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|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 4 | Page : 1089-1090
A Case of Encephalitis, Refractory Seizures and Ataxia
Laxmi Khanna, Nandini Agrawal
Department of Neurophysiology, Sir Ganga Ram Hospital, New Delhi, India
|Date of Submission||26-May-2020|
|Date of Decision||20-Jul-2020|
|Date of Acceptance||08-Jul-2020|
|Date of Web Publication||2-Sep-2021|
DNB Neuro, SCE Neurology (UK), Consultant Neurologist and Neurophysiologist, Department of Neurophysiology, Sir Ganga Ram Hospital, New Delhi - 110 060
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Khanna L, Agrawal N. A Case of Encephalitis, Refractory Seizures and Ataxia. Neurol India 2021;69:1089-90
Antiglutamic acid decarboxylase antibodies have been implicated in type I diabetes mellitus, polyendocrine autoimmune syndromes and stiff person syndrome. In recent years, nonparaneoplastic limbic encephalitis, cerebellar ataxia, and refractory temporal lobe epilepsy have been included in the armamentarium of neurological disorders.
A 54-year-old lady, working in a high-profile job developed left focal seizures with impaired awareness. There was a change in behavior with agitation, delusions of persecution, auditory hallucinations and seizures. There was no past history of diabetes mellitus or significant illnesses. There was no history of alcohol, tobacco, or drug abuse. On examination, she had clouding of consciousness with disorientation to time, place, and person. Attention, concentration, judgment and memory were impaired. Cranial nerve examination was normal. Tone was increased in all four limbs with hyperreflexia and bilateral extensor plantars. She had cerebellar signs with gait ataxia. Electroencephalogram showed right fronto-temporal epileptogenic focii. Cerebrospinal fluid routine examination was normal except for glutamic acid decarboxylase antibodies in the titre of 1012.97 IU/ml and serum antiglutamic acid decarboxylase antibodies of 965.20 IU/ml. Paraneoplastic panel was negative. MRI brain imaging revealed cerebral, hippocampal, and periventricular white matter atrophy. PET scan [Figure 1] shows inhomogeneous areas of increased FDG uptake in (1) basal ganglia (2) cerebellum and vermis. [Figure 2] reveals an encephalitis involving (1) bilateral cerebral hemispheres. Whole body PET was normal. She received therapy with intravenous immunoglobulin 0.4 gm/kg for 5 days every month for 6 months followed intravenous Rituximab. Eight months later, there was an improvement in the neurological functions. However, neuropsychological testing revealed a recent memory impairment with a dysexecutive syndrome.
|Figure 1: Increased FDG uptake in the (1) basal ganglia. (2) cerebellum. This is an original PET CT scan from Sir Ganga Ram Hospital, New Delhi|
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|Figure 2: (x) Encephalitis involving bilateral cerebral hemispheres. This is an original PET CT scan from Sir Ganga Ram Hospital, New Delhi|
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Glutamic acid decarboxylase antibody syndrome is caused by high levels of antibodies that block the conversion of glutamate to gamma aminobutyric acid resulting in neuronal excitation. Glutamic acid decarboxylase antibodies cause organ specific immune disorders by targeting cytotoxic T-cell-mediated responses in the brain, pancreas, thyroid, and the peripheral nerves. Pathogenic intracellular antigens target the CA1 neurons and the hippocampal dentate gyrus leading to hippocampal sclerosis, memory impairment, and white matter changes in the limbic system.
According to Graus et al., limbic encephalitis is diagnosed when there is subacute onset of working memory deficits, unexplained seizures, psychiatric manifestations, or an MRI showing bilateral FLAIR/T2 W changes in the medial temporal lobes., In recent years, FDG-PET imaging has been used as a biomarker of suspected limbic encephalitis as it is more sensitive than MRI to detect early changes in bilateral temporal lobes.
Glutamic acid decarboxylase mediated encephalitis can present with treatment resistant localization related epilepsy, refractory temporal lobe seizures, status epilepticus, cerebellar ataxia, behavioral and cognitive problems. Patients respond to immunotherapy with intravenous immunoglobulin with or without Rituximab. This case report highlights the importance of suspecting glutamic acid decarboxylase antibody-mediated encephalitis in unexplained psychosis, refractory seizures, and limbic encephalitis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Chairperson of Department of Neurophysiology Sir Ganga Ram Hospital, New Delhi – 92.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]