History Of Speech Difficulties Research Articles

Transient Global Amnesia as a Presenting Manifestation of Cerebral Venous Thrombosis

Transient Global Amnesia as a Presenting Manifestation of Cerebral Venous Thrombosis

OT-02 * PEDIATRIC ANAPLASTIC MENINGIOMA: A CASE REPORT SUCCESSFULLY TREATED WITH COMBINED CHEMOTHERAPY FOR SOFT TISSUE SARCOMAS AND RADIOTHERAPY

A 2 year-old child with motor deficits and headache was referred to our Hospital with an history of speech difficulties and walking difficulties. A brain MRI scan evidenced a giant tumor of the right hemisphere with diffuse contrast enhancement, hydrocephalus and mass effect. After diagnosis, he immediately underwent neurosurgery and a massive debulking of the lesion was performed. The histological diagnosis was anaplastic meningioma (WHO-grade III). Despite radical surgery, after one-month evidence of disease progression was documented by MRI (relapse on surgical margins) and lumbar puncture (neoplastic cells in liquor sample). Rapid progression, young age and absence of successful therapeutic alternatives determined the decision to start chemotherapy with a protocol for soft tissue sarcomas (EpSSG NRSSTS 2005 protocol). The treatment was started in December 2011 and ended in April 2012, and consisted in 3 cycles of Ifosfamide (3 g/m2/day for 3 days) plus Doxorubucin (37.5 mg/m2/day for 2 days) every 21 days. The cycles were followed by Ifosfamide (3 g/m2/day for 3 days ) concomitant with radiotherapy (54 Gy on tumor primary site). A last cycle of Ifosfamide plus Doxorubicin was after performed. In the last 2 cycles, Doxorubicin dose was reduced of 50% to avoid toxicity. End therapy evaluation showed stable disease at brain MRI and absence of tumor cells in liquor sample. Nonetheless, considering risk of disease progression and of late chemotherapy side effects, a strict follow-up was started. After 3 years from diagnosis the patient showed good clinical conditions, with no evidence of disease progression. Anaplastic meningioma is the most undifferentiated, invasive and aggressive type of meningioma (approximately 1-4%). Surgery and radiotherapy are the most effective therapeutic options, while chemotherapy is usually useless. This case represents a successful management of an anaplastic meningioma with chemotherapy in a pediatric patient.

P11.09 * A NEUROLOGIC PRESENTATION OF INTRAVASCULAR LARGE B-CELL LYMPHOMA

INTRODUCTION: Intravascular large B-cell lymphoma is a rare and fatal disease that can have central nervous system manifestations. Given its rarity and the multiplicity of presentations, diagnosis is extremely difficult. We report a case that presented with neurological manifestations and which diagnosis was made ante mortem. CASE REPORT: A 74-year-old man, with a background of hypertension and dyslipidemia was referred to our neurology department with a history of speech difficulties and walking disturbances that rapidly evolved to an acute confusional sate. Physical examination disclosed an obvious executive dysfunction, global dysphasia and gait dyspraxia. Computed tomography scan of the brain showed a right frontal paramedian subcortical hiperdensity. Further characterization with brain MRI showed that this lesion was not expansive and did not enhanced with gadolinium. On laboratory analysis there was a mild lymphopenia and increased lactate dehydrogenase and ferritin levels; erythrocyte sedimentation rate was in the normal range and the immunological and virological tests were negative. Glucocorticoids (methylprednisolone 1g, intravenous, per day, during five days) were administered, leading to some improvement in the patient's symptoms. However, the control brain MRI disclosed lesion worsening, with bihemispheric extension. The cerebrospinal fluid study was unremarkable and the anti-neuronal antibodies were negative. Control laboratory analysis revealed an increase in serum C-reactive protein, erythrocyte sedimentation rate, lactate dehydrogenase (>900 IU/l), hepatic enzymes and B2-microglobulin levels. Electroencephalography was unremarkable and there was no evidence of emboligenous cardiopathy on the transesophageal echocardiogram. A computed tomography scan of the chest abdomen and pelvis was performed, with evidence of nodular thickening of both adrenal glands. An adrenal gland biopsy was undertaken, and the histological exam confirm the diagnosis of intravascular large B-cell lymphoma. Patient's condition rapidly worsened, evolving to depression of consciousness, cortical blindness, tetraparesis with brachial diplegia, and the patient died. CONCLUSION: The clinically heterogeneous spectrum of presentation and the lack of pathognomonic features or markers have made ante mortem clinical diagnosis of intravascular large B-cell lymphoma difficult and challenging. This report highlights the diagnostic challenge, the aggressive behavior and the devastating clinical course associated with this clinical entity.