++
A spinocerebellar degeneration. Abnormal eye movements
include absent rapid saccades (scanning) and abnormally slow pursuit
(tracking). Lethal within 10 years of onset.
++
Cerebellar Degeneration with Slow Eye Movements.
++
Rare disease with autosomal
dominant inheritance (also possibly an autosomal recessive form).
++
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.
++
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]