PMID- 33327330
OWN - NLM
STAT- MEDLINE
DCOM- 20210106
LR  - 20221005
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 99
IP  - 50
DP  - 2020 Dec 11
TI  - Stiff-person syndrome coexisting with critical illness polyneuropathy: A case 
      report.
PG  - e23607
LID - 10.1097/MD.0000000000023607 [doi]
LID - e23607
AB  - RATIONALE: Stiff-person syndrome (SPS) is an uncommon neurological disorder with 
      autoimmune features. Here, we report a 60-year-old man with SPS associated with 
      critical illness polyneuropathy (CIP). CIP was diagnosed during an episode of 
      acute respiratory failure secondary to muscular rigidity and spasms, which has 
      rarely been reported in this condition. The overlapping of CIP and SPS 
      complicated the case. PATIENT CONCERNS: A 60-year-old man presented with gradual 
      onset of cramps, stiffness, and rigidity in his lower limbs 1 year before 
      admission, which eventually led to inability to stand and walk. The persistent 
      nature of his symptoms progressed to frequent acute episodes of dyspnea and he 
      was admitted to intensive care unit (ICU). DIAGNOSIS: SPS had been diagnosed 
      after 2 tests of electromyography (EMG) and the detection of an elevated 
      anti-GAD65 antibody titer. During the first EMG, low or absent compound muscle 
      action potentials (CMAP), and sensory nerve action potentials (SNAP) were shown. 
      Therefore, the diagnosis of SPS coexisting with CIP was made. INTERVENTIONS: 
      Symptomatic treatment was initiated with oral clonazepam (0.5 mg Bid) and 
      baclofen (5 mg Bid). Intravenous immunoglobulin (IVIG) (0.4 g/kg/d) was 
      administered for the patient for 5 days after admission. We observed a 
      significant clinical improvement during the administration period, and the 
      patient became ambulatory. OUTCOMES: On follow-up, the patient reported complete 
      relief of his pain and rigidity. LESSONS: We report this special case to address 
      the varied clinical features of SPS. Electrophysiological testing is an important 
      diagnostic approach. Accurate recognition of the disease ensures that the 
      patients can be given appropriate treatment without delay.
FAU - Cai, Qiong
AU  - Cai Q
AD  - Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, 
      Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major 
      Neurological Diseases, National Key Clinical Department and Key Discipline of 
      Neurology, Guangzhou, China.
FAU - Wu, Chao
AU  - Wu C
FAU - Xu, Wenxiao
AU  - Xu W
FAU - Liang, Yinxing
AU  - Liang Y
FAU - Liao, Songjie
AU  - Liao S
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Immunoglobulins, Intravenous)
RN  - 0 (Muscle Relaxants, Central)
RN  - 5PE9FDE8GB (Clonazepam)
RN  - H789N3FKE8 (Baclofen)
SB  - IM
MH  - Baclofen/administration & dosage/therapeutic use
MH  - Clonazepam/administration & dosage/therapeutic use
MH  - Diagnosis, Differential
MH  - Electromyography
MH  - Humans
MH  - Immunoglobulins, Intravenous/administration & dosage/therapeutic use
MH  - Male
MH  - Middle Aged
MH  - Muscle Relaxants, Central/administration & dosage/therapeutic use
MH  - Polyneuropathies/complications/*diagnosis
MH  - Respiratory Insufficiency/*etiology
MH  - Stiff-Person Syndrome/complications/*diagnosis
PMC - PMC7738057
COIS- The authors have no conflicts of interest to disclose.
EDAT- 2020/12/18 06:00
MHDA- 2021/01/07 06:00
PMCR- 2020/12/11
CRDT- 2020/12/17 01:02
PHST- 2020/12/17 01:02 [entrez]
PHST- 2020/12/18 06:00 [pubmed]
PHST- 2021/01/07 06:00 [medline]
PHST- 2020/12/11 00:00 [pmc-release]
AID - 00005792-202012110-00111 [pii]
AID - MD-D-19-09863 [pii]
AID - 10.1097/MD.0000000000023607 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2020 Dec 11;99(50):e23607. doi: 
      10.1097/MD.0000000000023607.