Contributed by Luis M. Tumialán, M.D.1 , Meenakshi Gupta, M.D.2, Stephen Hunter, M.D. 2, Luis Tumialán, M.D.3
Emory University School of Medicine, Department of Neurosurgery1, Department of Pathology and Laboratory Medicine2 Atlanta, Georgia;
Lutheran Medical Center3, Department of Pathology, St. Louis, Missouri
A 55-year-old African American man with an eight month history of poorly controlled seizures and longstanding history of cirrhosis, diabetes and hypertension was brought to the emergency department for progressively worsening confusion, memory loss and increasing frequency of seizures. On the day of presentation, the patient had a prolonged generalized tonic-clonic seizure. The patient was being treated for a hepatic encephalopathy with lactulose. Within the last eight months he had had a transjugular intrahepatic portal system shunt for management of his end stage liver disease and was awaiting a liver transplant. A non-contrast CT of the head revealed no acute intracranial process. An MRI was subsequently obtained. After admission, the patient's mental status continued to decline and ultimately progressed to multisystem organ failure. He suffered a cardiopulmonary arrest and expired.
Gadolinium enhanced T1-weighted MRI revealed multiple small punctate foci of leptomeningeal enhancement in the sulci between the folia of the superior cerebellar hemispheres, the flocculi and the ventral medulla (figures 1, 2, 3 and 4). On FLAIR sequence, the inferomedial left frontal lobe and the right gyrus rectus both demonstrated an area of increased signal intensity (figures 5 and 6). There was no enhancement of these areas with gadolinium.
PPOST MORTEM EXAMINATION:
Autopsy revealed enlargement of mediastinal lymph nodes with evidence of sarcoidosis, a cirrhotic liver with thrombosis of the transjugular intrahepatic portal system shunt and kidneys with evidence of hypertensive nephrosclerosis. Microscopic examination of the lungs demonstrated numerous granulomas in the mediastinal lymph nodes.
Gross examination of the brain revealed normal gyral pattern, unremarkable dura and leptomeninges. There was no evidence of uncal or tonsillar herniation. The Circle of Willis had symmetrical vessels without evidence of aneurysms or plaques. Coronal sections (5mm) demonstrated a normal cortical ribbon, a well demarcated gray/white matter and a normal ventricular system. Brain stem cerebellum and spinal cord showed no abnormalities on gross examination.
Microsections of the brain showed multifocal non-necrotizing granulomas in the leptomeninges covering the cerebral cortex (figure 7, 8, 9, 10), cerebellar hemispheres and the brain stem (figure 11 and 12). A predilection for the perivascular location was evident. These granulomas contained numerous multinucleated giant cells admixed with lymphocytes. Special stains for microorganisms including mycobacteria and fungi were negative. Hippocampal sclerosis consistent with metabolic/hypoxic ischemic type injury was also observed.