| Identifier |
936-4 |
| Title |
Palatal Tremor |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Contributors |
David Zee, MD; John Leigh, MD; Steve Smith, Videographer |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital; (DZ) Johns Hopkins Hospital, Baltimore, Maryland; (JL) University Hospital, Cleveland, Ohio |
| Subject |
Pendular Vertical Oscillations; Lid Nystagmus; Bilateral Horizontal Gaze Palsy; Palatal Tremor (Myoclonus); Pontine Hemorrhage; Degenerative Hypertrophy of the Inferior Olivary Nucleus; Lesion in the Guillain - Mollaret Triangle; Bilateral Horizontal Gaze Palsy Hemorrhage; Facial Weakness; Speech Language Disturbance |
| History |
The patient is a 44 year old left handed man with a history of IV drug abuse (heroin and cocaine) alcoholism, hypertension, and rheumatic fever. In March 1990, at 3 a.m. on the day of admission, he had acute onset of dizziness, slurred speech, left sided weakness and difficulty walking. He was admitted to a local hospital and intubated for airway protection. A CT scan showed a right pontine hemorrhage. He was transferred to the Massachusetts General Hospital. Neuro-ophthalmological examination in the ICU: Pupils pinpoint, 2 mm. OU reactive to light Eyes dysconjugate at rest Bilateral esotropia OS > OD Bilateral horizontal gaze palsy Good convergence He converged his eyes when attempting to look right and left Full vertical gaze Full upgaze with upbeat nystagmus Torsional (rotary) nystagmus (unsustained) on downgaze Absent horizontal oculocephalic reflexes Ice water calorics abnormal. No horizontal deviation of the eyes or nystagmus. Following irrigation in either ear, there was a slow intermittent downward conjugate bob of the eyes (without rhythmic frequency) persisting for a few seconds only. CT, non-contrast: An extensive hemorrhage was seen in the pons on the right greater than the left side extending to the midbrain. The pons appeared swollen down to the pontomedullary junction but not below. No blood in the fourth ventrical, no hydrocephalus. Punctate densities were noted in the area of hemorrhage suggestive of calcification or tortuous blood vessels of an arteriovenous malformation. Hospital course: By day 14 he was alert, answered yes/no questions shaking his head and followed simple one step commands. Eye movements at that time showed: • Bilateral horizontal gaze palsy • He converged his eyes in attempting to look right and left • Horizontal oculocephalic reflexes absent • Ice water calorics absent Neurological examination on day 17: Alert and cooperative Dysarthric speech Left motor neuron facial palsy (Bell's palsy) Left hemiplegia with hyperreflexia Plantar responses flexor Sensation normal Cerebral arteriogram on day 17: No arteriovenous malformation. The patient recovered slowly and was able to ambulate using a wheelchair before he was transferred to rehab. He was then discharged to a home for the handicapped. In September 1990 he was readmitted with a chief complaint of oscillopsia - the visual world moving up and down. Ocular motility showed: • Primary position vertical oscillations (VOs) • VOs increasing in amplitude on upgaze • Lid nystagmus with eyes closed • Accentuation of VOs with lid closure • Bilateral horizontal gaze palsy • Normal convergence • Full vertical gaze • Clockwise torsional movements on downgaze No abnormal movements of the facial muscles, palate, larynx or neck. Electronystagmogram: 1. Spontaneous and gaze nystagmus - There is constant eye movement. This has a horizontal component that is equal in amplitude and veolocity to the right and left and has amplitude of 3-4°. There is also a vertical component with equal velocity up and down and an amplitude of 2-3°. These movements decrease, but do not stop with eye closure. 2. Saccades - Saccades are slow to right and left 3. Pursuit (tracking) 0.2 Hertz: Saccadic breakdown to the right and left 0.4 Hertz: Saccadic breakdown to the right and left 4. Optokinetic nystagmus - No nystagmus provoked. 5. Positional Testing: a. Hallpikes/Eyes open in the light - Couldn't do because of the paralysis in his left side. b. Static/Eyes open in the dark - No nystagmus. Above noted eye movements persist. c. Static/Eyes closed - No nystagmus. Eye movements persist. 