Stiff-person syndrome coexisting with critical illness polyneuropathy: A case report

• Published in: Medicine (Baltimore) 2020-12, Vol.99 (50), p.e23607-e23607
• Main Authors: Cai, Qiong, Wu, Chao, Xu, Wenxiao, Liang, Yinxing, Liao, Songjie
• Format: Article
• Language: English
• Subjects: Baclofen - administration & dosage; Baclofen - therapeutic use; Clinical Case Report; Clonazepam - administration & dosage; Clonazepam - therapeutic use; Diagnosis, Differential; Electromyography; Humans; Immunoglobulins, Intravenous - administration & dosage; Immunoglobulins, Intravenous - therapeutic use; Male; Middle Aged; Muscle Relaxants, Central - administration & dosage; Muscle Relaxants, Central - therapeutic use; Polyneuropathies - complications; Polyneuropathies - diagnosis; Respiratory Insufficiency - etiology; Stiff-Person Syndrome - complications; Stiff-Person Syndrome - diagnosis
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000023607

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. 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). 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. 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. On follow-up, the patient reported complete relief of his pain and rigidity. 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.