A 15-year-old female patient with no relevant morbid history, no infectious episodes or vaccinations in the previous months.
He was admitted to the Clinical Hospital of the University of Chile for presenting a clinical picture of 3 weeks of evolution, characterized by behavioral changes, puerility and disinhibition.
A confusional syndrome and decreased strength in the left hemibody associated with mild to moderate holocraneal headache were added.
On admission, the neurological examination revealed a patient alert but confused, with symmetric photoreactive pupils, eye fundus without papilledema, paralysis of the 6th pair left vertical extensor hemiparesis B.
Computed tomography (CT) showed moderate hypodensity around the right temporal horn and another that compromised the thalamus on the same side, with little mass effect and no significant contrast uptake.
Magnetic resonance imaging (MRI) showed extensive hyperintense lesions in the FLAIR sequence at the right mesial temporal level medial to the temporal horn, at the level of the thalamus on the same side and around the third.
A small hyperintense focus was also observed in the left anterior subinsular region.
These lesions had little mass effect and an evident alteration of the adjacent hematoencephalic barrier, objectified by a significant increase of the T1 signal after the use of Gadolinium in the temporal periphery of the lesions, especially in the temporal periphery.
The findings described raised the possible diagnoses of encephalitis or lymphoma and, less likely, a pseudotumoral demyelinating lesion.
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General examinations were within normal limits.
The initial study of cerebrospinal fluid (CSF) showed 20 leukocytes (100% mononuclear) with glucose and normal proteins.
With these clinical elements and due to the severity of a possible herpetic encephalitis, empirical treatment was started with antiretroviral therapy.
Immunological studies (RF, ANA, ENA, ANCA) and systemic lymphoma (LDH, ß2 microglobulin, chest and abdominal CT) were negative.
Herpes virus PCR, oligoclonal bands and albumin/IgG ratio in CSF were normal.
Antiviral treatment was suspended and treatment with betamethasone (4 mg every 8 h) was started to reduce cerebral edema, in view of the possibility of an expansive process.
Without reaching a diagnosis yet, a brain SPECT with Talio 201 was performed, which showed a focal point of slight uptake in the right thalamus that extended towards temporal, suggestive of lymphoma, rather than demyelinating lesion.
The patient arrives with progressive neurological deterioration, characterized by greater commitment of consciousness, medium support, total compromise of the vertical gaze and left hemiplegia.
Given the severity of the patient and the lack of diagnosis, we decided to perform a stereotactic biopsy.
A centered archwire ring and a three-dimensional computer planning system were used to obtain the precise coordinates and angles to reach the target without injuring eloquent brain tissue through a right precoronal approach.
Samples were taken from the center of the thalamic lesion, procedure without complications.
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The histological study of the biopsy showed a discrete inflammatory infiltrate, predominantly mononuclear, preferably perivascular distribution and reactive gliosis, consisting of gemistocytes and foamy cytoplasm macrophages with PAS + granulocytes.
These findings are compatible with a demyelinating disease of subacute course.
Treatment with high doses of methylprednisolone (one gram per day for six days) was initiated, with which the patient showed a marked improvement in both her consciousness and her focal neurological defects.
He did not present new episodes after a year of evolution.
