Stiff limb syndrome with lower limb myoclonus A case report

被引:0
作者
Zhang, Chang-Guo [1 ]
Li, Lan-Lan [1 ]
Feng, Yao-Yao [1 ]
Chen, Jing [1 ]
机构
[1] Third Peoples Hosp Huzhou, Dept Neurol, 2088 Tiaoxi East Rd, Huzhou City, Zhejiang, Peoples R China
关键词
electromyogram; gAD-ab; stiff limb syndrome; stiff-man syndrome; PROGRESSIVE ENCEPHALOMYELITIS;
D O I
10.1097/MD.0000000000018160
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页数:3
相关论文
共 21 条
  • [1] A critical update on the immunopathogenesis of Stiff Person Syndrome
    Alexopoulos, Harry
    Dalakas, Marinos C.
    [J]. EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, 2010, 40 (11) : 1018 - 1025
  • [2] Review of 23 patients affected by the stiff man syndrome: Clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity
    Barker, RA
    Revesz, T
    Thom, M
    Marsden, CD
    Brown, P
    [J]. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1998, 65 (05) : 633 - 640
  • [3] The stiff leg syndrome
    Brown, P
    Rothwell, JC
    Marsden, CD
    [J]. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1997, 62 (01) : 31 - 37
  • [4] Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes
    Carvajal-Gonzalez, Alexander
    Leite, M. Isabel
    Waters, Patrick
    Woodhall, Mark
    Coutinho, Ester
    Balint, Bettina
    Lang, Bethan
    Pettingill, Philippa
    Carr, Aisling
    Sheerin, Una-Marie
    Press, Raomand
    Lunn, Michael P.
    Lim, Ming
    Maddison, Paul
    Meinck, H. -M.
    Vandenberghe, Wim
    Vincent, Angela
    [J]. BRAIN, 2014, 137 : 2178 - 2192
  • [5] A case report of rigidity and recurrent lower limb myoclonus: progressive encephalomyelitis rigidity and myoclonus syndrome, a chameleon
    Degeneffe, Aurelie
    Dagonnier, Marie
    D'hondt, Alain
    Elosegi, Jose Antonio
    [J]. BMC NEUROLOGY, 2018, 18
  • [6] Derksen A, 2013, BMJ CASE REP, V2013
  • [7] Inhibition of γ-aminobutyric acid synthesis by glutamic acid decarboxylase autoantibodies in stiff-man syndrome
    Dinkel, K
    Meinck, HM
    Jury, KM
    Karges, W
    Richter, W
    [J]. ANNALS OF NEUROLOGY, 1998, 44 (02) : 194 - 201
  • [8] Ehler Edvard, 2011, Acta Medica (Hradec Kralove), V54, P170
  • [9] Esplin NE, 2017, CASE REP NEUROL MED, V2017, DOI 10.1155/2017/2580620
  • [10] Progressive encephalomyelitis with rigidity responsive to plasmapheresis and immunosuppression
    Fogan, L
    [J]. ANNALS OF NEUROLOGY, 1996, 40 (03) : 451 - 453