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Show ORIGINAL CONTRIBUTION Periodic Alternating Nystagmus Provoked by an Attack of Meniere's Disease Bill Chiu, MD, and Timothy C. Hain, MD Periodic alternating nystagmus is a rare central nervous system disorder in which the eyes undergo a horizontal jerk nystagmus that periodically reverses direction. A patient with a hypoplastic cerebellum and enlarged cisterna magna exhibited transient periodic alternating nystagmus following an attack of Meniere's disease. We hypothesize that in susceptible individuals with cerebellar disturbances, periodic alternating nystagmus may be transiently induced by vestibular stimuli. ( J Neuro- Ophthalmol 2002; 22: 107- 109) An enlarged cisterna magna is usually an anatomic finding unaccompanied by clinical abnormalities ( 1- 3). Nevertheless, as it is a developmental anomaly, it may be associated with genetic disorders such as trisomy 18 ( 4,5) and cerebellar dysgenesis ( 6- 10). We report a case in which a patient with an enlarged cisterna magna and cerebellar hypoplasia exhibited transient periodic alternating nystagmus ( PAN) during an attack of Meniere's disease. PAN is a congenital or acquired central nervous system disorder in which the eyes undergo a horizontal jerk nystagmus that reverses direction about every two minutes ( 11). When acquired, PAN has been preceded by either loss of vision or a new brain stem disorder ( 12). Theoretical studies suggest that PAN in persons with cerebellar disturbances may be triggered by vestibular disturbances ( 13). We postulate that the transient PAN in our patient was a manifestation of the combination of a vestibular imbalance and latent cerebellar dysfunction. Case Report A 51- year- old Caucasian male with a history of decreased hearing in his left ear and recurrent vertigo over 10 years attributed to Meniere's disease presented with two Departments of Otolaryngology- Head and Neck Surgery, Neurology, and Physical Therapy- Human Movement Sciences, Northwestern University Medical School, Chicago, Illinois Address correspondence to Timothy C. Hain, MD, Associate Professor, Northwestern University Medical School, 645 North Michigan, Suite 1100, Chicago, IL 60611- 3008, USA; E- mail: t- hain@ northwestern. edu drop attacks. The drop attacks were not associated with headache, dizziness, vertigo, loss of consciousness, confusion, visual changes, nausea, vomiting, or chest pain, and were attributed to the " otolithic crises of Tumarkin" found in advanced Meniere's disease ( 14). He was not taking any anticonvulsant medications. His temperature was 98.7 F, respiratory rate 18, pulse 69, and blood pressure 138/ 77 without orthostatic variation. Neurologic examination was normal except for the following: decreased hearing in the left ear, inability to perform tandem walking, and a positive Romberg sign. While in the hospital, he developed an episode of vertigo with nausea and vomiting, during which his eyes showed strong horizontal nystagmus reported by one observer to be left-beating and by another to be right- beating. Magnetic resonance imaging ( MRI) of the brain demonstrated an enlarged cisterna magna and cerebellar hypoplasia ( Fig. 1). No previous images were available for comparison. Subsequent work- up ruled out epileptic seizures and cardiac causes of syncope. Electronystagmography confirmed an alternating right- beating and left- beating nystagmus with a periodicity of approximately 40 seconds, consistent with PAN ( Fig. 2). Clinical examination with Frenzel's goggles also documented PAN. Pursuit was normal for age. Fixation suppression was modestly impaired at low frequencies; gain was greater than 0.1 from 0.01 to 0.1 Hz during visual- vestibular interaction testing using a rotatory chair. Following treatment with baclofen 10 mg three times daily, the nystagmus abated. On repeat examination using Frenzel's goggles 1 month later, it was not evident. Baclofen was stopped at that point, and on a repeat examination 6 months later, PAN was again not evident. Discussion PAN has previously been reported in various forms. Congenital PAN is mainly found in albinism ( 15,16), while acquired PAN has been reported in cerebellar disorders and after visual impairment ( 17,18). Both congenital and acquired PAN can often be abolished by treatment with baclofen ( 19,20). „ JNeuro- QpMialpiol,. Vol. 22, Nn. t2, 2002 „ , „ , . , . , . . DOI: 10.1097/ 01. WNO. 0Q00019664.1S442.76 , .107 , Copyright © Lip pincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 Periodic Alternating Nystagmus FIG. 1. Magnetic resonance image of the brain demonstrates diffusely hypoplastic cerebellum and large cisterna magna. A: Axial view at the level of the 8th nerves. B: Sagittal view. Cerebellar disorders associated with PAN include arachnoid cyst of the posterior fossa ( 21), Arnold- Chiari malformation ( 22), spinocerebellar degeneration ( 23- 26), various focal cerebellar lesions associated with multiple sclerosis ( 27), and cysticercosis ( 28). PAN can occur as a side effect of anticonvulsant medication ( 29), and in this case it is presumably also related to cerebellar dysfunction. There is substantial evidence that PAN can be the result of damage to the cerebellar uvula and nodulus. For example, PAN can be induced in monkeys by lesions of the nodulus ( 30,31). In humans, many reports of PAN are associated with diffuse cerebellar lesions, but some have been associated with small lesions of the nodulus. Kennard et al. ( 32) reported a case of PAN in a patient with cerebellar medullo-blastoma, a tumor that arises in the cerebellar nodulus. In our case, we hypothesize that the cerebellum, including the FIG. 2. Electronystagmographic recording of patient's nystagmus. An infrared video method was used to record eye movements ( Visual- Eyes, Micromedical Technology, Chatham IL). A: Position trace between 25 and 60 seconds showing a right- beating nystagmus between 30 and 42 seconds, a period during which the eyes are nearly still between 42 and 45 seconds, followed by a left- beating nystagmus between 45 and 60 seconds. B: Median eye velocity is plotted for every second. Slow phase velocity alternates between 5 degrees/ s left and right beating, with a period of 40 seconds. 100 150 Time, Sec nlUo. , „.. „ . . © 2002 Lippincott Williams & Wilkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Chiu and Haiti JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 nodulus or uvula or their connections, was damaged early in life, thus resulting in an enlarged cisterna magna and a tendency toward PAN. PAN can also be associated with visual disturbances, such as cataract, and resolve when vision improves ( 17,18). This information suggests that PAN does not require a central lesion. Theoretical analyses of PAN postulate that it is related to loss of central inhibition of vestibular circuitry ( 13,33,34). In these models, PAN can be induced with vestibular signals. In our patient, perhaps the Meniere's attack provided the trigger for PAN in a similar fashion. REFERENCES 1. Adam R, Greenberg JO. The mega cistema magna. J Neurosurg. 1978; 48: 190- 2. 2. Haimovici JA, Doubilet PM, Benson CB, et al. Clinical significance of isolated enlargement of the cistema magna (> 10 mm) on prenatal sonography. J Ultrasound Med 1997; 16: 731- 4; quiz 735- 6. 3. Lusins JO, Nakagawa H. 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