Madras motor neuron disease (MMND): Clinical description and survival pattern of 116 patients from Southern India seen over 36 years (1971–2007)

Neurofilament localization and phosphorylation in the developing inner ear of the rat

“…Additionally, as aberrations in NFs may result from inherited disorders, as has been shown for e.g. CharcoteMarieeTooth disease (Butinar et al, 2008), genes encoding for proteins that regulate or interact with NFs should be considered as candidate genes for sensorineural hearing loss (Jordanova et al, 2003;Fabrizi et al, 2007;Nalini et al, 2008).…”

Chin fasciculations in Madras motor neuron disease: A new clinical feature

“…[6,7] The oldest age at onset reported until date is 39 years. [5] Chin fasciculations are reported for the first time since this feature has not been recorded either in MMND [1][2][3][4][5] nor in the recently described variant, MMNDV. [13] Fasciculations of chin is a rare clinical finding and seen only in a few disorders.…”

“…In MMND bilateral facial weakness has been reported in 30-50% of patients. [2][3][4][5] The rate of progression of the disease over 18 months was also very much slower than in ALS as there was only a slight decrease of score on Norris scale from 93 to 87 and ALSFRS from 37 to 32. MMND has a protracted course with slow progression and a mean survival of 334.9 ± 27.9 (SE) months.…”

“…The distinctive features of MMND include asymmetrical weakness and wasting of limb muscles, pyramidal signs, involvement of lower cranial nerves and facial muscles and association of bilateral perceptive deafness and slow prolonged course. [2] Subsequent to the first description, more cases were described from Madras and Bangalore in South India, [3][4][5] a few from other regions of India [6][7][8] and isolated case reports from other countries. [9][10][11][12] Additional features of optic atrophy and cerebellar signs were described in the MMND variant (MMNDV).…”

Triad of visual, auditory and corticospinal tract lesions: a new syndrome in a patient with HIV infection

“…Madras motor neuron disease [8] is occurring predominantly in southern India. Its clinical features are pyramidal dysfunction, wasting and weakness of distal muscles, facial and bulbar muscles and hearing impairment, and there is a variant with optic atrophy.…”