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Madras motor neuron disease

Mandaville Gourie-Devi, Siddharth Maheshwari, Akhila Kumar Panda, Kiran Bala
The characteristic features of Madras motor neuron disease (MMND) are onset in the young in the first two decades, sporadic occurrence, facial and bulbar paralysis, sensorineural hearing impairment, asymmetrical weakness of limbs and pyramidal signs with a slow progression. The majority of the cases reported are from South India. MMND variant has the additional features of optic atrophy and cerebellar signs. We are reporting a 48 year old female of MMND who had persistent fasciculations of chin, with electromyographic features of fasciculations and fibrillations in mentalis muscle...
November 2013: Neurology India
Atchayaram Nalini, Amelie Pandraud, Kin Mok, Henry Houlden
INTRODUCTION: Madras motor neuron disease (MMND), MMND variant (MMNDV) and Familial MMND (FMMND) have a unique geographic distribution predominantly reported from Southern India. The characteristic features are onset in young, weakness and wasting of limbs, multiple lower cranial nerve palsies and sensorineural hearing loss. There is a considerable overlap in the phenotype of MMND with Brown-Vialetto-Van Laere syndrome (BVVL) Boltshauser syndrome, Nathalie syndrome and Fazio-Londe syndrome...
November 15, 2013: Journal of the Neurological Sciences
Periyasamy Govindaraj, Atchayaram Nalini, Nithin Krishna, Anugula Sharath, Nahid Akhtar Khan, Rakesh Tamang, M Gourie-Devi, Robert H Brown, Kumarasamy Thangaraj
Although the Madras motor neuron disease (MMND) was found three decades ago, its genetic basis has not been elucidated, so far. The symptom at onset was impaired hearing, upper limb weakness and atrophy. Since some clinical features of MMND overlap with mitochondrial disorders, we analyzed the complete mitochondrial genome of 45 MMND patients and found 396 variations, including 13 disease-associated, 2 mt-tRNA and 33 non-synonymous (16 MT-ND, 10 MT-CO, 3 MT-CYB and 4 MT-ATPase). A rare variant (m.8302A>G) in mt-tRNA(Leu) was found in three patients...
November 2013: Mitochondrion
Baris Isak, Kayihan Uluc, Tulin Tanridag, Selda Ozsahin, Reinhard Dengler, Onder Us, Susanne Petri
We present a 19-year-old female patient complaining of hoarseness and eyelid drooping. The neurological examination and laboratory investigations including genetic, radiological and electrophysiological evaluations were consistent with a juvenile-onset, predominantly bulbar, motor neuron disease with sensorineural hearing loss. The syndrome fulfilled the diagnostic criteria of Madras Motor Neuron Disease (MMND). Very few cases with MMND have been reported to date, and the majority are from south-eastern Asia...
October 2009: Amyotrophic Lateral Sclerosis
Adesola Ogunniyi
Tropical neurology began less than two centuries ago. Consumption of dietary toxins predominated at the beginning and gave birth to the geographic entity. The story moved from lathyrism through Jamaican neuropathy to cassava-induced epidemic neuropathy, which was contrasted with Konzo, also associated with cassava. Other tropical diseases enumerated with chronological details include: Chaga's diseases, kwashiorkor, Madras type of motor neuron disease, atlanto-axial dislocation, Burkitt's lymphoma and Kuru, associated with cannibalism among the Fore linguistic group in New Guinea...
2010: Handbook of Clinical Neurology
Sivakumar Sathasivam
The Brown-Vialetto-Van Laere syndrome (BVVL) is a rare neurological disorder characterized by progressive pontobulbar palsy associated with sensorineural deafness. Fifty-eight cases have been reported in just over 100 years. The female to male ratio is approximately 3:1. The age of onset of the initial symptom varies from infancy to the third decade. The syndrome most frequently presents with sensorineural deafness, which is usually progressive and severe. Lower cranial nerve involvement and lower and upper motor neuron limb signs are common neurological features...
