Late‐onset monomelic amyotrophy in a Caucasian woman

Abstract

We describe a 53-year-old Caucasian woman with a 19-year history of an evolving amyotrophy confined to her dominant right arm and hand. Although this atypical case of a late-onset monomelic amyotrophy in some respects mimics Hirayama disease or O'Sullivan-McLeod syndrome, it does not conform precisely with either of those disorders. We compare this individual's difficulties and clinical temporal profile to other disorders considered in the differential diagnoses with regard to her evolving clinical setting.

The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

“…However, it has also been reported in pediatric and old age groups. 10,11 The initial symptoms of HD are slowly progressive hand weakness and fatigue, followed by cold paresis, tremors, and atrophy. Asymmetric distribution of symptoms and signs is characteristic, though bilaterally symmetric forms have also been reported recently.…”

Hirayama Disease in Children From North America

“…1 The majority of reported cases are from Asia, primarily Japan, 2 and other southern and eastern Asian countries, [3][4][5] with only a few cases from Europe [6][7][8] and North America. [9][10][11][12][13] The symptoms usually start from 1 upper limb and slowly progress to involve the same segments of the contralateral upper limb. 14 Electrodiagnostic examination showing bilateral C8, T1, and C7 segmental denervation with normal sensory nerve conduction is essential in confirming the diagnosis.…”

Hirayama Disease: A Case of an Albanian Woman Clinically Stabilized Without Surgery

“…It was first described in 1959 by Hirayama and is considered a rare disease, with <1,500 cases reported (2), especially in Asian countries. Usually, it occurs in males (3,4) aged between 15 and 20 (5), but cases with a later onset (6) have also been reported in literature (7)(8)(9); so, it is important not to exclude the disease in patients with an age outside the typical range with compatible clinical examinations. The etiopathogenesis is unknown, but various hypotheses have been formulated: the most accredited one recognizes as a causal factor the bending of the neck, which involves displacement of the dural sac and, consequently, anterior crushing of spinal cord against the back of the vertebral bodies; this also explains the negative pressure that is generated in posterior venous plexus of the marrow (10) with compression of anterior epidural venous plexus and reduced drainage through the jugular veins (11).…”