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Topic: Monomelic amyotrophy


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In the News (Sat 26 Dec 09)

  
  NEUROLOGICAL DISORDERS - Monomelic Amyotrophy (Benign Focal Amyotrophy, Hirayama Syndrome, O'Sullivan-McLeod Syndrome)
Monomelic Amyotrophy (Benign Focal Amyotrophy, Hirayama Syndrome, O'Sullivan-McLeod Syndrome)
Monomelic amyotrophy (MMA) is characterised by progressive degeneration and loss of motor neurons, the nerve cells in the brain and spinal cord that are responsible for controlling voluntary muscles.
It is characterised by weakness and wasting in a single limb, usually an arm and hand rather than a foot and leg.
www.medic8.com /neurological-disorders/monomelic-amyotrophy.htm   (423 words)

  
  AAPM&R - EMG Case No. 61, cont   (Site not responding. Last check: 2007-10-01)
They included 44 patients in their study who had met criteria for monomelic amyotrophy for a minimum of five years, to a maximum of 23 years (mean 9.7 years, 68% for longer than 10 years).
Monomelic amyotrophy, when first noted in the literature in 1959 (Hirayama et al., 1959), was called "juvenile muscular atrophy of the unilateral upper extremity," and the term, in its general use, refers to upper extremity involvement.
There have been descriptions of a few cases of monomelic amyotrophy of the lower limb (Uncini et al., 1992); however, it is a less well-defined entity than monomelic amyotrophy affecting the upper limb(s).
www.aapmr.org /education/emgcases/emg6103e.htm   (815 words)

  
 Introduction: Monomelic Amyotrophy - WrongDiagnosis.com
Monomelic Amyotrophy: Monomelic amyotrophy (MMA) is a disease of nerves, known as motor neurons, that are responsible for controlling voluntary muscles.
Researching symptoms of Monomelic Amyotrophy: Further information about the symptoms of Monomelic Amyotrophy is available including a list of symptoms of Monomelic Amyotrophy, or alternatively return to research other symptoms in the symptom center.
Treatments for Monomelic Amyotrophy: Various information is available about treatments available for Monomelic Amyotrophy, or research treatments for other diseases.
www.wrongdiagnosis.com /m/monomelic_amyotrophy/intro.htm   (181 words)

  
 eMedicine - Focal Muscular Atrophies : Article by Ramaratnam Sridharan, MD, FRCP, FAAN   (Site not responding. Last check: 2007-10-01)
Monomelic amyotrophy was associated with the 7472 insC mutation in the mtDNA tRNA (Ser(UCN)) gene in one family with a correlation between mutation load and clinical severity.
In some patients with monomelic amyotrophy, MRI demonstrates focal and unilateral atrophy in the lower cervical cord, which is limited to the anterior horn region.
Guglielmo GD, Brahe C, Di Muzio A: Benign monomelic amyotrophies of upper and lower limb are not associated to deletions of survival motor neuron gene.
www.emedicine.com /neuro/topic137.htm   (6906 words)

  
 Monomelic Amyotrophy   (Site not responding. Last check: 2007-10-01)
We must emphasize that no sourcebook on monomelic amyotrophy should affirm that a specific diagnostic procedure or treatment discussed in a research study, patent, or doctoral dissertation is “correct” or your child’s best option.
When their child has been diagnosed with monomelic amyotrophy, parents will often log on to the Internet, type words into a search engine, and receive several Web site listings which are mostly irrelevant or redundant.
Because of this, far less research is conducted on monomelic amyotrophy compared to other health problems afflicting larger populations, like breast cancer or heart disease.
www.icongrouponline.com /health/Monomelic_Amyotrophy.html   (1590 words)

  
 eMedicine - Focal Muscular Atrophies : Article Excerpt by: Ramaratnam Sridharan, MD, FRCP, FAAN   (Site not responding. Last check: 2007-10-01)
Monomelic amyotrophy has been reported more often in India, Korea, and Japan than in other countries.
In a hospital-based study from India, among 110 patients with anterior horn cell disease, 10.9% had progressive muscular atrophy; 1.8%, PPMA; and 22.7%, monomelic amyotrophy.
Age: Disorders such as polio and monomelic amyotrophy are more common in younger people.
www.emedicine.com /neuro/byname/focal-muscular-atrophies.htm   (442 words)

