Hirayama disease (HD)/cervical flexion-induced myelopathy (CFIM) is a lower motor neuron disease conventionally affecting a single upper extremity. We describe three men progressing after a long stable period to develop severe spastic paraparesis and bladder disturbances as a protracted implication of HD. The age at onset was 20, 24, and 15 years, while the age at presentation was 27, 41, and 57 years, respectively. The second phase of disease progression occurred after 4, 13, and 28 years of stationary period. All had CFIM with characteristic magnetic resonance imaging features as observed during progressive stages. The anterior dural shift extended variably from C4 to D4 levels with a median value of 5 mm and was maximum at C6 to C7 levels, pushing the cord anteriorly causing compression. This study emphasizes the need to recognize this unusual subgroup of HD and mandates long-term follow-up with timely intervention in arresting the progression/improving the deficits.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.4103/0028-3886.236966 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
McArdle sign and neck flexion-induced change in central motor conduction in multiple sclerosis.
Mult Scler
December 2024
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Article Synopsis
- * Researchers measured central motor conduction time (CMCT) in different groups, including MS patients with and without the McArdle sign, other myelopathy patients, and healthy controls.
- * Results showed that MS patients with a prominent McArdle sign displayed significantly prolonged CMCT during neck flexion, suggesting that the sign may be linked to nerve conduction slowing due to demyelination.
Phenotypic, Electrophysiologic, and Imaging Spectrum of Hirayama Disease from Northern India.
Ann Indian Acad Neurol
September 2024
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Article Synopsis
- Cervical flexion-induced myelopathy, or Hirayama disease (HD), primarily affects young males aged about 17.8 years, leading to asymmetric weakness and wasting in distal upper limbs, with future emphasis on early diagnosis and treatment.
- Retrospective analysis of 140 patients showed that nearly half had unilateral symptoms, and most had ongoing progression of the disease; abnormal electromyography findings were common, with significant MRI changes noted.
- Early identification and management of HD are crucial to prevent severe disability, emphasizing the need for dynamic MRI and aggressive treatment strategies in affected individuals.
Monomelic Amyotrophy/Hirayama Disease: Surgical Outcome in a Large Cohort of Indian Patients.
World Neurosurg
March 2024
Department of Neurology, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India. Electronic address:
Background: Hirayama disease (HD) is a cervical compressive myelopathy. Anterior cervical discectomy and fusion (ACDF) is identified as the best surgical approach. We evaluated surgical outcomes and factors influencing ACDF in HD.
View Article and Find Full Text PDFMcArdle Sign: A Specific Sign of Multiple Sclerosis.
Mayo Clin Proc
August 2019
Department of Neurology, Mayo Clinic, Rochester, MN. Electronic address:
Objective: To measure McArdle sign (rapidly reversible weakness induced by neck flexion) both qualitatively and quantitatively and to evaluate its specificity and clinical utility for diagnosis of multiple sclerosis (MS).
Patients And Methods: In this prospective study, McArdle sign was evaluated by a technician blinded to diagnosis by measuring changes in finger extensor strength in successive trials of neck extension and flexion, first clinically and then with a torque measurement device. We studied 25 healthy controls and 81 patients with finger extensor weakness.
Flexion-Induced Cervical Cord Compression: Hirayama Disease.
Balkan Med J
May 2019
Department of Physical Medicine and Rehabilitation, Medipol University School of Medicine, İstanbul, Turkey
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!