Open AccessA Case Report of Reverse Split-hand Syndrome: An Overlooked Clinical Sign of Hirayama Disease
Author(s) -
Sujan Kumar Saha,
Hamidur Rahman,
Md. Rafiqul Islam,
Shamsul Alam,
Md Abdullah Al Muzahid,
Ashish Chowdhury,
Md. Rakibul Hasan,
Uttam Kumar Roy,
Md Suman Kabir
Publication year - 2019
Publication title -
bangladesh journal of neuroscience
Language(s) - English
Resource type - Journals
eISSN - 2408-8382
pISSN - 1023-4853
DOI - 10.3329/bjn.v35i2.57638
Subject(s) - atrophy , medicine , amyotrophic lateral sclerosis , anterior horn cell , myelopathy , weakness , posterior cortical atrophy , anatomy , posterior column , disease , spinal cord , pathology , dementia , psychiatry , acetabulum
Hirayama disease (HD) is considered to be a relatively benign, slowly progressive and less disabling rare neurological disorder where flexion induced compressive ischemic lower cervical myelopathy causes selective anterior horn cell injury resulting weakness and atrophy of distal upper limb without any pyramidal, spinothalamic and posterior column disturbance. Herein, we report a young male with clinical and imaging features suggestive of Hirayama disease presented with dissociated hand muscle atrophy (hypothenar more affected than thenar) in both hands. This less recognized finding was previously termed as reverse split-hand syndrome just opposite to split-hand syndrome found in amyotrophic lateral sclerosis. We also observe electrophysiological correlation of reverse split-hand syndrome in HD.
Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 110-114