Facio-brachio-crural dystonic episodes and drop attacks due to leucine rich glioma inactivated 1 encephalitis in two elderly Indian women
A 60-year-old woman was admitted to our ward with recurrent falls of 1 month duration. She was a long standing diabetic of 10 years duration on insulin and oral hypoglycemic agents. Her husband had noticed that she had developed a voracious appetite, eating every 2 h; although, there was no evident weight gain. For the past 3 days, the falls had increased in frequency and now she was falling 2-3 times a day. She complained of an abnormal sensation in the epigastrium followed by sudden buckling at the knee. She usually fell backward; although, she could fall to either side. On the day of admission, she had fallen 4 times and bruised her left side of the chest and sustained lacerations over the forehead and the occipital region []. She denied any palpitations or loss of consciousness during the episodes and was able to pick herself up immediately after the falls. There was no history of tongue bite or urinary or bowel incontinence associated with these episodes. She was admitted for further evaluation of drop attacks. Prolonged EEG monitoring was normal. Recurrent clinical episodes were recorded without any EEG correlate or recordable movement. MRI brain with contrast was normal. Holter monitoring was normal. Audiometry revealed mild sensorineural hearing loss bilaterally 1 week after admission; she continued to have recurrent falls with injury. Owing to hyponatremia (serum Na of 115 meq/l) and drowsiness, she was shifted to the intensive care unit for observation. At this time, she was noticed to have frequent brief alternating facial grimacing and upper limb dystonic posturing lasting a few seconds. Each of these episodes was preceded by the typical discomfort or aura that she experienced prior to fall and a feeling of weightlessness. These episodes were now noticed to occur every time she became drowsy or woke up from sleep (approximately 30 times a day) []. She was noticed to be very confused and disoriented. Her Addenbrooke's cognitive examination (ACE) score was 68/100 with prominent deficits in attention, concentration and recall. Patient was treated with 1 g of intravenous (IV) methylprednisolone for 5 days followed by oral prednisolone 40 mg/day. She was shifted back to the ward where her husband continued to notice milder attacks with occasional posturing of the leg on the side of the attacks []. The attacks gradually declined in frequency and by day 7 after initiation of therapy, she was asymptomatic and ambulant. Routine blood evaluation, liver function test, renal function test, thyroid function test and vasculitic work-up were normal. The cerebrospinal fluid study showed 0 cells and normal sugar and protein levels. Her ACE score had improved to 82/100. Serum was sent for anti LGi1 and contact in associated protein 2 (CASPR2) antibodies to Oxford University, UK. She tested positive for LGi1 antibody and negative for CASPR2. A second simultaneous sample sent to Statens Serum Institute Copenhagen was positive for LGi1 immunoglobulin G (IgG) antibodies at a titer of 1:320 and negative for LGi1 IgA & IgM, CASPR2, ANNA-1 (Hu), ANNA-2 (Ri), PCA-1 (Yo), Tr, Myelin-associated glycoprotein (MAG) antibody, myelin, PNMA1 (Ma/Tr), glutamic acid decarboxylase antibody (GAD), Amphiphysin, Aquaporin-4 (Aqp4), glycine receptor (GlyR), Abs, GABAbR, GluR (AMPA type) glutamate receptor and (GluR) (NMDA type) antibodies.\nOwing to financial constraints, the family declined further testing. She was discharged on oral prednisolone 40 mg/day. At follow-up 45 days later, there were no further falls or dystonic episodes; however, she complained of disabling persistent amnesia and her ACE score was 80/100 with predominant recent memory impairment.\nAfter review of the second case, serum of the 1st patient was sent to Oxford University for LGi1 and CASPR2 antibodies. Serum was positive for LGi1 antibodies and negative for CASPR2 antibodies.