Hyperreflexic Guillain-Barré syndrome

Neeraj N. Baheti, Davis Manuel, Pranav D. Shinde, Ashalatha Radhakrishnan, and Muraleedharan Nair
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Abstract
Guillain-Barré syndrome (GBS) is an acquired acute autoimmune polyradiculoneuropathy. The 2 features considered essential for the diagnosis of GBS are progressive motor weakness and areflexia. There have been several descriptions of reflex preservation and hyperreflexia in axonal variant of GBS in Chinese, Japanese, and European populations but it is not common in the Indian subcontinent. We report 2 such cases discussing the pathophysiology and management aspects. This case report is to impress upon treating physicians and neurologists in training that a hyperreflexic variant of GBS albeit rare, should not be missed in a given clinical setting.

Keywords: AMAN, autoimmune polyradiculoneuropathy, Guillain-Barré syndrome
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Introduction
Guillain-Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy, presenting as areflexic, flaccid paralysis with variable sensory disturbances, and elevated cerebrospinal fluid (CSF) protein without pleocytosis.[1] The 2 features essential for a diagnosis of GBS are progressive motor weakness and areflexia. There are 2 distinctive pathologic subtypes of GBS: demyelinating and axonal. Recently, there have been several descriptions of reflex preservation and hyperreflexia in axonal GBS in Chinese, Japanese, and European populations.[1,2] Although this variant is not common in the Indian subcontinent, a few cases have been reported.[3,4] A high index of suspicion is needed to diagnose this rare presentation of GBS. We report 2 such cases discussing the pathophysiology and management aspects.

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Case Reports
Case 1

A 50-year-old male was admitted with fever with abdominal pain and diarrhea lasting 2 days, which improved with symptomatic treatment. Over the next 4 days, he had progressive weakness of limbs and he needed 1 person’s support to walk. There were no cranial nerve, sensory, or bladder symptoms. There was no muscle pain. There was no other antecedent illness or recent vaccination. On examination, limbs were flaccid, and power of the proximal muscles of the lower limb was 3/5 (Medical Research Council grading) and distal muscles was 2/5. In the distal upper limb muscles, power was 3/5. There was mild truncal weakness. Deep tendon reflexes were brisk throughout the course of illness and plantars were flexor bilaterally. Sensory and cerebellar system was normal. Investigations showed normal total and differential leukocyte counts, erythrocyte sedimentation rate (9 mm/h), and creatine kinase and electrolytes (Na, Ca, K, Mg, phosphate). Vasculitis work-up was negative. CSF examination showed 2 cells (100% lymphocytes). CSF sugar was 74 mg/dL (plasma glucose 121 mg/dL) and protein was 68 mg/dL. Magnetic resonance imaging of brain and spine were normal. Nerve conduction study [Table 1] was suggestive of pure motor axonopathic variant of GBS (acute motor axonal neuropathy [AMAN]). He was treated with intravenous immunoglobulin (IVIg) (400 mg/kg/day) for 5 days and he improved. At discharge 2 weeks later, he had a significant improvement and upper limb power was normal and lower limb power was 4/5 proximally and 3/5 distally.

Table 1

Table showing nerve conduction parameters of the 2 patients
Case 1 (Day 4 of illness) Median N
Ulnar N
Tibial N
Peroneal N
Sural N
Rt Lt Rt Lt Rt Lt Rt Lt Rt Lt
CMAP latency (ms) 3.0 3.2 2.7 2.7 5.0 4.2 3.9 5.0 – –
CMAP amplitude (mV) 5.1 6.6 3.0* 5.8 1.2* 0.6* 1.1* 0.7* – –
MCV (m/s) 59.0 59.0 63.6 62.2 45.1 50.6 45.3 51.4 – –
Sensory onset latency (ms) 2.4 2.3 2.2 1.9 – – – – 1.9 2.2
Sensory peak latency (ms) 3.1
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