Clinical Spectrum and Neuroimaging Changes in Neurodegenerative Disorder-Experiences in Tertiary Care Hospital of Bangladesh



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Submitted Oct 28, 2021
Published Nov 23, 2021
10.24018/clinicmed.2021.2.6.143

Abstract viewed = 432 times

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Neurodegenerative diseases (NDD) are a heterogeneous group of disorders characterized by progressive loss of previously acquired skills that are of varied etiology, clinical manifestations, and natural course. There is a paucity of data on clinical profile of neurodegenerative diseases in our population.

We conducted a retrospective study with 68 diagnosed cases of NDD at a tertiary care hospital in Bangladesh. Among them, more than one-third of children were in 1-5 years age group. The mean age was 10.2±3.1 year and male to female ratio was 2:1. Fifty percent of cases had a history of consanguineous parents. Leukodystrophy was most common (30.88%) among NDDs, followed by Wilson disease (26.47), SSPE (22.1%), and Degenerative Ataxia (20.59%).

Motor skill regression was the most common presentation (97%), followed by speech regression in 91% and Gait disorder in 83% of children. Seizure was presenting features in 24% of children. Neuroimaging abnormalities were found in 80.88% NDD cases. Among them white matter hyper intensity in 29.41%, cerabeller atrophy in 13.25 %, and cerebral atropy in 11.76% of children. Eye changes were found in about two-thirds (69.12%) of cases of NDD. Among them, optic atrophy was found in 29.41%, and KF rings in 25.00% of cases.

Keywords: Neurodegenerative disease, neuroimaging, ophthalmological findings

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