Abstract:
:Analysis of the temporal sequence of neurologic events, neurophysiologic abnormalities, and longevity in 36 Niemann-Pick type C patients revealed two clinical subgroups with five stages of severity within each group. Patients with a preschool onset (group I; n = 18) had a higher mortality than did patients with a school-age onset (group II; n = 18). An asymptomatic phase (stage 0) was defined by biochemical and histopathologic evidence of disease. The initial manifestations of stage 1 were a movement disorder (group I) and cognitive difficulties (group II) accompanied by impaired vertical saccadic eye movements and abnormal acoustic reflexes. Stage 2 was characterized by the sequential occurrence of vertical supranuclear gaze palsy (VSGP), cognitive difficulties, and dysarthria in group I and a movement disorder, VSGP, and dysarthria in group II. Pyramidal tract signs and abnormal brainstem auditory evoked responses defined stage 3 in both groups. Stage 4 culminated in a nonambulant, vegetative state.
journal_name
Neurologyjournal_title
Neurologyauthors
Higgins JJ,Patterson MC,Dambrosia JM,Pikus AT,Pentchev PG,Sato S,Brady RO,Barton NWdoi
10.1212/wnl.42.12.2286subject
Has Abstractpub_date
1992-12-01 00:00:00pages
2286-90issue
12eissn
0028-3878issn
1526-632Xjournal_volume
42pub_type
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