Posterior reversible encephalopathy syndrome following disulfiram intoxication

Abstract

A 43-year-old man with a history of chronic alcohol abuse (8 beers per day for the last 6 months) was prescribed 1.2 g disulfiram a day by his general practitioner 3 weeks before hospital admission. He continued drinking alcohol during this period, despite abundant vomiting. Soon after starting disulfiram, he developed behavioral disorder. He lost 10 kg. He was then admitted to the emergency room. The patient was apyretic, with a blood pressure of 150/100 mmHg. He was disoriented to place and time, had retrograde amnesia for the past few days, paranoid delirium, and visual hallucinations. Neurological examination disclosed paretic dysarthria, bilateral horizontal gaze-evoked nystagmus, ataxic gait, and limb dysmetria. Two days later, with improved collaboration, proximal paraparesis and areflexia were found. Over the next 10 days, the paresis progressed to the distal lower limbs, upper limbs, and oropharyngeal muscles, with apparition of facial diplegia, and dysphagia. The weakness was associated with sensitive loss prevailing at extremities with intense dysesthesias and burning pain. During the first 3 days, blood pressure values remained between 130/80 and 150/100 mmHg. Then blood pressure increased to a maximum of 180/120 mmHg, returning to initial values after a few days with treatment by perindopril. Biology showed macrocytosis (MCV: 105 10 m (N \ 99.2), slightly abnormal hepatic enzymes (GPT : 75 IU/l (N \ 45) and GOT : 36 IU/l (N \ 35))], normal chemistry, C-reactive protein, ammoniemia, ethanolemia, B12 vitamin, and folate. Vitamin B1 dosage and transketolase activity were not performed due to local constraints. Brain CT scan on admission was normal. Brain MRI revealed bilateral cortical and subcortical parieto-occipital hyperintensities on T2-weighted and fluid-attenuated inversion-recovery (FLAIR) images (Fig. 1a). Normal signals were observed on the diffusion-weighted images (DWI); no contrast enhancement was observed. EEG showed diffuse background slowing. CSF analysis was normal. Urinary excretion of porphobilinogen and deltaaminolevulinic acid was normal. Antigangliosides antibodies and HIV serology were negative. Nerve conduction studies at onset of paraparesia and 1 week later disclosed rapidly progressing axonal polyneuropathy. A Wernicke–Korsakoff’s syndrome was first considered in the emergency room. High doses of thiamine (1.5 g/day) were administrated for 5 days. The radiological pattern was compatible with posterior reversible encephalopathy syndrome (PRES). This unexpected discovery coupled with the progressive cranial nerve involvement and tetraparesis prompted us to reconsider our diagnosis. Finally, the diagnosis of PRES and acute polyradiculoneuropathy secondary to disulfiram intoxication was made. We could not rule out a potential contributing role from previous chronic alcoholism and the possibility of an associated vitamin B1 deficiency. Disulfiram was discontinued and neurological rehabilitation was proposed. Facial and oropharyngeal paresis rapidly improved followed by improvement in the upper limb paresis. Cognition returned to normal. A brain MRI 14 days after symptom onset showed almost complete disappearance of PRES lesions (Fig. 1b). Lower limb paresis improved only very slowly during the following months. S. Coppens (&) G. Naeije N. Mavroudakis Department of Neurology, Hopital Erasme, ULB, Route de Lennik 808, 1070 Brussels, Belgium e-mail: sacoppen@ulb.ac.be