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A spinocerebellar degeneration. Abnormal eye movements include absent rapid saccades (scanning) and abnormally slow pursuit (tracking). Lethal within 10 years of onset.

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Cerebellar Degeneration with Slow Eye Movements.

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Rare disease with autosomal dominant inheritance (also possibly an autosomal recessive form).

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Slowing of all eye movements thought to be caused by a brainstem lesion of the paramedian pontine reticular formation. Clinical features include an abnormal accompanying movements of head and neck; spinocerebellar degeneration with abnormal gait (ataxia); progressive intellectual impairment, extrapyramidal dysfunction and peripheral neuropathy. Skeletal abnormalities can occur. Muscle biopsy shows nonspecific mitochondrial abnormalities. Magnetic resonance imaging studies of the brain show a significant degree of cerebellar and brainstem atrophy. Death occurs within 10 years of onset.

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It is recommended to evaluate carefully the neurological function (clinical, EEG, CT/MRI) and muscular condition for the presence of myotonia. Careful intraoperative positioning is needed.

Najim al-Dim AS, al-kurdi A, Dasouki M, et al: Autosomal recessive ataxia, slow eye movements and psychomotor retardation. J Neurol Sci 12(4):61, 1994.
Wadia NH, Swami RK: A new form of heredo-familial spinocerebellar degeneration with slow eye movements. Brain 94:359, 1971.  [PubMed: 5571047]

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