An acute disseminated encephalomyelitis-like illness following alcohol withdrawal
Authors: Trip SA , Naryanaswamy HM , Wroe SJ 
Published: May 2014

A44-year-old man had a 1-year history of excessive alcohol consumption of three or more bottles of spirits per week. He decided to abruptly abstain from alcohol on holiday and, after approximately 24 hours, developed a generalized tonic clonic seizure. He was admitted to hospital in Greece where he was confused, disorientated, and had further seizures. Visual hallucinations and delusions developed. Investigations showed an elevated creatine kinase (CK) secondary to the seizure, raised gamma glutaryl transferase at 404 u/litre, a macrocytic anaemia with a mean corpuscular volume of 105.2 fl, and a thrombocytopenia with a platelet count of 86 × 109/litre. Serum sodium was normal at 139 mmol/litre. A computed tomography (CT) scan of the brain showed an enlargement of the subarachnoid spaces bilaterally in the frontal lobe, but no intracranial haemorrhage was seen. He was treated with benzodiazepines, thiamine, vitamin B compound preparation, and conservative intravenous fluids with 1 litre of dextrose per day. He was then transferred back to the UK after 2 weeks. As examination of the nervous system by a neurologist was normal, and there was no evidence of an active psychosis, he was discharged home.

He was admitted 5 days later with a 2-day history of weakness of the legs, unsteadiness and falls. He was able to give a clear history on admission, was alert and orientated, with no evidence of cognitive or psychiatric disturbance. Examination revealed a marked cerebellar dysarthria and no dysphasia. Optic discs were slightly pale but there was no papilloedema. Eye movements were grossly abnormal with square wave jerks, ocular bobbing in the primary position, jerky pursuit movement and gaze-evoked, almost pendular, nystagmus in the vertical and horizontal planes. Bilateral internuclear ophthalmoplegia was present, which was worse on the left. In the upper limbs he had downward drift of the right arm, grade 4 pyramidal weakness, poor fine finger movements and severe finger–nose ataxia, which was worse on the left. In the lower limbs he had a grade 2 flaccid paraparesis with areflexia, and no plantar response. General examination revealed a palpable bladder as a result of urinary retention. Urea, electrolytes and calcium were normal. Liver function tests were deranged. C-reactive protein was 40 mg/litre and erythrocyte sedimentation rate was 81 mm/hr. Full blood count revealed macrocytosis with a raised platelet count at 732 × 109/litre. Serum angiotensin-converting enzyme, CK, rheumatoid factor, anti-nuclear antibodies, anti-neutrophil cytoplasmic antibody, hepatitis B and C serology, toxoplasma serology, varicella zoster and herpes simplex immunoglobulin G were all normal or negative. CSF glucose was 3.7 mmol/litre (serum 7.4 mmol/litre), CSF protein was 1.36 g/litre, less than one polymorph, less than one red blood cell and five lymphocytes × 106/litre. No growth of any organisms occurred and polymerase chain reaction for herpes simplex, varicella zoster and enterovirus was negative. Oligoclonal bands were absent. Chest X-ray was normal. CT scan of the brain was normal. A magnetic resonance imaging scan of the brain revealed abnormal high T2 signal in the medulla, pons, left middle cerebral peduncle, mid brain and in the white matter of the right and to a lesser extent the left cerebral hemisphere (Figure 1). Moderate prominence of the ventricular and extra-ventricular CSF spaces was also seen. In the spinal cord there was a high T2 lesion in the cervical cord centred at C3/4 with further extensive skip lesions at the thoracic cord (Figure 2). Repeat imaging after 2 months showed less extensive brainstem white matter changes. Similarly, in the spinal cord the skip lesions showed modest resolution. The changes were thought to be more compatible with demyelination seen in acute disseminated encephalomyelitis rather than central pontine myelinolysis where symmetrical, central changes are seen.

Visual evoked potentials showed bilateral delay. Electroencephalogram showed background fast activity thought to be related to the benzodiazepines which later returned to normal.

He deteriorated with bulbar failure and aspiration pneumonia requiring intubation and ventilation. He received intravenous methyl prednisolone. He made a gradual recovery over 3 months with complete resolution of the brainstem syndrome. Bladder symptoms and a very mild paraparesis persisted.