| Identifier |
907-1 |
| Title |
Ataxic Gait |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Contributors |
Anne Osborn, MD; David Zee, MD; Steve Smith, Videographer |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital; (AO) Professor of Radiology, University of Utah, Salt Lake City, Utah; (DZ) Johns Hopkins University, Baltimore, Maryland |
| Subject |
Square Wave Jerks; Dysmetria; Horizontal Saccadic Hypermetria; Horizontal Gaze Evoked Nystagmus; Saccadic Pursuit; Gait Ataxia; Alcoholic Cerebellar Degeneration; Gaze Evoked Horizontal Nystagmus; Horizontal Saccadic Dysmetria; Alcohol |
| History |
The patient is a 72 year old woman who presented with a 4 year history of progressive difficulty with balance, frequent falls and unsteadiness walking. She required a cane to steady herself. Past History: Significant for alcohol abuse. In 1980, she came to Boston for a second opinion and was seen in consultation by Dr Raymond Adams. Neuro-ophthalmological examination: Visual acuity, visual fields, pupils and fundus examination normal. Ocular motility: • Square wave jerks • Saccadic hypermetria, right gaze to center and left gaze to center • Horizontal gaze evoked nystagmus • Saccadic horizontal pursuit • Smooth vertical pursuit • A mild eso deviation of the eyes Neurological examination: Motor strength normal Reflexes 1+ symmetric Absent ankle jerk, plantar responses flexor Co-ordination: Slight titubation No limb ataxia Trunkal ataxia Gait ataxia Inability to tandem walk Hematological studies: Metabolic workup and liver function tests normal Diagnosis: Alcoholic cerebellar degeneration Treatment: The patient received intravenous thiamin and nutrition counseling prior to discharge. |
| Anatomy |
In ACD all types of neurons in the cerebellum are affected but the Purkinje cells in the dorsal vermis are most severely affected. The dorsal vermis (lobules VI and VII) receives inputs from the paramedian pontine reticular formation , nucleus reticularis tegmenti pontis (NRTP), dorsal and medial pontine nuclei, vestibular nuclei, and nucleus prepositus hypoglossi as well as from the inferior olivary nucleus. Purkinje cells in the dorsal vermis discharge before saccades. They encode target velocity during smooth pursuit and combined eye-head tracking. Stimulation of the vermis produces saccades. A topographical organization is evident: upward saccades are evoked from the anterior part, downward saccades from the posterior part, and ipsilateral, horizontal saccades from the lateral part. The main projection of the Purkinje cells of the dorsal vermis is to the caudal part of the fastigial nucleus - the most medial of the deep cerebellar nuclei. The fastigial ocular motor region receives input from the inferior olive and from the pontine nuclei especially the NRTP. Lesions of the fastigial nuclei generally cause marked saccadic hypermetria. |
| Pathology |
Alcoholic cerebellar degeneration is a restricted form of cerebellar degeneration. The histological criteria of ACD are: • Lesions varying in severity and restricted to the anterior and superior vermis • Nearly complete loss of the Purkinje cells • Narrowing of the molecular layer • Bergmann glia are proliferated and granular cells markedly reduced. In an ACD autopsy study in Japan only cases in which severe lesions had spread from the superior vermis to the anterior lobe and/or simple lobule presented with cerebellar signs. The clinical signs and the autopsy findings closely match the very detailed description and case illustrations in Victor, Adams and Mancall's landmark paper. The authors suggested that the restricted form of cortical cerebellar degeneration seen in the serious alcoholic represents a discreet clinical pathological entity. |
| Disease/Diagnosis |
Alcoholic Cerebellar Degeneration |
| Clinical |
The ocular motility signs in this patient with alcoholic cerebellar degeneration are: • Square wave jerks • Saccadic hypermetria, right gaze to center and left gaze to center • Horizontal gaze evoked nystagmus • Saccadic horizontal pursuit • Smooth vertical pursuit • A mild eso deviation of the eyes • Trunkal ataxia • Gait ataxia • Inability to tandem Square wave jerks are pairs of uncalled for small horizontal saccades, typically less than 2 degrees, that take the eye away from the target and then return it within 200 msec. They disrupt fixation in primary gaze in patients with cerebellar disease. Saccadic dysmetria is an important cerebellar sign. When present it helps to localize the lesion. A lesion of the dorsal vermis produces asymmetrical dysmetria, with hypometric (undershoot) saccades ipsilateral to the lesion and