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Progressive muscular atrophy (PMA) is a rare subtype of Motor neurone disease (MND) which affects only the lower motor neurones. This is contrast to the most common form of MND, amyotrophic lateral sclerosis, which affects both the upper and lower motor neurones.
As a result of lower motor neurone degeneration, the symptoms of PMA include:
However, in contrast to amyotrophic lateral sclerosis or primary lateral sclerosis, PMA is distinguished by the absence of:
To be diagnosed with PMA one must have the LMN symptoms described above in the absence of UMN symptoms for a given period of time. Some authors recommend 3 years, others 5 years or even up to 7 years. However it is worth bearing in mind that PMA can progress into typical ALS at any time, even as much as 26 years after the initial diagnosis.
The significance of diagnosing PMA as opposed to ALS is twofold. Crucially, the prognosis is better. The typical prognosis for ALS is approximately 2-5 years after initial diagnosis. In PMA survival is in the order of 5-10 years. More recently evidence has shown that patients with PMA do not suffer from the cognitive change identified in at least a subgroup of patients with ALS.
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| Pathology of the nervous system, primarily CNS (G00-G47, 320-349) |
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Inflammatory diseases
of the CNS | Meningitis (Arachnoiditis) - Encephalitis - Myelitis - Encephalomyelitis (Acute disseminated) - Tropical spastic paraparesis |
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Systemic atrophies
primarily affecting the CNS | Huntington's
Spinocerebellar ataxia (Friedreich's ataxia, Ataxia telangiectasia, Hereditary spastic paraplegia)
Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio Londe - MND (ALS, PMA, PBP, PP, PLS) |
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Extrapyramidal and
movement disorders | Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration
Dystonia/Dyskinesia (Spasmodic torticollis, Meige's, Blepharospasm)
Essential tremor - Myoclonus - Lafora
Chorea (Choreoathetosis) - Restless legs - Stiff person |
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Other degenerative /
demyelinating diseases | Alzheimer's - Pick's - Alpers' - Dementia with Lewy bodies - Leigh's
demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis |
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| Seizure/epilepsy | Focal (Simple partial, Complex partial) - Generalised (Tonic-clonic, Absence, Atonic, Benign familial neonatal)
Lennox-Gastaut - West - Epilepsia partialis continua - Status epilepticus (Complex partial status epilepticus) |
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| Headache | Migraine (Familial hemiplegic) - Cluster - Vascular - Tension |
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| Vascular | Transient ischemic attack (Amaurosis fugax, Transient global amnesia)
Cerebrovascular disease (MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke) |
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| Sleep disorders | Insomnia - Hypersomnia - Sleep apnea (Ondine's curse) - Narcolepsy - Cataplexy - Kleine-Levin - Circadian rhythm sleep - Delayed sleep phase - Advanced sleep phase |
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| Other | Intracranial hypertension: Hydrocephalus (Normal pressure) - Idiopathic intracranial hypertension
Encephalopathy - Brain herniation - Cerebral edema - Reye's - Syringomyelia - Syringobulbia - Spinal cord compression |
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