1. Gliomatosis cerebri presenting as epilepsia partialis continua: A case- report
GARCIA-FRUCTUOSO G1.3, Alamar M1, Candela S1, Regueira M2, Nunes TF1, Rumia J3, Guillen A1,Donaire A3, Campistol J1, Pineda M1,, Costa JM1 Sanmarti FX1,3.
1.  Universitary  Hospital Sant Joan de Déu. Barcelona. Spain 2..Hospital Xeral Vigo. Spain. 3. Epilepsy Unit-HSJD/Hospital Clinic. Barcelona. Spain
Introduction
Gliomatosis cerebri (GC) is a rare, infiltrative primary brain tumor from glial origin The term GC applies to the the implication of two or more brain lobes with possible extension to brain stem, cerebellum, spinal cord and subarachnoid space.
Diagnosis and treatment is difficult, it has been mainly reported in adults, but can also affect children. It presents with progressive headache, hemiparesis and focal seizures.
Objective: To review an atypical clinical presentation of Gliomatosis Cerebri
Fig 1: EEG: focal epileptic activity in the left hemisphere, with 200 SPS per day, maximal frequency of initial discharges located in O1
Case report
We report the case of a 8 year-old child who presented
with epilepsia partialis continua on the right side of his body,
perioral, eyelid and right limbs clonic seizures, confirmed with
video-EEG (fig.1)
Early neuroimaging was compatible with first stages of
Rasmussen encephalitis (RE).(Fig 2)
He was treated with steroids, human gamma globulin, and
antiepileptics drugs, without relief of seizures and with progressive
language impairment.
After clinical worsening,he was reassessed:
there was a lack of common brain atrophy of RE in the new MRI,
and PET showed a significant hypometabolism. (Fig 3,4)
A neuronavigator-guided brain
biopsy was performed.
Pathological study was compatible with
Gliomatosis cerebri (Fig 5,6,7)
Fig 2 : MRI brain (at 2 m of evolution) (T1 , T2 ): : Thickening and signal change in posterior frontal cortex, operculum, temporal cortex, posterior parietal and left parasagittal well as the thalamus, described as early stages of RE.
Fig 3: MRI brain (at 5m of evolution) (T1 contrast axial- Flair coronal ): there is a significant mass effect and there is no contrast enhancement
Fig 4: PET: Extense area of left hemysphere hypometabolim. There are no images suggestive of active focal high-grade brain tumor.
Fig 5: (H&E) Tissue section. Brain cortex showing astrocytosis and satellytosis.
Fig 6: (H & E) Higher magnification shows hypercelullarity of atipycal glial cells.
Fig 7: Ki-67 staining .Positive: frequent dark brown nuclei indicating high proliferative index.
Comments
Gliomatosis cerebri may present with epilepsia partialis continua. Confusing cases deserve brain biopsy. This association is rarely described
in literature. Epilepsia partialis continua in children has most commonly been asociated with Rasmussen encephalytis, focal cortical dysplasia
and hemimegalencephaly and has been rearely associated with various respiratory chain mitochondrial disorders including MELAS,
Leigh syndrome and others..
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