Abstract：
:Krabbe's disease is an autosomal recessive leukodystrophy characterized by a lack of galactocerebroside beta-galactosidase activity. In contrast to the classic early infantile-onset form of Krabbe's disease, less recognized, late-onset variants exist. The authors present a case of late juvenile-onset Krabbe's disease, including the associated magnetic resonance imaging (MRI) findings. Most patients with late-onset Krabbe's disease present with visual loss due to optic atrophy. Associated gait abnormalities and parental consanguinity should increase the clinician's suspicion that a child may have late-onset Krabbe's disease. Because of the prolonged survival in late-onset Krabbe's disease, the recent development of bone marrow transplantation for these patient makes diagnosis of this disorder particularly important.
journal_name
Ophthalmologyjournal_title
Ophthalmologyauthors
Baker RH,Trautmann JC,Younge BR,Nelson KD,Zimmerman Ddoi
10.1016/s0161-6420(90)32439-9subject
Has Abstractpub_date
1990-09-01 00:00:00pages
1176-80issue
9eissn
0161-6420issn
1549-4713pii
S0161-6420(90)32439-9journal_volume
97pub_type
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