Patient Mr. R, a 43-years-old illiterate, married, male, and belonging to lower SES presented with altered behavior, visual hallucinations, jerky movements of face and arm, cognitive decline, ataxic gait, and multiple falls associated with physical injuries. His symptoms started approximately 5 months prior admission to our hospital. Facio-brachial dystonic seizures with fluctuating mental status and progressive cognitive impairment (particularly verbal and visiospatial domains) dominated the clinical picture.
Patient was diagnosed as a case of myoclonus at an outside hospital and anti-epileptics were prescribed. Despite strict adherence to AEDs regimen, there was deterioration in patient’s symptomatology and he also developed allergic reaction to AEDs (carbamazepine and levetiracetam) resulting in treatment discontinuation.
On admission, he had regular pulse, was afebrile, and found to be hypertensive. Pupils were normal and fundus examination revealed clear media and pink optic disk with clear margins. Facio-brachial dystonic seizures with impaired awareness occurred at frequency of 40–50 episodes/h. Attention was arousable, but he had difficulty concentrating; he was not oriented to time, place, and person and scored 15/30 on MMSE.
His gait was mildly ataxic with patient often requiring assistance from family members. Normal muscle tone and bulk with elicitable deep tendon reflexes was noted in all four limbs with bilaterally flexor plantar. He had no other neurological deficits, and the remainder of his examination was unremarkable.
Blood counts and standard serum chemistry were within normal limits. Serum sample tested strongly positive for LGI-1 (leucine rich glioma inactivated-1) antibodies by immunofluorescence method at NIMHANS, Bangalore, India (one of the few government centers having this facility at an affordable cost). X-ray of left wrist (anteroposterior and lateral) revealed fractured distal end of radius. MRI brain, EEG, and CSF analysis were otherwise unremarkable. Paraneoplastic work up to rule out any underlying malignancy was unyielding.
Patient is managed at our center as he is very poor and refused to go any other dedicated center of neurosciences.
Patient was treated with Injection methylprednisolone (1 gm intravenous) brief pulse therapy for 7 days and then underwent four sessions of plasma exchange. Patient responded to the treatment, returned to his previous baseline function, and was shifted to oral steroid and immunosuppressant, and AEDs were stopped gradually.
Patient also developed polyphagia, polyuria, and polydipsia with low platelet counts following plasma exchange and steroid Pulse therapy. HbA1c came out to be 7% with random blood sugar in the range 300–400 mg/dl. Patient also developed sleep disturbance (insomnia), had increased self-esteem, and became more talkative than usual following oral steroid and immunosuppressant therapy. A diagnosis of comorbid treatment induced diabetes mellitus and treatment induced hypomania was made and tablet haloperidol 5 mg BD with injectable S/C insulin advised after endocrinology consultation.
Patient was discharged with complete resolution of symptoms. Patient was followed up at 1 month post-discharge through telephone consultation when family members reported relapse of symptoms and patients’ demise about a week back.