| Identifier |
943-1 |
| Title |
Pendular Vertical Oscillations |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Contributors |
David Zee, 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 |
| Subject |
Pendular Vertical Oscillations; Lid Nystagmus; Bilateral Sixth Nerve Palsy; Bilateral Horizontal Gaze Palsy; Palatal Tremor (Myoclonus); Pontine Hemorrhage; Degenerative Hypertrophy of the Inferior Olivary Nucleus; Lesion in the Guillian Mollaret Triangle; Bilateral Horizontal Gaze Palsy Hemorrhage; Facial Palsy; Pontomedullary Cavernous Angioma; Bilateral Lid Nystagmus; Cavernous Hemangiomas |
| History |
In 1996 this 50 year old woman had the acute onset of tingling in the left arm and mild ataxia. In 1974 because of progressive symptoms and the onset of double vision attributed to a bilateral sixth nerve palsy, she consulted a neurologist at an outside hospital. She was given a diagnosis of Multiple Sclerosis. In 1990, she developed slurred speech and difficulty swallowing, due to a pseudobulbar palsy and a right facial weakness. Brain MRI showed a pontomedullary cavernous angioma with evidence of previous hemorrhage. In 1992, she was admitted to the Massachusetts General Hospital for surgical treatment. Neurological Examination: Alert and oriented x3 Speech dysarthric Pupils equal, normal reflexes. Complete bilateral 6th nerve palsy with esotropia Trigeminal sensation intact to light touch and pinprick Brisk corneal reflex OU Right lower motor neuron 7th nerve palsy Decreased hearing on the right Tongue protruded in the midline Palatal tremor (myoclonus) Motor System: 5/5 throughout without drift Reflexes 2+ upper extremity,1+ lower extremity with equivocal plantar responses Sensory Examination: Normal Co-ordination: Ataxia finger-to-nose, left > right Left dysdiadochokinesis Wide based ataxic gait On 7/20/92 Posterior Fossa Craniectomy and C1 arch removal. Microsurgical resection of a pontomedullary cavernous angioma with placement of a right occipital ventriculosotomy. The operative course was remarkable for several episodes of severe bradycardia. Post-operatively she had labile blood pressure and was slow to recover from the anesthesia On examination of her eye movements she was found to have pendular vertical oscillations (PVOs). A CT brain scan showed the cavernous bed had only a minimal rim of blood. The ventricles were small, the ventriculostomy was in good position and there was no hemorrhage or hydrocephalus. Hospital Course: In the Neurosurgical ICU she had a tracheostomy and gastrostomy. On 7/25/92 she was able to move her head, shoulders, right arm and both legs on command. The left arm was paretic. She became decerebrate to deep pain. Neuro-ophthalmic Consultation: Alert and followed one step commands Ocular Motility: Pendular vertical oscillations Bilateral 6th nerve palsy Bilateral horizontal gaze palsy Esotropia OD > OS Skew deviation with vertical misalignment left eye hypertropic Vertical gaze full on command Horizontal oculocephalic reflex absent Vertical oculocephalic reflex normal Bilateral lower motor neuron facial palsy Palatal tremor (myoclonus) Hospital Course: On 8/8/92 bilateral tarsorrhaphies were performed to protect the cornea. She had a stormy post-operative course in the Neurosurgical ICU. In September 1992 she was transferred to the Respiratory ICU to help wean her off the ventilator. In the RICU she remained neurologically stable but had great difficulty being weaned off the ventilator. On 9/10/92 Brain MRI showed: Resection of a portion of the pons, and a portion of the rostral medulla in the floor of the 4th ventricle, with post surgical filling of the space with cerebrospinal fluid. In addition, there was T2 hypointensity in the surgical bed with a blooming effect on the susceptibility sequence due to chronic blood products. On the flow sequence imaging, there was a punctate region of slightly increase signal in the upper pons which was thought to represent blood products or a small residual portion of the cavernos angioma with slow flow within it. The patient left the MGH to go to a skilled nursing facility. |
| 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. |
| 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 |
Pontomedullary cavernous angioma; Pontine hemorrhage; Palatal tremor (myoclonus) |
| Clinical |
This patient was filmed in the intensive care unit shortly after surgical resection of a pontomedullary cavernous angioma. The striking signs are: • Pendular vertical oscillations • Bilateral 6th nerve palsy • Bilateral horizontal gaze palsy • Esotropia OD > OS • Skew deviation with vertical misalignment left eye hypertropic • Vertical gaze full on command • Horizontal oculocephalic reflex absent • Vertical oculocephalic reflex normal • Bilateral lower motor neuron facial palsy • Palatal tremor (myoclonus) PVO's are characterized by • Smooth, pendular movements occurring at a frequency of 1 to 3 Hz (typically 2 Hz). • PVOs are accentuated under closed lids as in this patient • PVOs are synchronized with movements of the palate • Patient had no synchronous movements of the face, tongue or pharynx. Review ID923-1 and 936-4 alongside this case. |
| Presenting Symptom |
Double vision |
| Ocular Movements |
Pendular Vertical Oscillations; Lid Nystagmus; Bilateral Sixth Nerve Palsy; Bilateral Horizontal Gaze Palsy |
| Neuroimaging |
No neuroimaging studies are available in this patient. Brain MRI findings in two other patients with palatal tremor are illustrated: Case 1: Figure 1. Axial NECT scan shows a large pontine hemorrhage extending to the midbrain in a patient (ID936-4), who survived this massive hypertensive intracranial hemorrhage. Two years later he developed a 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 show a small mixed signal intensity in the dorsal midbrain tegmentum. Figure 3. Axial T2WI (same case as Fig. 2) shows bilateral enlargement of the inferior olivary nucleus with striking hyperintensity characteristic of classic hypertrophic olivary degeneration. Courtesy Anne Osborn, M.D. |
| Treatment |
Surgical resection and placement of a ventriculostomy |
| Etiology |
Pontomedullary cavernous angioma with 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 |
1992 |
| References |
1. 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 2. 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 3. 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 4. 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. 5. Keane JR. Acute vertical ocular myoclonus. Neurology 1986;36:86-89. http://www.ncbi.nlm.nih.gov/pubmed/3941790 6. 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. 7. 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. 8. Lopez LI, Bronstein AM, Gresty MA, Du Boulay EP, Rudge P. Clinical and MRI correlates in 27 patients with acquired pendular nystagmus. Brain. 1996;119:465-472. http://www.ncbi.nlm.nih.gov/pubmed/8800942 9. Nishie M, Yoshida Y, Hirata Y, Matsunaga M. Generation of symptomatic palatal tremor is not correlated with inferior olivary hypertrophy. Brain. 2002;125:1348-1357. http://www.ncbi.nlm.nih.gov/pubmed/12023323 |
| 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/s6vx3d4g |
| Setname |
ehsl_novel_shw |
| ID |
188562 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6vx3d4g |
