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|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 456-457
Bilateral isolated basal ganglia bleed: An atypical presentation of Japanese encephalitis
Rohan Mahale, Anish Mehta, Srinivasa Rangasetty
Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India
|Date of Web Publication||5-Jun-2015|
Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mahale R, Mehta A, Rangasetty S. Bilateral isolated basal ganglia bleed: An atypical presentation of Japanese encephalitis. Neurol India 2015;63:456-7
Japanese encephalitis (JE), a mosquito-borne flaviviral encephalitis, remains a major health problem in the Indian subcontinent.  Though no age is immune, children between 5 and 15 years of age happen to bear the brunt of the disease. The typical cranial imaging abnormalities in JE involve bilateral thalami, basal ganglia, substantia nigra, brainstem, cerebellum and cerebral cortical and white matter. , Although haemorrhagic lesions involving bilateral thalami have been described previously, such lesions are usually accompanied by similar lesions elsewhere in the brain. Bilateral isolated basal ganglia bleed as the sole imaging abnormality in JE has not been reported before. Here, we report the case of a child with the diagnosis of JE wherein the cranial imaging revealed bilateral isolated basal ganglia bleed.
A 5-year-old boy born out of a non-consanguineous parentage and having a normal perinatal and developmental history presented with a history of fever of 5 days duration and 4 episodes of generalised tonic-clonic seizures in 1 day. There was no regaining of consciousness in between the episodes of seizures suggesting the presence of status epilepticus. The fever was associated with headache and vomiting although no history of loose stools, cold or cough could be elicited. A diagnosis of febrile status epilepticus was made at this point. On examination, the child was febrile (100.2° F). His pulse rate was 130/min, and he had a blood pressure of 98/72 mmHg and a respiratory rate of 28/min. He was stuporous with no eye opening and minimal limb movements to painful stimulus. His fundus examination was normal. His pupils were equal but sluggishly reacting to light. The doll's eye response was preserved. The motor examination showed an increased muscle tone in all 4 limbs with brisk deep tendon reflexes and an extensor plantar response bilaterally. There was terminal neck stiffness with a positive Kernig's sign. Complete hemogram showed leucocytosis (16,000 cells/cumm) with a normal platelet count. Renal and hepatic functions including serum electrolytes were normal. Computed tomography (CT) of the brain showed bleed in the right basal ganglia. Magnetic resonance imaging (MRI) showed blooming in bilateral basal ganglia on gradient echo (GRE) sequence suggestive of bleed [Figure 1]. Cerebrospinal fluid (CSF) examination showed mildly raised protein (62 mg/dl), normal glucose with mild lymphocytic pleocytosis (28 cells per cumm) and positive IgM anti-JE antibodies. CSF polymerase chain reaction (PCR) analysis for cytomegalovirus, herpes simplex virus and Mycobacterium tuberculosis re negative. Serological testing for human immunodeficiency virus was negative. The child was started on antiepileptics and put on mechanical ventilator. The seizures were eventually controlled on two antiepileptic drugs. He was later discharged in an akinetic-mute state.
|Figure 1: CT brain showing bleed in the right basal ganglia (a); MRI brain GRE sequence shows blooming in bilateral basal ganglia on gradient echo imaging (b and c; white arrow)|
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Prognosis in patients with JE is often poor with occurrence of mortality in about one third of the patients; and, half of the patients who survive are often left with severe neuropsychiatric sequelae.  The typical presentation of JE includes an acute febrile illness associated with behavioural abnormalities, altered state of consciousness, seizures, abnormal involuntary movements, meningeal signs and focal neurological deficits in different combinations. Atypical presentations of JE have been reported and include isolated acute onset abnormal behaviour, aseptic meningitis, an acute flaccid paralysis-like illness and hemiplegia.  Diagnosis of JE depends on the clinical features and radiological changes in the thalamus and basal ganglia, supported by CSF abnormalities.  Poor prognostic predictors of JE in children include the presence of high fever, deep coma, a short prodromal stage, abnormalities in tone, and rapid breathing, decerebration, and focal neurological deficits.  Bilateral T2 hyperintense and T1 hypo-to isointense thalamic lesions, especially if hemorrhagic, are the characteristic imaging abnormalities seen in JE. The occurrence of bilateral isolated basal ganglia bleed as the only imaging abnormality in JE is rare. Sarkar et al., (2005) reported a 40-year-old patient with JE who presented with febrile illness and seizures. The cranial imaging in that patient revealed bilateral basal ganglionic bleed.  Differential diagnosis in patients with isolated bilateral basal ganglia bleed include arterial hypertension, amyloid angiopathy, methanol poisoning, migraine, head trauma, reversible vasoconstriction syndrome, hyperglycaemic hyperosmolar coma, diabetic ketoacidotic coma, and lightning strike.
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