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Published on: June 9, 2021
Hirayama disease in Austria.
Josef Finsterer1, Wolfgang Löscher, Julia Wanschitz
1Krankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria.
Joint Bone Spine
|December 15, 2012
Summary
Hirayama disease (HD), a rare cervical myelopathy, affects upper limbs. Austrian cases show similar clinical features, predominantly in males, to international findings, with physiotherapy showing potential benefits.
Area of Science:
- Neurology
- Neuroscience
- Clinical Medicine
Background:
- Hirayama disease (HD) is a rare segmental cervical myelopathy impacting C7-D1 myotomes, characterized by unilateral or asymmetric upper limb weakness and wasting.
- Understanding the geographical distribution and clinical spectrum of HD is crucial for diagnosis and management.
Purpose of the Study:
- To systematically collect and analyze Hirayama disease cases in Austria.
- To describe the clinical and instrumental findings of Austrian HD patients.
- To compare these findings with international cases of Hirayama disease.
Main Methods:
- Systematic data collection from Austrian neurological departments and outpatient units.
- Standardized data collection on clinical presentation, disease course, and diagnostic investigations.
- Comparison of Austrian cases with reported international data.
Main Results:
- Nine unrelated Austrian cases of Hirayama disease were identified, with a mean age at onset of 18.3 years and a female-to-male ratio of 0.29.
- All patients exhibited distal upper limb weakness/wasting; five had unilateral, and four had bilateral involvement.
- EMG confirmed chronic neurogenic changes; cervical MRI findings varied, with dynamic MRI revealing cord compression in one case. Physiotherapy showed benefit in one patient.
Conclusions:
- Hirayama disease, though rare, occurs in Austria and affects predominantly males.
- The clinical presentation, disease progression, and outcomes in Austrian patients align with those reported globally.
- Further research into the specific mechanisms and optimal management of Hirayama disease is warranted.
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