April 17, 2008: Orphanet Journal of Rare Diseases
A Nalini, K Thennarasu, B K Yamini, D Shivashankar, Nithin Krishna
Madras motor neuron disease (MMND), MMND variant (MMNDV) and Familial MMND (FMMND) have a unique geographic distribution predominantly reported from Southern India. The characteristic features are onset in young, weakness and wasting of limbs, multiple lower cranial nerve palsies and sensorineural hearing loss. We describe the clinical features and survival pattern in 116 patients with Sporadic MMND, MMND variant and FMMND. A retrospective review of patients' medical records for clinical manifestations, electromyography, imaging, audiological and histopathology findings was performed...
June 15, 2008: Journal of the Neurological Sciences
A Nalini, B K Yamini, N Gayatri, K Thennarasu, R Gope
Madras motor neuron disease (MMND) and Madras motor neuron disease variant (MMNDV) have an unique geographic distribution with concentration of majority of cases in the southern states of India. They have the characteristic features of onset in young, weakness and wasting of the limbs, multiple cranial nerve palsies particularly involving the 7th, 9th to 12th and sensorineural hearing impairment. In addition, all patients with MMNDV have bilateral optic atrophy. During the past 32 years, a total of 104 patients were diagnosed to have MMND and among these 25 patients with the familial form were further evaluated...
December 1, 2006: Journal of the Neurological Sciences
Dongsheng Fan, Yu Fu, Aping Sun, Dexuan Kang
A 13-year-old Chinese boy with features of the Madras pattern of motor neuron disease (MMND) presented to us. The benign focal atrophy of the extremities, especially the upper, and associated hearing impairment were important clues to the clinical diagnosis. A recent pathological report suggests that an inflammatory aetiology needs to be considered for this special type of motor neuron disease. Therefore, treatment with intravenous immunoglobulin 400 mg/kg once daily was administered for 5 days, and improvement of symptoms was noted at 6 months of follow up...
May 2004: National Medical Journal of India
M Gourie-Devi, A Nalini
Madras motor neuron disease (MMND) has the characteristic features of onset in the young, atrophy and weakness of the limbs, multiple cranial nerve palsies particularly the seventh, ninth to twelfth and sensorineural hearing loss with unique geographic distribution to southern part of India. During a period of 28 years (1974-2001), 7 (13%) among 54 patients of MMND seen at a tertiary referral center at Bangalore, India, had the additional features of optic atrophy in all and cerebellar involvement in three of them...
May 15, 2003: Journal of the Neurological Sciences
S K Shankar, M Gourie-Devi, L Shankar, T C Yasha, V Santosh, S Das
A neuropathological report of Madras type of motor neuron disease (MMND) is presented and the differences from other forms of MND are discussed. An 18-year-old girl presented with nerve deafness and slowly progressive bulbo-spinal muscular atrophy, characteristic of MMND. Post-mortem examination of the spinal cord showed a severe loss of anterior horn cells, prominent dilatation of vessels, diffuse, but sparse sprinkling of microglial cells and lymphocytes, and demyelination and sclerosis of the ventrolateral columns...
April 2000: Acta Neuropathologica
L Gómez-Fernández, D J Calzada-Sierra, R Macías, I Pedroso
INTRODUCTION: There are scanty reports on juvenile forms of amyotrophic lateral sclerosis, specially amyotrophic lateral sclerosis and deafness, and it is known as Madras pattern of motor neurone disease. CLINICAL CASE: We describe an sporadic case of juvenile amyotrophic lateral sclerosis with deafness in a young person who started with hearing loss at 21 years old, loss of strength in upper limbs and muscular atrophy. He was seen by a neurologist when he was 25 years old, there were evident generalized fasciculation activity in proximal and distal muscles in the four limbs and the tongue, with swallowing troubles, and increased tendon reflexes in lower limbs with abnormal plantar extensor responses...
