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Year : 2022  |  Volume : 16  |  Issue : 1  |  Page : 37-40

Physiotherapeutic management of hirayama disease: A case report

1 Department of Physiotherapy, Apollo Institute of Medical Sciences and Research, Hyderabad, Telangana, India
2 Department of Physiotherapy, National Institute for Locomotor Disabilities, Kolkata, West Bengal, India
3 Department of Surgical Disciplines, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Anil Kumar Oraon
Jr. Physiotherapist, Department of Surgical Disciplines, AIIMS, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pjiap.pjiap_10_22

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Hirayama disease (HD) is a rare condition which is also known as juvenile muscular atrophy of distal upper extremities (JMADUE). The case study of a 22-year-old man is presented who reported weakness in his distal upper extremities and many of his clinical presentation, electrophysiological, and neuroimaging findings were consistent with HD. The purpose of this case study was to present a detailed assessment and evaluation of this rare disease and also to formulate a general physiotherapeutic intervention. Neurological examination demonstrated moderate-to-severe atrophy of distal muscles, preserved reflexes, and normal sensation in his both upper limbs. Electrophysiological studies revealed neurogenic changes in the muscles innervated by the lower cervical spinal cord. Magnetic resonance imaging showed atrophy of the midcervical cord with high signal intensity in the anterior horn cell region. These examination and investigation findings were compatible with the diagnosis of JMADUE also known as HD. A physiotherapeutic regimen of 6 weeks, consisting of strengthening exercises for the upper limbs and neck muscles, and the postural correction was carried out. Grip strength was measured by Jamar handheld dynamometer and functional disability by the disabilities of arm, shoulder, and hand (DASH) questionnaire. After 6 weeks of intervention, improvement in grip strength (left side 20.2 lbs to 25.6 lbs and right side 35.7 to 38.6 lbs) and many of his activities of daily life were noted along with DASH score (left side 90/125 to 76/125 and right side 55/125 to 30/125). HD is a self-limiting disorder and there is no definitive treatment. Physiotherapy helps in preventing complications resulting from immobility such as joint stiffness and muscle wasting. A general strengthening exercise program can limit the disorder and improves the quality of life.

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