Cardiovascular and sudomotor dysfunction in Hirayama disease
1Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India. animeshdas05@gmail.com.
Acta Neurologica Belgica
|December 14, 2019
Summary
Hirayama disease affects young males, causing muscle atrophy and significant autonomic nervous system dysfunction. This study reveals both localized and systemic autonomic issues in patients, highlighting the need for thorough evaluation.
Area of Science:
- Neurology
- Autonomic Neuroscience
Background:
- Hirayama disease is a rare neurological disorder primarily affecting young males.
- It is characterized by progressive atrophy of muscles in the hand and forearm.
- Localized autonomic dysfunction, like cold extremities and excessive sweating, has been noted in some cases.
Purpose of the Study:
- To investigate the extent of both localized and systemic autonomic nervous system involvement in Hirayama disease.
- To correlate clinical symptoms with objective measures of autonomic function.
Main Methods:
- A cohort of 44 patients with Hirayama disease was studied.
- Patients underwent assessment of symptom profiles and autonomic nervous system function.
- Objective evaluation included sympathetic skin response (SSR) measurements.
Main Results:
- 88.6% of patients exhibited localized clinical autonomic dysfunction.
- 75% showed objective generalized autonomic dysfunction.
- SSR showed increased latency and decreased amplitude in upper limbs compared to controls, correlating with symptoms.
Conclusions:
- Hirayama disease presents with both localized and systemic dysautonomia.
- Subtle systemic autonomic dysfunction may be identified through careful longitudinal evaluation during the disease's progression.
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