July 1, 1999: Revista de Neurologia
R Massa, A Scalise, C Iani, M G Palmieri, G Bernardi
We report the case of a young man from the south of India, initially presenting the typical signs of benign monomelic amyotrophy (BMA) in the left upper limb. After several years, the involvement of other limbs and the appearance of bulbar signs suggested the possible diagnosis of the Madras pattern of motor neuron disease (MMND). Serial motor evoked potential (MEP) recordings allowed detection of the onset of a focal involvement of upper motor neurons (UMN) controlling innervation in the originally amyotrophic limb...
December 1998: Electroencephalography and Clinical Neurophysiology
S P Saha, S K Das, P K Gangopadhyay, T N Roy, B Maiti
A clinical study about the pattern of motor neurone disease in eastern India was carried out from July 1993 to June 1995 at Bangur Institute of Neurology, Calcutta and SSKM Hospital, Calcutta. A total of 110 cases were studied and they constituted 0.11% of all neurological cases seen in the general OPD. Of 110 cases, amyotropic lateral sclerosis (ALS) constituted 43.6%, progressive muscular atrophy (PMA) 10.9%, post-polio progressive muscular atrophy (PPMA) 1.8%, spinal muscular atrophy (SMA) 20%, atypical form Madras pattern of MND (MMND) 0...
July 1997: Acta Neurologica Scandinavica
A J Fischman, J W Babich, D R Elmaleh, S A Barrow, P Meltzer, R N Hanson, B K Madras
UNLABELLED: Parkinson's disease is characterized by degeneration of dopamine (DA) neurons and their terminals. Since these neurons contain dopamine transporters (DAT), radioligands that bind to these sites are promising radiopharmaceuticals for diagnosis and therapeutic monitoring of disease progression. We evaluated [123I]-2 beta-carbomethoxy- 3 beta-(4-fluorophenyl)-N-(1-iodoprop-1-en-3-yl)nortropane ([123I]IACFT) for SPECT imaging in an MPTP model of parkinsonism. METHODS: Three rhesus monkeys were imaged before and at 1 and 2 mo after treatment with MPTP...
January 1997: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
K Phanthumchinda, O Supcharoen, E Mitrabukdi
A 38-year-old male farmer with classical features of Madras type motor neuron disease was reported. The benign focal atrophy of extremities especially the upper extremities and associated hearing impairment are important clues to the clinical diagnosis. Although there is no pathologic proof of this subgroup of motor neuron disease our data and previous reports strongly suggest anterior horn cell disease and motor nuclei involvement in the brainstem. The nature of the lesion which is responsible for auditory impairment is still controversial...
June 1996: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
N Deivanayagam, K Nedunchelian, S S Ahamed, T P Ashok, N Mala, S R Ratnam
In order to identify the role of intramuscular injection (IM) as a provoking factor for poliomyelitis, a case control study as done at the Institute of Child Health, Madras from May 1988 to May 1989. The case was defined as acute poliomyelitis if he had acute asymmetric flaccid paralysis of lower motor neurone type without objective sensory disturbance following a short episode of fever. Controls were taken from children attending outpatient department for fever. Two controls matched for aged and sex were recruited for each case...
March 1993: Indian Pediatrics
E Meenakshisundaram, K Jagannathan, B Ramamurthi
No abstract text is available yet for this article.
December 1970: Neurology India
K Valmikinathan, M Mascreen, E Meenakshisundaram, C Snehalatha
A detailed study of four cases of MND-Madras has shown a significantly low plasma citrate level in these subjects. Comparison with other related groups of neurological disorders indicates the citrate/pyruvate ratio to be of diagnostic value in MND-Madras. This seems to suggest that the pathophysiology of MND-Madras may centre round altered citrate metabolism.
October 1973: Journal of Neurology, Neurosurgery, and Psychiatry
B A Summers, M Swash, M S Schwartz, D A Ingram
A girl with rapid-onset, bulbospinal muscular atrophy and deafness is described. The patient's mother and brother showed EMG features consistent with subclinical involvement. T is bulbospinal form of spinal muscular atrophy associated with deafness described by Vialetto and van Laere closely resembles the Madras type of motor neuron disease, also associated with deafness, described by Jagganathan and colleagues.
August 1987: Journal of Neurology
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