‘Wine Glass’ sign in recurrent postpartum hypernatremic osmotic cerebral demyelination
A 23-year-old female presented in June 2009 twelve days after second delivery with 8 days fever followed by dysarthria with progressive quadriparesis and progressive altered sensorium. She was febrile and in altered consciousness groaning, opening eyes, and flexing limbs to pain. She had generalized hypotonia, brisk muscle stretch reflexes, and flexor plantars.\nShe had severe hypernatremia, elevated creatinine kinase (CK) and azotemia []. Cerebral MRI revealed symmetrical white matter hyperintensities in FLAIR, T2 and diffusion weighted imaging (DWI) sequences, which were hypointense in T1 images []. Quadriplegia (proximal 0/5, distal 2/5) was noted on day 3 when she became conscious with slow correction of hypernatremia. On discharge at 18 days, she was conscious, alert with emotional lability, moderate limb, and gait ataxia with mild quadriparesis (MRC 4/5), which recovered completely at 2 months.\nShe delivered her third baby 25 days before admission in May 2011. Moderate fever started 15 days later. Progressive quadriparesis began 5 days before admission followed by irrelevant speech, disorientation, progressively reducing word output, and incontinence 2 days later. She was febrile with dehydration and tachycardia. She lay with open eyes, obeyed simple commands, spoke irrelevantly, and was disoriented. Eyes were convergent. She had flaccid quadriplegia (upper limbs 2/5, lower limbs 1/5). Muscle stretch reflexes were normal with flexor plantars.\nInvestigations revealed recurrence of hypernatremia, azotemia and hyperCKemia []. MRI revealed extensive symmetrical T2, FLAIR, and DWI hyperintensities of white matter with prominent changes in internal capsule extending through midbrain and pons to middle cerebellar peduncle simulating the ‘wine-glass’ Figures appearance [Figures and ]. Auditory evoked potentials (AEP) revealed bilateral prolongation of interpeak intervals III-V and I-V while I-III intervals were normal []. Following recovery of encephalopathy and quadriplegia with gradual hypernatremia correction, she had generalized hyperreflexia, ankle clonus with limb and gait ataxia. She had diplopia from transient bilateral lateral rectus palsy. She was discharged 12 days after admission when her biochemical abnormalities had recovered, with muscle power 4+/5 and moderate ataxia, which resolved at 1 month.\nAt follow-up 7 months later, she was asymptomatic with normal neurological examination and biochemical values. Repeat MRI revealed partial resolution of the white matter hyperintensities [Figures and ] whereas AEP was normal.