'Sand-watch' spinal cord: a case of inferior cervical spinal cord atrophy

摘要

Hirayama's disease, or juvenile muscular atrophy of the distal upper extremity (JMADUE), is a monomelic amy-otrophy that is relatively uncommon in Europe, compared with Asian countries. It typically presents with unilateral or asymmetrical distal upper limb weakness and atrophy in young patients. Electrophysiological findings are not specific to the condition, and subtle imaging signs can be challenging to recognise. A number of sinister differential diagnoses need to be carefully considered before establishing the diagnosis [1]. However, early diagnosis of Hirayama's disease and management might halt progression.The aetiology of the condition remains controversial. Detachment of the posterior dura from the subjacent lamina during a growth spurt [2], repetitive anterior horn trauma due to vigorous physical activity [3, 4], and segmental vascular mechanisms have all been implicated. Pathological characterisation of Hirayama's disease was not available until 1982 [5].