Essential Palatal Tremor

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Identifier 929-1
Title Essential Palatal Tremor
Ocular Movements Normal
Creator Shirley H. Wray, M.D., Ph.D., FRCP, Professor of Neurology Harvard Medical School, Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Contributor Primary Shirley H. Wray, MD, PhD, FRCP, Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Subject Essential Palatal Tremor (Myoclonus); Brainstem Encephalitis
Supplementary Materials PowerPoint Presentation: Essential Palatal Tremor: Shirley H Wray, M.D., Ph.D.
Presenting Symptom Clicking tinnitus
History The patient is a 25 year old meteorologist from Tennessee who came to Boston in the summer of 1992 to vacation with his family on the Cape. His illness started with flu-like symptoms, low grade fever from 99 to 100F, sweating and episodes of light headedness associated with occasional nausea, indigestion, generalized fatigue and headaches. He described a feeling of "dizziness" associated with the feeling of passing out. He denied vertigo or spinning sensation. Approximately at the same time of onset of systemic symptoms, he developed a clicking noise in the back of his eyes, bridge of his nose, through his ears and in his throat most noticeable in bed at night. The clicking noise interfered with his sleep and he found he could only sleep for about 1 hour at a time before waking. When he examined the back of his mouth in the mirror, he saw his palate moving up and down and he showed this abnormality to his fiancée and subsequently consulted his dentist. He was prescribed Valium 10 mg. nocte to treat insomnia. Past History: Positive for EBV infection with hepatitis in 1986. Concussion playing hockey at school. An unexplained episode of high fever in 1981 when traveling in Europe. Family History: Positive for migraine, grandmother and mother Social History: Heavy drinker in college up to age 22. Marijuana use and cocaine on two or three occasions only over a period of four to five years. Symptomatic Inquiry: Negative for hiccups Difficulty swallowing or breathing Visual symptoms and deafness Neurological examination: Normal examination apart from the presence of palatal tremor (myoclonus). Palatal tremor is one of the rarest movement disorders among the rhythmic hyperkinesias. Formerly, palatal tremor was called palatal myoclonus, rhythmic palatal myoclonus, oculopalatal myoclonus, palatal nystagmus and brainstem or palatal myorhythmia. The movements of the palate meet all the criteria of a tremor i.e. a rhythmic ongoing movement of at least one functional region, in this case, the soft palate. Consequently, at the First International Congress of Movement Disorders in 1990, it was reclassified among tremors. Palatal tremor can be separated into two distinct clinical entities arising from different pathophysiological mechanisms. 1. Symptomatic palatal tremor (SPT) 2. Essential palatal tremor (EPT). The palatal tremor in SPT is due to activation of the levator veli palatini muscle. The audible click in EPT is due to the rhythmic action of the tensor veli palatini muscle which is responsible for opening the eustachian tube. Valsalva's maneuver does not consistently affect the rhythm of the tremor in either group. Essential palatal tremor: Nearly all patients with EPT complain of rhythmic ear clicks and have no visual symptoms. Patients with SPT are often unaware of the palatal movements and have no ear clicks. They may complain of coincidental oscillopsia and on examination have symptoms and signs of brainstem or cerebellar dysfunction. Neurological examination: The rhythmic ear clicks were audible to the examiner at the bedside. They were a source of severe psychological distress to the patient. The other features of EPT are: 1. Males predominate over females 2. In general, the frequency of the palatal tremor is below 120 jerks per minute 3. It is not associated with pendular vertical oscillations or rhythmic movements of the face, lips, neck or diaphragm. 