AMYOTROPHIC LATERAL SCLEROSIS PLUS SYNDROME : A RARE CASE REPORT
English

Vanitha S, Balaraj K P
2015 Journal of Evolution of Medical and Dental Sciences  
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder. In 1994, the El Escorial criteria were proposed for the diagnosis of ALS. These criteria include ALS-plus syndromes, which are defined by an association of ALS with extrapyramidal features or dementia. we present a case report of 31 years young man presented with weakness of lower limbs since one and half years and slurring of speech since 8 months and on examination patient had scanning of speech, eye saccades were slow,
more » ... des were slow, upper motor neuron type of quadriparesis with cerebellar ataxia and dysmetria with no sensory or bowel bladder involvement. Routine blood investigations were normal and MRI Brain showed features of atrophy of cerebrum and cerebellum and ENMG shows neurogenic pattern of fasciculations and spontaneous activity. Clinically cerebellar ataxia and dysmetria with pure motor neuron disease suggests the possibility if ALS Plus syndrome especially ubiquitinated forms of TDP-43 and ubiquitinated p62-positive inclusions were frequently observed. As such it is the rare presentation in young adults, further studies over ALS plus syndrome will bring additional information about neuropathological details and its effect on outcome.
doi:10.14260/jemds/2015/437 fatcat:wijkgkyaifewvlvj3msocgnfs4