6. CALORICS - No right beating nystagmus provoked. Appropriate left beating nystagms provoked after right cool and left warm. Good fixation suppression of caloric nystagmus after left 44, but poor fixation suppression after right sided stimulation. In 1991, he became aware, for the first time, of twitching around the right corner of his mouth. In 1992 a palatal tremor (myoclonus) was noted. Electromyographic studies Periodic myoclonic discharges were recorded in the orbicularis oris bilaterally. A palatal needle electerode recorded rhythmic 2 to 3 Hz movement synchronous with the ocular PVOs. Eye movement recordings made in horizontal and vertical planes demonstrated rhythmic 2 to 3 Hz vertical oscillations. Brain MRI showed: 1. A 20 x 9 x 6 mm area of tissue loss present in the superior pons with a T2 hypointense rim. 2. An area of magnetic susceptibility in the right external capsule and clostrum consistent with old hemorrhage 3. A hyperintense T-2 signal in the right inferior olivary nucleus Brainstem MRI, with 3 mm thick interleaved T1 and T2 weighted signal sequences was performed to evaluate the inferior olives. In the right inferior olivary nucleus there were areas of heterogeneous T2 bright signal and on T1 weighted axial images a subtle decrease signal intensity. The findings were consistent with unilateral degenerative hypertrophy of the inferior olivary nucleus. Impression: Infarction of the right inferior olivary nucleus. |
| Anatomy |
According to Guillain and Mollaret the crucial location for the lesion(s) producing palatal tremor is one that involves the dentato-olivary pathway through the superior cerebellar peduncle. This pathway is an interconnecting circuit connecting three brainstem nuclei - the dentate, the red nucleus and the inferior olivary nucleus. The lesion can be located in one of four places: 1. The dentate nucleus 2. The dentate outflow through the superior cerebellar peduncle 3. At the level of the red nucleus where the pathway passes dorsally and inferior to the contralateral red nucleus or 4. In the descending central tegmental tract to the contralateral inferior olivary nucleus. More recent studies have implicated interruption of a pathway from the deep cerebellar nuclei through the superior cerebellar peduncle, which then loops caudally through the central tegmental tract to the inferior olive. When the syndrome is due to unilateral infarction of the dentate nucleus and superior cerebellar peduncle, hypertrophic changes in the inferior olivary nucleus appear on the contralateral side, as in this patient with a cavitary infarct in the left brainstem and contralateral hypertrophy of the right inferior olivary nucleus. |
| Pathology |
Histologically, the olivary nucleus is enlarged, due to hypertrophy of neurons that contain increased acetylcholinesterase reaction product. Such changes begin within a month of the stroke and maximize in about six months, and are accompanied by astrocytosis, and synaptic and axonal remodeling. At the same time, the number of olivary neurons progressively declines, so that after six years, they are less than 10% of control brains. Also, both the myelin and the axons of efferent fibers from olivary neurons are severely degenerated in patients with persistent palatal tremor who survive several years. Despite the anatomic demonstration of atrophy, functional imaging studies suggest increased metabolism of the inferior olive. |
| Disease/Diagnosis |
Palatal tremor (myoclonus); Pontine hemorrhage; Degenerative hypertrophy of the inferior olivary nucleus |
| Clinical |
This patient with a pontine hemorrhage was filmed in the ICU. He had: • Bilateral esotropia left > right in primary gaze • Bilateral horizontal gaze palsy • Normal convergence and in attempting to look right or left. He made convergence movements • Vertical gaze normal • Upbeat nystagmus on upgaze • Horizontal oculocephalic reflex absent (Doll's head maneuver) • Left Bell's palsy The second recording was made seven months after his hemorrhage when he had: • Primary position vertical oscillations (VOs) • VOs increasing in amplitude on upgaze • Lid nystagmus with eyes closed • Accentuation of VOs with lid closure • Bilateral horizontal gaze palsy • Normal convergence • Full vertical gaze • Clockwise torsional movements on downgaze Two years later, this patient was found to have vertical ocular oscillations, palatal tremor (myoclonus) and rhythmic movements of the facial muscles and neck. (9) Comment: In 1992 I presented this case at the Dizziness Update Course in Toronto. Dr. David Zee and Dr. John Leigh reviewed the eye movement recordings. They considered that the eye movements were significant for their torsional component and that they did not mimic the pendular vertical oscillations (PVOs) seen in another patient of mine (ID923-1) with palatal tremor. PVOs are characterized by: • Smooth, pendular movements occurring at a frequency of 1 to 3 Hz (typically 2 Hz). • PVOs are accentuated under closed lids • PVOs are synchronized with movements of the palate, facial muscles, pharynx, tongue, larynx and diaphragm. Review ID923-1 and 927-1 alongside this case. Box10-10 Clinical Features of Acquired Pendular Nystagmus Pg 506 (13). |
| Presenting Symptom |
Coma |
| Ocular Movements |
Pendular Vertical Oscillations; Lid Nystagmus; Bilateral Horizontal Gaze Palsy; Normal Convergence; Full Vertical Gaze; Clockwise Rotary Eye Movements on Downgaze |