hypermetric (overshoot) contralateral to it. Bilateral horizontal saccadic hypermetria, right gaze to center and left gaze to center, as in the patient, is probably due to damage to the fastigial nuclei and cerebellar pathways. Review Box 12-4 Deficits caused by lesions of the dorsal vermis, fastigial nucleus and uncinate fasciculus (ref (3)). Gaze evoked nystagmus with quick phases directed away from the central position is due to a deficient eye position signal. The nucleus prepositus hypoglossi and the medial vestibular nucleus are the major brainstem nuclei that maintain eccentric horizontal gaze. Disorders of ocular misalignment associated with cerebellar disease include: 1. Esotropia (illustrated by this patient) 2. Alternating skew deviation 3. Divergence paralysis, especially for distance viewing The clinical syndrome of alcoholic cerebellar degeneration (ACD) is remarkably stereotyped. The usual presentation, as in this patient, is progressive unsteadiness in walking evolving over months and years. ACD predominantly affects stance, eye movements, and gait, sometimes with trunkal ataxia and titubation. Dysarthria and upper limb ataxia are rare. Ataxia may develop during periods of abstinence. Identical cerebellar degeneration has been observed in non-alcoholic patients with severe malnutrition. |
| Presenting Symptom |
Unsteady walking |
| Ocular Movements |
Square Wave Jerks; Dysmetria; Horizontal Saccadic Hypermetria; Horizontal Gaze Evoked Nystagmus; Saccadic Pursuit |
| Neuroimaging |
Neuroimaging studies were not available in this patient. Illustrative images in another patient with ACD are shown here. Figure 1. Sagittal T1WI shows striking atrophy of the superior vermis. Figure 2. Axial T2WI through the upper midbrain and vermis shows the cerebellar folia are thinned and the CSF spaces increased. (Courtesy of Anne Osborn, M.D.) |
| Treatment |
Damage to the cerebellum is irreversible. Stopping the addiction to alcohol plus vitamin B therapy and improved nutrition may help prevent further degeneration. |
| Etiology |
Alcohol consumption contributes to cerebellar degeneration, although the correlation between the quantity of alcohol consumption, the severity of cerebellar atrophy and the development of clinical cerebellar signs are still a matter of dispute. The Japanese study confirmed the significant frequency of subclinical cerebellar degeneration in alcoholics, supporting the hypothesis that early intervention in alcoholism in the subclinical phase is important to prevent the development of cerebellar symptoms. The therapeutic approach includes counseling, improved nutrition and vitamin B therapy. |
| Supplementary Materials |
Alcoholic Cerebellar Degeneration: https://collections.lib.utah.edu/details?id=2174173 |
| Date |
1980 |
| References |
1. Gallucci M, Amicarelli I, Rossi A, Stratta P, Masciocchi C, Zobel BB, Casacchia M, Passariello R. MR imaging of white matter lesions in uncomplicated chronic alcoholism. J Comput Assist Tomogr. 1989;13:395-398. http://www.ncbi.nlm.nih.gov/pubmed/2723168 2. Gilman S, Adams K, Koeppe RA, Berent S, Kluin KJ, Modell JG, Kroll P, Brunberg JA. Cerebellar and frontal hypometabolism in alcoholic cerebellar degeneration studied with positron emission tomography. Ann Neurol. 1990;28:775-785. http://www.ncbi.nlm.nih.gov/pubmed/2285264 3. Leigh RJ, Zee DS. Diagnosis and Central Disorders of Ocular Motility Ch12;598-718. In: The Neurology of Eye Movements. 4th Ed. Oxford University Press, New York, 2006. 4. Nicolas JM, Fernandez-Sola J, Robert J, Antunez E, Cofan M, Cardenal C, Sacanella E, Estruch R, Urbano-Marquez A High ethanol intake and malnutrition in alcoholic cerebellar shrinkage. QJM 2000; 93:449-456. http://www.ncbi.nlm.nih.gov/pubmed/10874054 5. Victor M, Adams RD, Mancall EL. A restricted form of cerebellar degeneration occurring in alcoholic patients. Arch Neurol 1959;1:579-688. 6. Yokota O, Tsuchiya K, Terada S, Oshima K, Ishizu H, Matsushita M, Kuroda S, Akiyama H. Frequency and clinicopathological characteristics of alcoholic cerebellar degeneration in Japan: a cross-sectional study of 1,509 postmortems. Acta Neuropathol (Berl). 2006;112:43-51. http://www.ncbi.nlm.nih.gov/pubmed/16622656 7. Zasorin NL, Baloh RW. Downbeat nystagmus with alcoholic cerebellar degeneration. Arch Neurol. 1984;41:1301-1302. http://www.ncbi.nlm.nih.gov/pubmed/6497735 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
3/4" Umatic master videotape |
| Relation is Part of |
924-3 |
| 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/s6zs5t1d |
| Setname |
ehsl_novel_shw |
| ID |
188538 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6zs5t1d |
