Mills hemiparetic or hemiplegic variant of amyotrophic lateral sclerosis

Amit R. Barua, Nomal C. Borah, Mausumi Barthakur, Dhruba J. Choudhury, Jai P. Sharma, Pari Kemprai


Mills hemiplegic variant of Amyotrophic lateral sclerosis (ALS) is a gradually progressive, spastic ascending or descending hemiparesis or hemiplegia without any sensory involvement. Authors presented a 47 years old female with history of gradually progressive left sided wasting of muscles including the tongue, left hemiparesis along with dysarthria and fasciculation’s of tongue and left sided muscles with left sided cortico-spinal tract signs of 2 years duration. There were no sensory as well as bowel bladder involvement. Her cognition was intact. Relevant blood and CSF examinations were within normal limit. MRI Brain and whole spine were unremarkable. Nerve conduction study was essentially normal. Electromyography(EMG) showed chronic denervation potentials which is in accordance to Revised El Escorial criteria, 2015 for the diagnosis of this extremely uncommon entity- Mills hemiplegic variant of ALS. The major challenge in diagnosis of this disease entity is to exclude other diseases/disorders that may mimic its symptomatology.


Amyotrophic lateral sclerosis, Electromyography, Lower motor neuron, Mills syndrome, Revised El escorial criteria, Upper motor neuron

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Ropper AH, Samuels MA, Klein JP. Degenerative diseases of the nervous system;Syndrome of muscular weakness and wasting without sensory changes. Adams and Victor’s Principles of Neurology. 10th ed. Mc Graw Hill; 2014:1109-1111.

Porto FHG, Orsini M, Antônio Araújo M, Santos JM, Pulier S, Mello M, Nasciment O. Mills syndrome: Case report. Neurol Int. 2009 Nov 16;1(1): e15.

Ekmekci H, Ozturk S, Demir A. Mills' Syndrome--A Clinical Variant-Case Report. J Neurol Sci. 2013 Mar 1;30(1).

Bäumer D, Butterworth R, Menke RA, Talbot K, Hofer M, Turner MR. Progressive hemiparesis (Mills syndrome) with aphasia in amyotrophic lateral sclerosis. Neurol. 2014 Feb 4;82(5):457-8.

Alexander M. Approach to Motor neuron disease, Indian Academy of Neurology textbook of Neurology. 1st ed. 2018:672-79.

Chakor R. Brain and neurological disorders: A simplified health education guide. Ann Indian Acad Neurol. 2017 Jan 1;20(1):84.

Statland JM, Barohn RJ, McVey AL, Katz JS, Dimachkie MM. Patterns of weakness, classification of motor neuron disease, and clinical diagnosis of sporadic amyotrophic lateral sclerosis. Neurol Clini. 2015 Nov 1;33(4):735-48.

Lachaud S, Soriani MH, Delmont E, Budai M, Desnuelle C, Lebrun C. Mills' syndrome: a rare clinical entity. Revue Neurol. 2007;163(3):335-40.

Toepfer M, Folwanczy C, Klauser A, Riepl RL, Müller-Felber W, Pongratz D. Gastrointestinal dysfuntion in amyotrophic lateral sclerosis. Amytroph Lat Scler Other Motor Neuron Disord. 1999;1:2.

Chugh A, Shahbazi M, Lange D. Hemiplegic Form of Amyotrophic Lateral Sclerosis (ALS) with Cognitive Changes: An Autopsy Study (P02. 176).