  
 Monomelic Amyotrophy Information Page: National Institute of Neurological Disorders and Stroke (NINDS)
Monomelic Amyotrophy Information Page: National Institute of Neurological Disorders and Stroke (NINDS)
You are here: Home > Disorders > Monomelic Amyotrophy
Synonym(s): Benign Focal Amyotrophy, Hirayama Syndrome, O'Sullivan-McLeod Syndrome
www.ninds.nih.gov /health_and_medical/disorders/monomelic_amyotrophy.htm   (448 words)

  
 Symptoms of Monomelic Amyotrophy - CureResearch.com
- Misdiagnosis of Underlying Causes of Monomelic Amyotrophy
Furthermore, symptoms of Monomelic Amyotrophy may vary on an individual basis for each patient.
Symptoms of Monomelic Amyotrophy: It is characterized by weakness and wasting in a single limb, usually an arm and hand rather than a foot and leg.
www.cureresearch.com /m/monomelic_amyotrophy/symptoms.htm   (393 words)

  
 MR Imaging of the cervical cord in juvenile amyotrophy of distal upper extremity -- Biondi et al. 10 (2): 263 -- ...
MR Imaging of the cervical cord in juvenile amyotrophy of distal upper extremity -- Biondi et al.
MR Imaging of the cervical cord in juvenile amyotrophy of distal upper extremity
known as monomelic amyotrophy or benign focal amyotrophy, is distinct from
www.ajnr.org /cgi/content/abstract/10/2/263   (336 words)

  
 Evincii: Medical Search   (Site not responding. Last check: 2007-10-01)
Postradiation lower motor neuron syndrome presenting as monomelic...
Postradiation lower motor neuron syndrome presenting as monomelic amyotrophy.
and monomelic amyotrophy are more common in younger people.
www.evincii.com /~julien/cache/monomelic_amyotrophy.html   (191 words)

  
 ALS References 2003
OBJECTIVES -: Monomelic amyotrophy of a single upper limb termed "brachial monomelic amyotrophy" (BMMA) is a benign lower motor neuron disorder in the young, with male preponderance, insidious onset of atrophy and weakness, electromyographic evidence of neurogenic pattern without conduction block, slow progression for 2-4 years followed by a stationary course.
The aim of the study was to determine whether (i) atrophy and weakness in the affected limb progresses beyond 5 years; (ii) the illness spreads to the other limbs; and (iii) the disease progresses to amyotrophic lateral sclerosis.
The proband was a 68-year-old woman with an 8-year history of parkinsonism which was followed by psychiatric symptoms and neurogenic amyotrophy 5 years after the onset.
lansbury.bwh.harvard.edu /als_references_2003.htm   (15150 words)

  
 Indian Journal of Medical Research: Clinical & genetic analysis of four patients with distal upper limb spinal ...   (Site not responding. Last check: 2007-10-01)
Di Bella P, Logullo F, Tulli D, Ragno M, Scarpelli M. Benign monomelic amyotrophy: a description of a patient with a focal motor neuron disorder.
Biondi A, Dormont D, Weitzner I Jr, Bouche P, Chaine P, Bories J. MR imaging of the cervical cord in juvenile amyotrophy of distal upper extremity.
Benign focal amyotrophy: rnetrizart-ride CT evidence of cord atrophy.
www.findarticles.com /p/articles/mi_qa3867/is_200110/ai_n8961366/pg_2   (1224 words)

  
 ALS-nyc.org Template Book page
The unique neuropathologic findings include anterior horn cell degeneration producing muscle atrophy or amyotrophy and degeneration and sclerosis of the corticospinal tracts.
The signs of progressive bulbar palsy and bulbar-onset ALS reflect predominant motor neuron loss in the brainstem, supplying the lingual and pharyngeal muscles that leads to early or predominant dysarthria, dysphagia, and respiratory insufficiency (2).
MND localized to a single limb is termed monomelic amyotrophy.
www.als-nyc.org /ch32a.html   (836 words)

