A 63-year-old woman with no relevant morbid history presented with a two-month history of dysarthria, gait instability, upper limb tremor, and mood disorder.
The examination also highlighted the presence of cognitive defects (Minimental test score: 21), diffuse hyperreflexia, bilateral dentate wheel and short-term gait with brace reduction.
Blood count, ESR, renal and hepatic function, VDRL, HIV study, thyroid tests and antithyroid antibodies were requested, all of them being normal, except for a slight decrease in the level of vitamin B12 replacement was initiated.
Computed tomography (CT) of the brain was normal.
A few days later, multifocal myoclonus and bilateral Babinski were added to the clinical picture.
An electroencephalogram (EEG) was requested due to the suspicion of Creutzfeldt-Jakob disease (CJD), where a moderate generalized slowness was observed, with delta rhythm and the
Magnetic resonance imaging (MRI) of the brain showed bilateral hyperintense lesions in white matter in T2 and FLAIR sequences, which did not change with administration of 87% Gram-negative VDRL and normal glucose in the cerebrospinal fluid 30dl.
Faced with the suspicion of acute disseminated encephalomyelitis (ADEM), methylprednisolone boluses of one gram per day were administered for five days, with which the patient showed a slight improvement.
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However, three weeks later, the patient presented a progressive exacerbation of cognitive and motor symptoms, so it was decided to extend the study: cerebral CT angiography was normal, ELISA negatives rheumatologic diseases rheumatologic
Anti smooth muscle, thyroid, anticardiolipin and topical anticoagulant antibodies were negative.
The level of angiotensin converting enzyme was normal and the search for occult neoplasia was negative.
Two months later, the patient was bedridden, with bilateral pyramidal and extrapyramidal signs, myoclonus and verborrhoea with paraphasia.
A new EEG was requested which showed pseudoperiodic activity, which was not evidenced in the posterior control EEG.
The condition partially resolved with a new dose of 60 mg Prednisone daily for seven days.
During her evolution the patient repeatedly presented fever without evident infectious focus.
After four months of evolution, the patient presented a sudden deterioration with appearance of right hemianopsia and flaccid paresis of the right upper limb.
The study was repeated with brain MRI, which showed hyperintensity of left temporo-occipital signal and multiple subcortical foci at the level of semioval center and radiated cortex, associated with diffuse cerebral involvement.
There were no pathological uptakes or alterations in diffusion or spectroscopy.
Cerebrospinal fluid showed 100 leukocytes per field, predominantly mononuclear, with proteins of 286 mg/dl and without glucose alteration.
Suspicion of a PCNSV was made a cortical, subcortical and left occipital leptomeningeal biopsy, without previous conventional angiography.
Histopathology showed the presence of mononuclear infiltrate in the wall of small parenchymal vessels and reactive gliosis, with preservation of leptomenin-ges, compatible with cerebral vasculitis.
Treatment was initiated with cyclophosphamide in intravenous pulses of 1 gr/m2 and oral prednisone 60 mg/day. The patient presented a clear decrease in neurological deterioration with cognitive and myoclonus recovery, associated with mild neurological deterioration.
