LAUSR

Annals of Indian Academy of Neurology ¦ Volume 23 ¦ Issue 3 ¦ May-June 2020 387 absence of progressive neuroimaging changes. In pure forms, MRI spine can be normal; however, spinal cord atrophy and hydromyelia has been reported in cases with SPG56 variation.[5] We propose that hydromyelia seen in the index child may be an incidental finding and cannot explain the entire spectrum of clinical symptoms seen in this child. Hence, it probably is not the cause of spastic paraparesis in the index child. MRI brain may reveal white matter abnormalities on corticospinal tracts with thinning of corpus callosum.[6] Common clinical differential diagnoses include spastic diplegic cerebral palsy, subacute combined degeneration of spinal cord, copper deficiency, Segawa disease, Friedreich’s ataxia, mitochondrial respiratory chain disorders, organic acidurias such as biotinidase deficiency, and methylmalonic aciduria, arginase deficiency, and structural abnormalities of brain and spinal cord.[7] Family history may give a clue to the diagnosis, prompting a genetic confirmation. The rate of progression in ATL1-related HSP is slow, and wheelchair dependency is relatively rare as exhibited by the disease course in the father who suffers from disability but is able to carry out his activities of daily living and preserved intellect.