  
 Amyotrophy   (Site not responding. Last check: 2007-10-01)
The Official Parent's Sourcebook on Monomelic Amyotrophy (reference)
Monomelic amyotrophy (MMA) is a disease of nerves, known as motor neurons, that are responsible for controlling voluntary muscles.
In PLS, there is no evidence of the degeneration of spinal motor neurons or muscle wasting (amyotrophy) that occurs in a similar disorder called amyotrophic lateral sclerosis or ALS (Lou Gehrig's disease).
www.websters-online-dictionary.org /am/amyotrophy.html   (258 words)

  
 The Official Parent's Sourcebook on Monomelic Amyotrophy: A Revised and Updated Directory for the Internet Age: Current ...
Collectively, the material presented in Part I is a complete primer on basic research topics for patients with monomelic amyotrophy.
While this sourcebook covers monomelic amyotrophy, your doctor, research publications, and specialists may refer to your condition using a variety of terms.
Therefore, you should understand that monomelic amyotrophy is often considered a synonym or a condition closely related to the following:.
seo-toys.com /seo-books/free.php?&asin=0597830371   (982 words)

  
 Diagnosis & Treatment of Motor Neuropathies
Benign, focal motor neuron disorders, such as monomelic amyotrophy, were subsequently reported.
These syndromes were usually considered as variants of ALS, as early pathological studies suggested that the primary focus of the disease was on cell bodies in the ventral horn.
Monomelic amyotrophy, a syndrome that affects mainly young (15 to 25 years) males (80%), presents with weakness of the distal musculature of one upper extremity that progresses for 1 to 2 years and then remains stable.
www.neuro.wustl.edu /neuromuscular/antibody/motpn2.htm   (3658 words)

  
 Hereditary Motor Syndromes
Onset with monomelic leg weakness suggests familial ALS
Amyotrophy: Mild; Distal; Legs > Arms; Increases with age
Cerebellar signs with distal amyotrophy in later onset (Types II and III)
www.neuro.wustl.edu /neuromuscular/synmot.html   (3032 words)

  
 Basic Summary for Monomelic Amyotrophy - WrongDiagnosis.com
Parent types of Monomelic Amyotrophy: Motor neuron disease, Motor neuron diseases
Prognosis of Monomelic Amyotrophy: The symptoms of MMA usually progress slowly for one to two years before reaching a plateau, then remain stable for many years.
There is also a slowly progressive variant of MMA known as O'Sullivan-McLeod syndrome, which affects the small muscles of the hand and forearm and has a slowly progressive course.
www.wrongdiagnosis.com /m/monomelic_amyotrophy/basics.htm   (239 words)

  
 [No title]   (Site not responding. Last check: 2007-10-01)
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).
Therefore, serial MEP recordings can be useful for the early detection of sub-clinical UMN damage in motor neuron disease presenting with pure lower motor neuron (LMN) signs.
www.elsevier.com /cdweb/journals/0924980X/articles/109/6/S138824579800029.abstract.en   (109 words)

  
 The Official Parent's Sourcebook on Monomelic Amyotrophy: A Revised and Updated ...
The Official Parent's Sourcebook on Monomelic Amyotrophy: A Revised and Updated...
Although it gives information useful to doctors, caregivers and other health professionals, it also tells parents where and how to look for information covering virtually all topics related to monomelic amyotrophy, from the essentials to the most advanced areas of research.
The Essentials on Monomelic Amyotrophy: Guidelines; Chapter 2.
goldbamboo.com /store-1detail-2B0007YT5H8.html   (486 words)

  
 Arch Neurol -- Abstract: Monomelic amyotrophy, April 1984, Gourie-Devi et al. 41 (4): 388
Arch Neurol -- Abstract: Monomelic amyotrophy, April 1984, Gourie-Devi et al.
From 1977 through 1981, we examined 23 patients with single-limb atrophy.
Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity
archneur.ama-assn.org /cgi/content/abstract/41/4/388   (185 words)

  
 Catalogo Articoli   (Site not responding. Last check: 2007-10-01)
We report here magnetic resonance imaging (MRI) findings of two patients with benign monomelic amyotrophy of lower limb.
Both subjects showed unilateral amyotrophy of the tower limb with a benign clinical course, and the affected muscles demonstrated neurogenic changes.
We concluded that muscle MRI is very useful for detecting affected muscles, especially deep skeletal muscles in patients with benign monomelic amyotrophy of lower limb.
serials.cib.unibo.it /cgi-ser/start/it/spogli/df-s.tcl?prog_art=1908464&language=ITALIANO&view=articoli   (247 words)

