A Demyelinating Disease; What Lies Beneath?

Autor: Alaa Elmanzalawy, Fatma Rabah, Ismail Beshlawi, Nagwa El-Banna, Dipali Bhuyan
Rok vydání: 2015
Předmět:
Male
medicine.medical_specialty
Hydrocortisone
Leucovorin
Central Nervous System Neoplasms
Diagnosis
Differential
White matter
Lesion
03 medical and health sciences
0302 clinical medicine
Antineoplastic Combined Chemotherapy Protocols
medicine
Humans
Cyclophosphamide
Etoposide
medicine.diagnostic_test
business.industry
Brain biopsy
Encephalomyelitis
Acute Disseminated
Cranial nerves
Cytarabine
Glasgow Coma Scale
Primary central nervous system lymphoma
Brain
medicine.disease
Combined Modality Therapy
Magnetic Resonance Imaging
Methotrexate
medicine.anatomical_structure
Doxorubicin
Vincristine
Child
Preschool
030220 oncology & carcinogenesis
Pediatrics
Perinatology and Child Health
Prednisone
Lymphoma
Large B-Cell
Diffuse
Radiology
Cranial Irradiation
medicine.symptom
business
Diffuse large B-cell lymphoma
Demyelinating Diseases
030215 immunology
Diffusion MRI
Zdroj: The Indian Journal of Pediatrics. 83:365-367
ISSN: 0973-7693
0019-5456
DOI: 10.1007/s12098-015-1849-5
Popis: A 5-year-old boy presented with 1 mo history of excessive sleepiness, early morning headache and vomiting. Moreover, a 5-day history of increasing irritability was noted. Clinical examination demonstrated bradycardia, hypertension and right-side facial palsy. Subsequently, an urgent CT brain was done which revealed diffuse vasogenic cerebral edema with extensive white matter hypodensities. Such white matter changes were further defined by MRI (Fig. 1). Herpetic encephalitis and acute demyelinating encephalomyelitis were the top differential diagnoses. Accordingly, the child was given IVIG and IV acyclovir. Despite treatment, his Glasgow coma scale dropped gradually to 7 meriting intubation. The deteriorating clinical course necessitated exclusion of primary central nervous system lymphoma (PCNSL). Therefore, a stereotactic brain biopsy was executed. The pathology report was diagnostic of diffuse large B cell lymphoma (DLBCL). Further workup has unveiled spinal drop metastases. PCNSL constitutes 3 % of all brain tumors [1]. About 90–95 % of the cases are constituted by DLBCL, possibly affecting brain, spinal cord, eyes, meninges, and cranial nerves [2]. Our patient was commenced on chemotherapy protocolB LMB 89 group C^ with high dose methotrexate and whole brain irradiation [3]. He was brought into remission over the 3 y. MRI features of PCNSL in immunocompetent patients usually are non-specific; MRI shows a single lesion in 65 % of cases. Such a lesion is either homogenously isointense or hypointense to cortex on T1weighted images or hyperintense on T2-weighted images [4]. The borders of the lesion are either well defined or irregular with variable extent of surrounding edema and homogenous gadolinium enhancement [2, 4]. The lesions are frequently hyperintense on diffusion weighted imaging and hypointense on apparent diffusion coefficient maps. Such diagnostic radiologic feature is attributed to the restricted diffusion rate of unbound extracellular water molecules due to hypercellularity of PCNSL [5]. The rarity of such childhood malignancy coupled with the nonspecific presenting symptoms that could be explained by commoner benign diseases comprise a diagnostic challenge [1]. CNS malignancy is an important cause of diffuse demyelination that should be explored if the presentation of ADEM is compounded with persisting or worrisome clinical picture. Reaching such a diagnosis in a timely fashion is of crucial significance to patient’s outcome [1]. * Fatma Rabah fatmarabah@gmail.com
Databáze: OpenAIRE
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