Stiff limb syndrome with lower limb myoclonus: A case report.

Medicine 2019 Vol.98(49) p. e18160

Zhang CG, Li LL, Feng YY, Chen J

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Abstract

[RATIONALE] stiff limb syndrome (SLS) is a variant of stiff-man syndrome, primarily affecting a specific limb. Its diagnosis has always been challenging due to the lack of a specific confirmation test. We present a rare case of a patient with lower limb myoclonus and rigidity.

[PATIENT CONCERNS] A 53-year-old male presented with a sudden onset of progressive left lower extremity myoclonus and muscle rigidity for 3 days. He rapidly showed signs of right lower limb involvement with severe joint stiffness and inability to walk.

[DIAGNOSIS] The symptoms nature, physical examination, careful elimination of differential diagnosis suggested a diagnosis of stiff limb syndrome.

[INTERVENTIONS] Intravenous infusion of gamma globulin 0.4 mg/kg coupled with baclofen and clonazepam were given after admission. He also received an injection of botulinum toxin A to relieve his muscle stiffness.

[OUTCOMES] The patients' condition improved after the initial treatment with complete disappearance of muscle twitching. Further improvements were seen later on after the local administration of botulinum toxin A.

[LESSONS] Stiff limb syndrome shares the same complex symptoms with many other conditions. Its diagnosis relies heavily on clinical presentations and on ruling out other conditions. However, unusual symptoms such as myoclonus can occur in few cases and together with the rarity of the condition, the prevalence of misdiagnosis is high. Therefore, being aware and recognizing the signs and symptoms is crucial for proper management. Additionally, EMG is a very important test if the present condition is suspected. However, a negative EMG result or a negative anti-glutamic acid decarboxylase antibody test should not exclude SLS diagnosis.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
시술 botulinum toxin 보툴리눔독소 주사 dict 2

MeSH Terms

Baclofen; Botulinum Toxins, Type A; Diagnosis, Differential; Electromyography; Humans; Immunoglobulins, Intravenous; Lower Extremity; Male; Middle Aged; Muscle Rigidity; Myoclonus; Stiff-Person Syndrome

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