  
 AAPM&R - October 1998 EMG Case   (Site not responding. Last check: 2007-10-01)
A central process is less likely given symptomatology in one limb only unless there is a focal lesion of the spinal cord at C5 and C6.
Myopathy or monomelic amyotrophy is unlikely given that the patient has sensory changes.
There is minimal atrophy of the left deltoid muscle and no scapular winging.
www.aapmr.org /education/archive/emg9810a.htm   (814 words)

  
 Indian Pediatrics - Editorial
Therefore, a diagnosis of juvenile muscular atrophy of distal upper extremity (Hirayama Disease) was made mainly by a process of exclusion.
Juvenile asymmetric segmental spinal muscular atrophy (JASSMA), Monomelic amyotrophy, and Juvenile muscle atrophy of unilateral upper extremity are some other terms used for this condition.
Familial occurrence is rare and the onset is insidious with muscular weakness and wasting in the hand and forearm sparing the brachioradialis muscle.
www.indianpediatrics.net /aug2003/aug-780-783.htm   (1859 words)

  
 monomelic amyotrophy   (Site not responding. Last check: 2007-10-01)
This response submitted by Dr. Barbara Shapiro on 8/4/95.
Monomelic amyotrophy, which has several other names including
benign focal amyotrophy, and Sobue's syndrome, is a rare
neuro-www.mgh.harvard.edu /neurowebforum/NeuromuscularDisordersArticles/monomelicamyotrophy.html   (131 words)

  
 IngentaConnect Sympathetic skin response in monomelic amyotrophy   (Site not responding. Last check: 2007-10-01)
Objectives – Monomelic amyotrophy (MMA) a variant of motor neuron disease, has the characteristic features of wasting and weakness usually confined to a single upper or lower limb occurring predominantly in young males and a benign outcome.
Symptoms of increased sweating, coldness and cyanosis have been observed in a few patients.
Keywords: autonomic nervous system; monomelic amyotrophy; sympathetic skin response
www.ingentaconnect.com /content/mksg/ane/2001/00000104/00000003/art00016   (398 words)

  
 NIMHANS Home Page
Descriptive epidemiological studies focused on the prevalence and pattern of mental and neurological disorders in the rural and urban communities
Analytical case control studies on monomelic amyotrophy, head injuries, hot water epilepsy and stroke for identifying potential risk factors in the causation of these disorders.
Health policy oriented Interventional research in the area of injury prevention by enabling policy makers for helmet legislation on safety of motorcycle occupants.
www.nimhans.kar.nic.in /epidemiology.htm   (523 words)

  
 Thieme-connect - Abstract   (Site not responding. Last check: 2007-10-01)
In addition, certain motor syndromes, such as monomelic amyotrophy, postpolio muscular atrophy, and multifocal motor neuropathy, can clinically mimic ALS/MND.
Therefore, not only may the diagnosis of ALS/MND be clinically missed in the early stages, but worse, the patient may be wrongly labeled as having ALS/MND.
Atypical amyotrophic lateral sclerosis - motor neuron disease - progressive muscular atrophy - spinal muscular atrophy - postpolio muscular atrophy - monomelic amyotrophy - lathyrism - dementia
www.thieme-connect.com /ejournals/abstract/sin/doi/10.1055/s-2001-15263   (333 words)

  
 Treatments for Monomelic Amyotrophy - WrongDiagnosis.com
Treatment list for Monomelic Amyotrophy: The list of treatments mentioned in various sources for Monomelic Amyotrophy includes the following list.
Always seek professional medical advice about any treatment or change in treatment plans.
Treatments of Monomelic Amyotrophy discussion: There is no cure for MMA.
www.wrongdiagnosis.com /m/monomelic_amyotrophy/treatments.htm   (152 words)

  
 Monomelic Amyotrophy
Monomelic Amyotrophy Information Page National Institute of Neurological Disorders and Stroke
DISCLAIMER: NOAH is an information guide only and cannot answer personal health-related or research questions.
All medical information needs to be carefully reviewed with your health care provider.
www.noah-health.org /en/bns/disorders/other/monomelic.html   (87 words)

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