A clinical study of hirayama disease in Taiwan

Abstract

The purpose of this study was to review the clinical manifestations of 40 patients who fulfilled the clinical criteria for Hirayama disease (juvenile muscular atrophy of distal upper extremities), identified in our neuromuscular clinic between February 1995 and December 2005. Of these 40 patients, 87.5% were male. The mean age at onset was 16.8 years, which was 4.5 years later than the peak age of the normal growth curve. Progressive muscle weakness and wasting were characteristic and occurred predominantly in the distal part of the right upper limb. Neurogenic symptoms ceased to progress within 5 years in most patients (92.5%). About one third of patients had participated frequently in heavy physical activity before onset of muscle symptoms. Reduced amplitude of the compound muscle action potential of the ulnar nerve was the most prominent finding in nerve conduction studies. Electromyography showed acute or chronic neurogenic changes, most frequently in muscles supplied by the C7-T1 segments. Magnetic resonance imaging showed anterior shifting of the posterior dura and engorged posterior venous plexus at the cervical level in 95% of patients. Our results support the belief that Hirayama disease is a self-limited, focal lower motor neuron disease involving the lower cervical segments. Disproportionate growth between the vertebral column and the contents of the spinal canal may be the underlying cause, and strenuous physical activity may be a precipitating factor.

Waterskier’s Hirayama syndrome

“…Huang et al [3] reported that 37.5% of their patients with Hirayama disease frequently participated in vigorous sports or physical activity for at least 6 months before the onset of symptoms. These activities included basketball, baseball, martial arts, body-building, strenuous farm work or military training.…”

Hirayama Disease

“…studies. [5][6][7][17][18][19][20][21] The degree of male preponderance reported in large series ranged from purely male to a male/female ratio of 2.8:1. 6,22,23 A delay of age of onset after the peak age of normal growth curve was observed in several studies.…”

“…[5][6][7] However, early reports in the 1960s and 1970s emphasized clinical features and electrophysiological evaluations. It was not until 1982 that the first autopsy case was obtained by Hirayama and colleagues, 8,9 which revealed anterior-posterior flattening of the lower cervical cord associated with ischemic and atrophic changes of the anterior horn cells.…”

“…6,22,23 A delay of age of onset after the peak age of normal growth curve was observed in several studies. [5][6][7] Hirayama 5 observed that the peak of onset age is approximately 2 years later than the peak of longitudinal growth curve of juveniles in Japan. Based on this finding, Hirayama 1,5 speculated that disproportional growth between the vertebral column and the contents of the spinal canal, especially the dural sac, during the juvenile growth spurt underlies this phenomenon.…”

Pattern of Moter Neuron Disease in North India - Study at Teriary Care Center

“…(7) The phenomenon of cold paresis is widely observed in the patients, characterized by exacerbating of finger weakness on exposure to cold environment. (8,9) Resting fasciculation is not observed, but contraction fasciculation is well documented. (10,11,12,) …”