Description

A woman in her early 40s with no significant medical history presented with acute ascending bilateral lower limb weakness and tingling over four days, associated with worsening hoarseness of voice and dysphagia. These symptoms were preceded by diarrhoea two weeks prior to presentation. Neurological examination revealed generalised areflexia with weakness throughout bilateral upper and lower limbs - power 3/5 on the Medical Research Council (MRC) grading scale. She was also found to have a glove and stocking pattern of numbness with reduced pinprick sensation distally up to her upper arms and thighs. Nerve conduction studies revealed demyelinating polyneuropathy, and cerebrospinal fluid showed cyto-albuminologic dissociation, consistent with Guillain-Barre syndrome (GBS). She was treated with five days of intravenous immunoglobulin (IVIG) to good effect with improvement in her limb power.

Two weeks later, she developed mild headaches, blood pressure fluctuations and sudden onset worsening left-sided weakness. Neurological examination revealed a new left facial droop with reduction in power of her left upper limb (MRC 3/5) and left lower limb (MRC 1/5). Right upper and lower limb power remained full (MRC 5/5). Urgent brain MRI showed acute right-sided deep watershed infarcts with multifocal vessel wall stenoses in the anterior and posterior circulations (figure 1A–C). She was immediately started on antithrombotics and intravenous hydration. Thereafter, investigations for cardioembolism, malignancy, infective, and autoimmune vasculitic aetiologies returned negative. She underwent intensive physical rehabilitation and was eventually discharged one month later. Repeat brain MRI performed two weeks post-stroke showed complete resolution of the multi-vessel stenoses (figure 1D–F). The clinical and radiological findings were consistent with reversible cerebral vasoconstriction syndrome (RCVS). An invasive digital subtraction angiogram was not performed due to cost constraints.

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Figure 1

Brain MRI revealed diffusion-weighted imaging (DWI) hyperintense lesions in the right centrum semiovale deep watershed regions (A) with corresponding apparent diffusion coefficient (ADC) hypointensities (B), consistent with acute infarction. Magnetic resonance angiography (MRA) showed multifocal vessel wall stenoses in the anterior and posterior circulation (C, red arrowheads). Two weeks later, there was complete resolution of the multi-vessel stenoses on repeat brain MRI (D, E) and MRA (F, red arrows).

The occurrence of acute stroke in patients with GBS is rare. Possible explanations include frequent blood pressure fluctuations from dysautonomia which may in turn affect cerebral perfusion, and a pro-thrombotic state from ongoing underlying disease inflammation. IVIG is commonly used as the first-line treatment for GBS. Known rare adverse effects associated with its use include hypercoagulability which can result in cerebral infarction,1 as well as RCVS. RCVS is an uncommon cerebrovascular condition characterised by severe headaches secondary to diffuse segmental vasoconstriction of cerebral arteries. This may result in intracranial infarction and haemorrhage leading to seizures, altered consciousness and focal neurological deficits. It can occur secondary to various medications, recreational drugs or autoimmune conditions. Only a few case reports have described the rare association of RCVS with GBS and IVIG.1 2 Postulated mechanisms include GBS-related dysautonomia which can disrupt cerebral vascular tone regulation.3 Our case report illustrates the importance of recognising RCVS as a possible cause of headache and sudden onset asymmetrical weakness in patients with GBS, especially in those treated with IVIG.