Hirayama disease/cervical flexion-induced myelopathy progressing to spastic paraparesis: A report on three cases with literature review.

Abstract:

:Hirayama disease (HD)/cervical flexion-induced myelopathy (CFIM) is a lower motor neuron disease conventionally affecting a single upper extremity. We describe three men progressing after a long stable period to develop severe spastic paraparesis and bladder disturbances as a protracted implication of HD. The age at onset was 20, 24, and 15 years, while the age at presentation was 27, 41, and 57 years, respectively. The second phase of disease progression occurred after 4, 13, and 28 years of stationary period. All had CFIM with characteristic magnetic resonance imaging features as observed during progressive stages. The anterior dural shift extended variably from C4 to D4 levels with a median value of 5 mm and was maximum at C6 to C7 levels, pushing the cord anteriorly causing compression. This study emphasizes the need to recognize this unusual subgroup of HD and mandates long-term follow-up with timely intervention in arresting the progression/improving the deficits.

journal_name

Neurol India

journal_title

Neurology India

authors

Preethish-Kumar V,Polavarapu K,Nashi S,Bhattacharya K,Saini J,Vengalil S,Pruthi N,Bhat DI,Nalini A

doi

10.4103/0028-3886.236966

subject

Has Abstract

pub_date

2018-07-01 00:00:00

pages

1094-1099

issue

4

eissn

0028-3886

issn

1998-4022

pii

ni_2018_66_4_1094_236966

journal_volume

66

pub_type

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