Case 477 -- A 64-year-old woman with progressive dementia and leukoencephalopathy

Contributed by Anne Vital, MD, PhD1; Igor Sibon, MD2
1Neuropathology and 2Neurology Departments, Victor Segalen-Bordeaux University, France.


CLINICAL HISTORY:

A 64-year-old diabetic woman presented with progressive cognitive deterioration and gait ataxia. Symptoms appeared one month before admission, without hyperthermia. Clinical examination showed temporo-spatial disorientation, loss in attention, judgment and memory capacities, a gait ataxia with a kinetic cerebellar syndrome and a left side bradykinesia. There was neither pyramidal syndrome nor cranial nerve palsy. General examination was normal. Brain MRI (T1-wi with and without gadolinium, Flair and T2-wi) found a periventricular diffuse leukoencephalopathy (Figure 1A) without gadolinium enhancement (Figure 1B). EEG showed diffused slow waves and epileptic seizures without periodic paroxystic activity. Hematological and biological serum analyses (including LDH, beta2-microglobulinemia, fibrinogen, CRP, electrophoresis and immunoelectrophoresis of the proteins, transaminases, urea, creatinine, lymphocyte immunophenotyping) were normal. All serological investigations (HIV, HSV1 and HSV2, CMV, VZV, EBV, JC virus) remained negative. The CSF presented normal cell count (2 lymphocytes / mm3) and protein level (0,3 g / L), without abnormal cells or intrathecal protein synthesis except a positivity of 14.3.3 protein. The neurological state worsened progressively with dementia, epileptic seizures, extrapyramidal and cerebellar syndromes. The patient died three months after onset of symptoms, and an autopsy restricted to the brain was performed.

GROSS AND MICROSCOPIC EXAMINATION:

The brain weighed 1330 g. Coronal sections of the cerebral hemispheres, as well as horizontal sections of the cerebellum and brain stem were not informative. Microscopic examination revealed infiltrates of atypical lymphoid cells (Figure 2A; bar = 400 ) in the deep layers of cortex, white matter, basal ganglia, substantia nigra, pons, medulla and cerebellar white matter. These infiltrates were diffuse and present on all sections from the brain hemispheres, brain stem and cerebellum. In most areas, the tumor cells were scattered but atypia and mitoses were evident (Figure 2B; bar = 100 ). A perivascular distribution was noticed in some areas, and no necrosis was observed. There was a strong reactivity of the tumor cells for CD 20 (Figure 2C; bar = 100). A search for EBV by in situ hybridisation was negative.

FINAL DIAGNOSIS


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