A 30-year-old agricultural laborer presented to the neurology services with a history of three episodes of generalized tonic clonic seizures, irritability, bursts of anger, abusive behavior and inability to recognize close relatives spanning over a period of 1 year and fever with a head ache for 7 days. At 3 days after the onset of fever patient lapsed into altered sensorium along with urinary incontinence. On the day of admission to the hospital, the patient developed right sided weakness.
On examination, the patient was febrile, the rest of physical parameters and vitals being normal. Neurological examination revealed GCS of 6/15, E1V2M3 and right hemiparesis. The patient was not opening his eyes to deep painful stimuli and the extra ocular movements were restricted with papilledema. Both pupils were not reactive to light and dilated. The deep tendon reflexes were brisk with an extensor plantar response on the right side and flexor plantar on the left side.
Complete hemogram and peripheral smear examination were normal, but for a total leucocyte count of 15,200 and erythrocyte sedimentation rate of 65 mm/1 h (Westergren). The routine biochemical parameters were normal except for mildly elevated liver enzymes. Tests for HIV and HBs were negative. CSF analysis revealed a cell count of 170 cells (80% polymorphs), protein 117 mg% and sugar -11 mg%. Cranial CT scan revealed multiple disc enhancing lesions both in the cerebral grey and white matter and brain stem with edema and hydrocephalus.
MRI revealed [Figure 2a-e] multiple subcortical hyperintensities in the bilateral frontal, parietal, temporal white matter and in the pons and midbrain region on T2 and FLAIR images. A few well defined T2 hypointense lesions suggestive of small tuberculomas were also noted in the subcortical location in the bilateral frontal regions. Heterogeneous signal intensity was noted in the left thalamus and caudate nucleus. These lesions were iso to hyper on T1W1 and predominantly hypointense on T2 weighted images with perilesional edema. The lesions bloomed on gradient imaging suggesting a hemorrhagic component. The hyperintense lesions on T2 and FLAIR showed a mixed pattern of restricted and facilitated diffusion. DWI revealed hyper intensity in right frontal subcortical white matter showing restricted diffusion on apparent diffusion coefficient (ADC) maps. MRI brain revealed multiple discoid irregular enhancing lesions in the white matter extending into grey matter. FLAIR images revealed multiple serpegineous hypointense lesions in high parietal section showing thrombosed cortical veins as hypointense curvilinear structures.
The patient received antitubercular drugs and steroids. Clinical diagnosis of TBM with multiple tuberculoma was made. Possibility of cerebral toxoplasmosis and cysticercosis were also considered in view of ring enhancing lesions and the endemicity. However he succumbed on 4th day of hospital admission. Autopsy confined to the examination of the brain alone was conducted 13 h after death following informed consent of the close relatives [Figure 3a-e]. Smear from the basal exudate revealed numerous acid fast organisms on Zeihl-Neelson stain. Post mortem serum and CSF were positive for mycobacterial antibody and IgG and IgM type mycobacterial immune complex and negative for cysticercosis and toxoplasmosis. Brain weighed 1200 g and appeared edematous and congested. The superior sagittal sinus along its entire length was thrombosed with extension into the left transverse and sigmoid sinus and involvement of proximal right transverse sinus, straight sinus and the vein of galen were patent. Several superficial cortical veins over the right posterior frontal and parietal lobes were thrombosed, the parenchyma underlying these vessels being softened and hemorrhagic.
The leptomeninges were hazy with creamy exudates and small tubercles along the course of the sylvian fissure and basal cisterns, obscuring the underlying structures. Coronal slicing of the brain revealed a large hemorrhagic lesion in the parasagittal right frontoparietal area involving the white matter with edema reflecting venous pathology. Left basal ganglia and thalamus had ischemic infarcts with zones of petechial hemorrhage, representing reperfusion. The brain stem and cerebellum were covered by thick exudates and in ventral mid brain (substantia Nigra) a tiny tuberculoma was found.
Histological examination revealed organizing thrombus in the venous sinuses and major superficial veins. Tiny tubercles were observed in the frontal lobe along the walls of the superficial anastomotic veins. In the subarachnoid space the veins were entrapped in the chronic inflammatory exudates. Numerous Acid Fast Organisms were found in the exudates covering the vessels. The ventricular ependyma was normal. There was no granulomatous response or inflammation in the thrombosed dural venous sinuses.