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Published on: November 21, 2013
Unveiling Multiple Sclerosis: Acute Hemichorea Masquerading as Sydenham's Chorea
Jayaram Saibaba1, Gopinath Karuppiah2, Amiya Ranjan Nayak2
1Junior Resident, Department of General Medicine, JIPMER, Puducherry, India, Corresponding Author.
Background:
Multiple sclerosis (MS) is a demyelinating disorder of the central nervous system characterized by the dissemination of lesions in time and space. Although tremors are the most commonly reported movement disorder in MS, less frequent manifestations such as paroxysmal tonic spasms, dystonia, chorea, ballism, myoclonus, myokymia, restless leg syndrome, and hemifacial spasms can also occur. Chorea and ballism are particularly rare in MS.
Case Presentation:
We report the case of a 20-year-old female who presented with a 1-month history of unsteady gait and involuntary movements of the right upper limb. Neurological examination revealed slight deviation of the angle of the mouth, hypertonia of both lower limbs, brisk knee reflexes, and involuntary, rapid, irregular, semi-purposeful movements of the right upper limb. The initial suspicion was Sydenham's chorea due to prolongation of PR interval (220 ms) and the presentation of choreiform movements. However, extensive workup including normal acute rheumatic fever markers, normal metabolic and infectious profiles, and negative autoimmune screenings did not support this diagnosis. Cerebrospinal fluid (CSF) analysis revealed positive oligoclonal bands, and Magnetic resonance imaging (MRI) showed multiple demyelinating plaques in characteristic locations for MS. The patient was diagnosed with MS and treated with steroids and valproate, followed by azathioprine. At 3-month follow-up, the patient showed significant improvement in motor functions, regaining the ability to walk and perform daily activities independently.
Conclusion:
Acute unilateral dyskinesia in young patients, especially when other common etiologies are ruled out, should prompt consideration of MS. Detailed evaluation, including neuroimaging and CSF analysis, is crucial for accurate diagnosis and appropriate management.
Insights
Multiple sclerosis (MS) can present with rare movement disorders like chorea. Early diagnosis through neuroimaging and CSF analysis is key for effective treatment and recovery.
Area of Science:
- Neurology
- Neuroimmunology
Background:
- Multiple sclerosis (MS) is a central nervous system demyelinating disorder.
- While tremors are common, rare movement disorders like chorea can occur in MS.
- Chorea and ballism are exceptionally infrequent manifestations of MS.
Purpose of the Study:
- To report a rare case of choreiform movements as an initial presentation of Multiple Sclerosis (MS).
- To highlight the importance of considering MS in young patients with acute unilateral dyskinesia when other causes are excluded.
Main Methods:
- Case report of a 20-year-old female with involuntary movements.
- Diagnostic workup included neurological examination, blood tests, cerebrospinal fluid (CSF) analysis, and Magnetic Resonance Imaging (MRI).
- Treatment involved steroids, valproate, and azathioprine.
Main Results:
- Patient presented with choreiform movements, initially suspected as Sydenham's chorea.
- Extensive workup ruled out rheumatic fever, infections, and autoimmune conditions.
- CSF analysis revealed oligoclonal bands, and MRI showed demyelinating plaques, confirming MS diagnosis.
- Significant motor function improvement was observed after 3 months of treatment.
Conclusions:
- Acute unilateral dyskinesia in young individuals warrants consideration for MS, especially after excluding common causes.
- Comprehensive neurological evaluation, including MRI and CSF analysis, is vital for diagnosing MS.
- Prompt diagnosis and management lead to significant recovery of motor functions.
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