Open AccessClinical Spectrum and Neuroimaging Changes in Neurodegenerative Disorder-Experiences in Tertiary Care Hospital of Bangladesh
Author(s) -
Gopen Kumar Kundu,
Rumana Islam,
Noor E-Sabah,
Abm Mukib
Publication year - 2021
Publication title -
european journal of clinical medicine
Language(s) - English
Resource type - Journals
ISSN - 2736-5476
DOI - 10.24018/clinicmed.2021.2.6.143
Subject(s) - pediatrics , medicine , etiology , neuroimaging , atrophy , ataxia , retrospective cohort study , tertiary care , population , white matter , leukodystrophy , neurology , disease , magnetic resonance imaging , psychiatry , radiology , environmental health
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.