Subacute onset fluctuating sensorium, mimetic facial palsy and vertical supranuclearophthalmoplegia in an young female
A twenty eight year old female delivered a baby 5 months before presentation. She started experiencing repeated episodes of blackouts in front of her eyes, since two weeks. These consisted of brief spells of diminishing of vision and inability to comprehend the surroundings lasting for a few seconds and passed off completely. She did not have falls or accidents due to these episodes. Along with these episodes, she developed a dull headache, lasting few hours every day, bilateral, frontal and was not associated with nausea or vomiting. She was able to continue her daily activities in spite of the headaches. The problem increased further when her relative noticed her to have a generalized tonic clonic convulsion, frothing at mouth and loss of consciousness lasting for 8-10 minutes. She remained confused for the next few hours and returned to normal. Her behavior gradually changed and she spoke only few words, low in volume without associated emotions. She would remain silent most of the day. She became slow in her routine activities. She found it difficult in maintaining her balance in sitting position, and would sway backwards whist sitting and standing. Her relatives noticed that when she smiled, her mouth deviated to left side. Gradually she became sleepier in daytime. Over these days she became further slow and eventually became bedbound. She could not stand without support. It was noticed that she had difficulty in holding objects with her right hand. She passed urine in clothes. Curiously, her relatives noticed fluctuation in her alertness in 3-4 days before coming to the hospital. During these days sometimes she used to be normal intermittently responding to verbal interactions, speaking few words and again after some time she would become drowsy. Relatives retrospectively felt that her disease could have begun earlier with some slowing and lack of interest which was put down to the puerperal setting and tiredness. There was no history of dysphagia, nasal regurgitation, fever or neck pain. There was no history of sensory complaints in the limbs, nor any visual impairment. She had not had any joint pains/recurrent abortions/deep venous thrombosis. There was no history of seizures or recurrent headache in past.\nPatient was afebrile with pulse 80/min regular and blood pressure of 124/80mmHg. There was no significant postural fall of blood pressure. There were no signs of meningeal irritation. She had no pallor, clubbing, cyanosis, lymphadenopathy, edema or pain in the lower limbs.\nWide fluctuations of level of consciousness were noted while she was in the wards. At times she would be conscious and responded appropriately to verbal interactions, with some impaired attention. At other time, often only few hours apart, she would become unresponsive, only opening her eyes to painful stimuli. These episodes of intermittent drowsiness continued for 2-3 days and then she became unresponsive. At that time her Glasgow Coma Scale was 10.\nExamination in the early stages before the persistent unresponsiveness developed, showed her speech output to be reduced with hypophonic, monotonous melody. Higher mental status could not be analyzed satisfactorily at any time due to her impaired attention, even when she was at her best. Pupils were bilaterally normal, equally reacting to light. External ocular movements were restricted in vertical gaze, upward more than downward, with preserved vestibulo ocular reflex suggestive of supranuclearophthalmoplegia.\nOn motor system examination she had right sided pronator drift, brisk reflexes in both upper limbs (right more than left) and extensor Babinski sign. Power examination was not possible in detail but still showed right more than left sided pyramidal pattern of weakness. Sensory examination was difficult to comment upon.\nHer complete blood count, liver function tests and kidney function tests were normal. Also her repeated electrolyte examinations during ward stay were normal. Her HIV, HBsAg and VDRL were non reactive. Her CSF examination was showing no cells with proteins of 26mg% and sugar 48mg% with normal opening pressure. MRI was done [].