4. Duration is usually lifelong but remissions may occur, as in this case. Cessation during sleep can occur. Pathology: The pathology is unknown. EPT is not associated with hypertrophy of the inferior olivary nucleus. Investigations: Lumbar puncture: Normal cerebrospinal fluid, protein 33 mg/dl, glucose 75 mg/dl, no cells Lyme disease serology: Negative. Brainstem auditory evoked responses: Normal. Brain MRI with Gadolium: No abnormality. Brain FDG PET scan: Tomographic images of the brain were obtained No definite focus of abnormal metabolic activity detected. Electromyographic studies: An attempt was made to record from the soft palate using a direct needle electrode but the electrode was repeatedly dislodged. Duodenal biopsy: Normal. PAS stain negative. Whipple's disease was considered ruled out. Diagnosis: Essential palatal tremor Microsurgical therapy: An ENT specialist advised the patient that the palatal movements were due to activation of the tensor veli palatini muscle and that the muscle could be cut to stop the clicking noise. The procedure, however, carried the risk of leaving him with a nasal quality to his speech and, as a part-time weather man on the radio, he declined the procedure. Botulinum toxin: In 1993 experience with botulinum toxin (BT) treatment of palatal tremor was limited. A phone call to the Movement Disorder Unit at the National Institute of Health revealed that two SPT cases had recently been successfully treated with small doses of botox. In both cases they found the levator veli palatini muscle to be quite hypertrophied. The successful treatment, however, only lasted three months before a second injection was required. Medications: The patient selected a trial of Valium 10 mg b.i.d. which helped him to sleep. Over time the palatal tremor began to slow down. Depression: The patient became very concerned that he may have "a widespread viral disease" . He had noted fasciculations in his quadriceps and forearm muscles following exertion. He consulted a psychologist and benefited from supportive therapy and hypnosis for relaxation. Whilst under hypnosis the palatal tremor slowed down and the clicking noise was softer. The patient demonstrated this to the psychologist by finger tapping in time with the click that he could hear. The frequency was reduced to 1 cycle per second. A trial of Wellbutrin was unsuccessful. In April 1993, the patient obtained a second neurological opinion. The diagnosis of EPT was confirmed. Klonopin 0.5 mg one tablet t.i.d. was prescribed to replace valium. The patient felt that Klonopin worked as well as valium in suppressing the palatal tremor but was less sedating. He continued on Klonopin and returned for annual follow-up in Boston in 1994. At this time, the tremor and click dramatically reduced and he was able to return to full time work.
Clinical This young patient with EPT has: • Constant rhythmic palatal tremor associated with a loud click. • No pendular vertical oscillations • No rhythmic movements of the face, lips or neck A second recording was made one year after treatment with Klonopin by which time the tremor and click were reduced. He was able to return to full time work.
Neuroimaging MRI: Brain MRI in 1993 without gadolinium showed no abnormality. 1993 FDG PET scan of the brain showed no definite focus of abnormal metabolic activity. Specifically, the region of the brainstem does not demonstrate any definite focus of increased metabolism.
Anatomy Medulla
Pathology Idiopathic
Disease/Diagnosis Essential rhythmic palatal myoclonus.
Treatment See Discussion.
References 1. Deuschl G, Toro C, Valls-Solé J, Zeffiro T, Zee DS, Hallett M. Symptomatic and essential palatal tremor. 1. Clinical, physiological and MRI analysis. Brain. 1994 Aug;117 ( Pt 4):775-788. 2. Dubinsky RM, Hallett M, Di Chiro G, Fulham M, Schwankhaus J. Increased glucose metabolism in the medulla of patients with palatal myoclonus. Neurology. 1991 Apr;41(4):557-562. 3. Guillain G, Mollaret P. Deux cas myoclonies synchrones et rhythmées vélo-pharyngo-laryngo-oculodiaphragmatiques: Le problèm anatomique et physiolopathologique de ce syndrome. rev. Neurol (Paris) 1931;2:545-566. 4. Leigh RJ, Hong S, Zee DS, Optican LM. Oculopalatal tremor: clinical and computational study of a disorder of the inferior olive. Soc Neurosci Abstr 2005; 933.8.
Relation is Part of 3-2, 927-1,936-4
Contributor Secondary Steve Smith, Videographer; Ray Balhorn, Digital Video Compressionist
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 1993
Type Image/MovingImage
Format video/mp4
Source 3/4" Umatic master videotape
Rights Management Copyright 2002. For further information regarding the rights to this collection, please visit:
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E, SLC, UT 84112-5890
Collection Neuro-ophthalmology Virtual Education Library: NOVEL
Language eng
ARK ark:/87278/s6jm5782
Setname ehsl_novel_shw
Date Created 2005-08-22
Date Modified 2017-11-22
ID 188549
Reference URL
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