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
  • [11] Rhabdomyolysis and autoimmune variant stiff-person syndrome
    Gangadhara, Shreyas
    Gangadhara, Suhas
    Gandhy, Chetan
    Robertson, Derrick
    [J]. CLINICS AND PRACTICE, 2016, 6 (04) : 117 - 118
  • [12] Hajjioui Abderrazak, 2010, Cases J, V3, P60, DOI 10.1186/1757-1626-3-60
  • [13] Repetitive Discharge in a Case of Isaacs Syndrome with Burning Sensation
    Kurihara, Masanori
    Sugimoto, Izumi
    Hatanaka, Yuki
    Sakurai, Yasuhisa
    [J]. INTERNAL MEDICINE, 2018, 57 (17) : 2597 - 2597
  • [14] Neuromyotonia
    Maddison, Paul
    [J]. CLINICAL NEUROPHYSIOLOGY, 2006, 117 (10) : 2118 - 2127
  • [15] Antiglycine-receptor encephalomyelitis with rigidity
    Mas, Natalia
    Saiz, Albert
    Leite, Maria Isabel
    Waters, Patrick
    Baron, Manuel
    Castano, Dolores
    Sabater, Lidia
    Vincent, Angela
    Graus, Francesc
    [J]. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 2011, 82 (12) : 1399 - 1401
  • [16] Axonal neuropathy with neuromyotonia: there is a HINT
    Peeters, Kristien
    Chamova, Teodora
    Tournev, Ivailo
    Jordanova, Albena
    [J]. BRAIN, 2017, 140 : 868 - 877
  • [17] Autoimmune stiff person syndrome and related myelopathies: Understanding of electrophysiological and immunological processes
    Rakocevic, Goran
    Floeter, Mary Kay
    [J]. MUSCLE & NERVE, 2012, 45 (05) : 623 - 634
  • [18] Razmeh S, 2017, CASP J INTERN MED, V8, P329, DOI 10.22088/cjim.8.4.329
  • [19] Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports
    Sarva, Harini
    Deik, Andres
    Ullah, Aman
    Severt, William L.
    [J]. TREMOR AND OTHER HYPERKINETIC MOVEMENTS, 2016, 6
  • [20] Spinal Cord Stimulation to Abort Painful Spasms of Atypical Stiff Limb Syndrome
    Ughratdar, I.
    Sivakumar, G.
    Basu, S.
    [J]. STEREOTACTIC AND FUNCTIONAL NEUROSURGERY, 2010, 88 (03) : 183 - 186