A young boy of 18 years was admitted at department of Neurology, Dhaka Medical College Hospital with the complaints of progressive generalized hyper-pigmentation, gradual loss of vision, hearing impairment, abnormal behaviors and one episode of seizure. Examination finding revealed, abnormal behaviors, generalized hyper pigmentation of skin, oral mucosa, gum, tongue and palmer creases. He has diffuse hair loss, bilateral primary optic atrophy, bilateral sensoryneural deafness. All routine investigations revealed normal findings except, CSF protein were elevated, biochemical features (very high ACTH, low basal cortisol) of primary adrenal failure, Magnetic resonance imaging (MRI) of the head showed bilateral symmetrical white matter abnormalities in parieto-occipital regions. The diagnosis of Adreno-leukodystrophy (ALD) was strongly suggested from the medical history, biochemical and radiological (MRI) findings of brain. The purpose of our report is to highlight this very rare nontreatable disease to all. A patient of neuropsychiatric symptoms with Addison's disease we must think about ALD, because it's progression can be delayed with early diagnosis and supportive treatments, it's incidence can be reduced by genetic counseling.Key words: Adrenoleukodystrophy (ALD); Addison's disease; Very long chain fatty acid (VLCFA). DOI: 10.3329/jbcps.v28i3.6514J Bangladesh Coll Phys Surg 2010; 28: 189-192
Neurocysticercosis (NCC), the infection caused by the larval stage of the tapeworm Taenia solium, is the most common parasitic disease of the nervous system (NS) in humans, where it is the leading cause of late-onset seizures in the endemic areas. Fortunately it is still rare in Bangladesh due to some religious binding in eating pork. Here we present a young man who came to us with Neurocysticercosis as the cause of recurrent seizure and various neurological manifestations. Keyword: Neurocysticercosis, Taenia solium, seizure. DOI: http://dx.doi.org/10.3329/jom.v12i2.8425 JOM 2011; 12(2): 177-179
DOI: 10.3329/jbcps.v28i3.6518J Bangladesh Coll Phys Surg 2010; 28: 202
Fig.-1: (a) Left sided proptosis (b) Contrast-enhanced CT scan of brain showing homogenously enhancing mass lesion involving the falx cerebricomplaints. Her examination was unremarkable except for proptosis of left eye and bilateral papilledema. Complete blood count and peripheral blood films were normal. A contrast CT scan of brain revealed homogenously enhancing mass lesion involving the falx cerebri ( Figure 1). The consulting neurosurgeon performed craniotomy and decompression with resection of the mass lesion. Histopathological study of the resected mass showed diffuse monomorphic population of lymphocyte copmpatible with non-Hodgkin's lymphoma. She was referred to the oncology unit.Primary CNS lymphoma (PCNSL) is a rare type of nonHodgkin's lymphoma affecting the brain, leptomeninges, spinal cord or eyes in the absence of lymphoma outside the CNS. It accounts for 1% of non-Hodgkin's lymphoma (mostly of B-cell origin) and 3-5% of all primary brain tumour. 1 Though immunodeficiency is the principal risk factor, majority of patients are immunocompetent. PCNSL presents with a focal mass lesion (50%), neuropsychiatric signs (43%) and raised intracranial pressure (33%). 2 Treatment options include high-dose methotrexate with or without whole-brain radiotherapy. Prognosis depends on several factors of which age and performance status are the most important. Conflict of interest: None References:1.del Rio MS, Rousseau A, Soussain C et al.
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