal myoclonus. Use of botulinum toxin: a case report.

[My paper]Noemi Conill Tobías, Carlos de Paula Vernetta, Francisco Javier García Callejo, Jaime Marco Algarra

        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.

Ther Adv Neurol Disord. 2011 Jan ;4 (1):47-62  21339907

Myoclonic disorders: a practical approach for diagnosis and treatment.

[My paper]Maja Kojovic, Carla Cordivari, Kailash Bhatia

        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.

Clin Colon Rectal Surg. 2008 May ;21 (2):138-45  20011410

Paradoxical puborectalis contraction and increased perineal descent.

[My paper]Ron G Landmann, Steven D Wexner

        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.

Rev Laryngol Otol Rhinol (Bord). 2009 ;130 (2):117-9  19813474

[Objective tinnitus and essential palatal tremor in children: report of a case]

[My paper]M Daval, M Cohen, I Mari, D Ayache

        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.

Ann Thorac Surg. 2009 Oct ;88 (4):1360-2  19766848

Latissimus dorsi myoclonus after video assisted thoracoscopic lung volume reduction.

[My paper]Muhammad I Aslam, Inger F Oey, David A Waller

        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.

Expert Rev Gastroenterol Hepatol. 2007 Dec ;1 (2):219-228  19072412 Cit:2

Botulinum toxin A for the treatment of chronic anal fissure.

[My paper]Giuseppe Brisinda, Federica Cadeddu, Pasquale Mazzeo, Giorgio Maria

        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.

Int Tinnitus J. 2007 ;13 (2):157-8  18229797 Cit:2

Objective tinnitus associated with essential palatal myoclonus: report in a child.

[My paper]Maya Elziere, Stéphane Roman, Richard Nicollas, Jean-Michel Triglia

        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.

Mov Disord. 2007 Oct 25;:   17960817

Moving ear syndrome: The role of botulinum toxin.

[My paper]Clecio Godeiro-Junior, Andre Carvalho Felicio, Evandro Penteado Villar Felix, Gilberto Mastrocola Manzano, Sonia Maria de Azevedo Silva, Vanderci Borges, Henrique Ballalai Ferraz, Orlando G P Barsottini

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.

Mov Disord. 2007 May 21;:   17516476

Efficacy and complication of botulinum toxin injection in palatal myoclonus: Experience from a patient.

[My paper]Pramod Kumar Pal, Ponnathpur Satish Lakshmi, Muninarayanappa Nirmala

        Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.

There is help!

Rhythmic palatal myoclonus (RPM) is a rare movement disorder consisting of continuous synchronous jerks of the soft palate. Patients with essential RPM usually have objective earclicks as their typical complaint due to the pulling on the Eustachian tube. Head pressure iis also common with PM. The cause of PM is not known but it is our opinion that a dis-functional immune system is suspect and genetic predisposition that the inflammation from the immune system favors a certain area in the brain stem.

I am a Chiropractic Neurologist and Registered Medical Assistant and work mainly with chronic neurological conditions such as PM. I have treated dozens of cases and have developed a method called functional cranial release that has given many PM sufferers their life back. Besides FCR our clinic also uses medical treatments such as IV therapies and Stem Cells. It is my opinion that many PM conditions are rooted in chronic inflammation of a specific area in the brain stem. This inflammation can originate from chronic infection or toxic exposure.  Many cases continue to have symptoms after this original insult resolves. The dis regulated immune system is at the core of this inflammation! There may be an autoimmune connection is many cases of PM. Our therapy consists of proper testing and targeted therapy to bring back balance to the nervous system. Please feel free to look over some of the video’s on this site explaining what I do and some testimonials from PM patients I have treated.