| Neuroimaging |
The MRI in this case and in one other patient who developed palatal tremor are illustrated here. Figure 1. Axial NECT scan in this patient shows a large pontine hemorrhage extending to the midbrain. The patient survived this massive hypertensive intracranial hemorrhage and two years later developed palatal tremor. Case 2: Figure 2. Axial T2WI in a patient who developed palatal tremor 6 months after a midbrain bleed from a cavernous malformation shows a small mixed signal intensity lesion in the dorsal midbrain tegmentum. Figure 3. Axial T2WI (same case as Fig. 2) shows enlarged olives with striking hyperintensity characteristic for classic hypertrophic olivary degeneration. Courtesy Anne Osborn, M.D. |
| Treatment |
Only rarely does oculopalatal tremor resolve spontaneously. Gabapentin, ceruletide, memantine, and anticholinergic agents may help some patients. Drugs that block connexin channels and thereby reduce synchronized discharge of electronically coupled olivary neurons might provide a new therapeutic approach. |
| Etiology |
Pontine hemorrhage |
| Supplementary Materials |
Palatal Tremor: https://collections.lib.utah.edu/details?id=2174223 Pendular Vertical Oscillations: https://collections.lib.utah.edu/details?id=2174230 |
| Date |
1990 |
| References |
1. Averbuch-Heller L, Tusa RJ, Fubry L, Rottach KG, Ganser GL, Heide W, Büttner U, Leigh RJ. A double-blind controlled study of gabapentin and baclofen as treatment for acquired nystagmus. Ann Neurol 1997;41:818-25. http://www.ncbi.nlm.nih.gov/pubmed/9189045 2. Barton JJ, Cox TA. Acquired pendular nystagmus in multiple sclerosis: clinical observations and the role of optic neuropathy. J Neurol Neurosurg Psychiatry. 1993 Mar;56(3):262-267. http://www.ncbi.nlm.nih.gov/pubmed/8459242 3. Dehaene I., Van Zandycke M, Appel B. Acquired pendular nystagmus. Neuro-ophthalmol 1987;7(5);297-300. 4. 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. http://www.ncbi.nlm.nih.gov/pubmed/7922465 5. 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. http://www.ncbi.nlm.nih.gov/pubmed/2011257 6. Gautier JC, Blackwood W. Enlargement of the inferior olivary nucleus in association with lesions of the central tegmental tract or dentate nucleus. Brain 1961;84(3):342-361. http://www.ncbi.nlm.nih.gov/pubmed/13897315 7. Goyal M, Versnick E, Tuite P, Saint Cyr J, Kucharczyk W, Montanera W, Willinsky R, Mikulis D. Hypertrophic olivary degeneration: meta-analysis of the temporal evolution of MR findings. Am J Neuroradiol 2000; 21:1073-1077. http://www.ncbi.nlm.nih.gov/pubmed/10871017 8. 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. 9. Katz B, Hoyt W, Townsend J. Ocular Bobbing and Unilateral Pontine Hemorrhage. Report of a Case. J Clin Neuro-ophthalmol 1982;2:193-195. http://www.ncbi.nlm.nih.gov/pubmed/6217223 10. Keane JR. Acute vertical ocular myoclonus. Neurology 1986;36:86-89. http://www.ncbi.nlm.nih.gov/pubmed/3941790 11. Koeppen AH. Olivary hypertrophy; histochemical demonstration of hydrolytic enzymes. Neurology 1980;30:471-480. http://www.ncbi.nlm.nih.gov/pubmed/6245389 12. 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. 13. Leigh RJ, Zee DS. Diagnosis of Nystagmus and Saccadic Intrusions. Chp 10:475-558. In: The Neurology of Eye Movements, 4th Edition. Oxford University Press, New York 2006. 14. Lopez LI, Bronstein AM, Gresty MA, Du Boulay EP, Rudge P. Clinical and MRI correlates in 27 patients with acquired pendular nystagmus. Brain. 1996 Apr;119 ( Pt 2):465-472. http://www.ncbi.nlm.nih.gov/pubmed/8800942 15. Nishie M, Yoshida Y, Hirata Y, Matsunaga M. Generation of symptomatic palatal tremor is not correlated with inferior olivary hypertrophy. Brain. 2002 Jun;125(Pt 6):1348-1357. http://www.ncbi.nlm.nih.gov/pubmed/12023323 16. Ruigrok TJ, deZeeuw CI, Vogel J. Hypertrophy of inferior olivary neurons : a degenerative regenerative or plasticity phenomenon. Eur J Morphol 1990 ;28 :224-239. http://www.ncbi.nlm.nih.gov/pubmed/2245132 17. Samuel M, Torun N, Tuite PJ, Sharpe JA, Lang AE. Progressive ataxia and palatal tremor (PAPT): clinical and MRI assessment with review of palatal tremors. Brain. 2004 Jun;127(Pt 6):1252-1268. Epub 2004 Apr 16. http://www.ncbi.nlm.nih.gov/pubmed/15090471 18. Yokota T, Hirashima F, Furukawa T, Tsukagoshi H, Yoshikawa H. MRI findings of inferior olives in palatal myoclonus J Neurol 1989;236:115-116. http://www.ncbi.nlm.nih.gov/pubmed/2709052 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
3/4" Umatic master videotape |
| Relation is Part of |
3-2, 167-6, 923-1, 927-1, 936-4 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Shirley H. Wray Collection: https://novel.utah.edu/Wray/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6f21w7h |
| Setname |
ehsl_novel_shw |
| ID |
188519 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6f21w7h |
