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Olivopontocerebellar atrophy
Olivopontocerebellar atrophy
Classification & external resources
| ICD-10 |
G23.8[1] |
| ICD-9 |
333.0 |
| DiseasesDB |
2012 9208 |
| MedlinePlus |
000758 |
| eMedicine |
neuro/282 |
| MeSH |
D009849 |
Olivopontocerebellar atrophy (OPCA) is a term used to define neuronal degeneration in the cerebellum, pontine nuclei, and inferior olive. The use of the term has changed considerably in recent years due to the progressing knowledge of the genetic bases of the disease.
Sporadic OPCA
Sporadic (non-hereditary) OPCA is considered a form of multiple system atrophy. [2]
Hereditary OPCA
Four types of hereditary OPCA have disappeared from medical nomenclature, as they have been found to be the same as an already classified form of spinocerebellar atrophy. Type 2, autosomal recessive and Type 5 are still classified as olivopontocerebellar atrophy, though when their genetic associations are identified they may be renamed or combined with other conditions.
| OPCA number |
OPCA name |
SCA # |
Gene |
OMIM |
| OPCA type 1 |
"Menzel type OPCA" |
SCA1 |
ATXN1 |
164400 |
| OPCA type 2, autosomal dominant |
"Holguin type OPCA" |
SCA2 |
ATXN2 |
183090 |
| OPCA type 2, autosomal recessive |
"Fickler-Winkler type OPCA" |
none known |
? |
258300 |
| OPCA type 3 |
"OPCA with retinal degeneration" |
SCA7 |
ATXN7 |
164500 |
| OPCA type 4 |
"Schut-Haymaker type OPCA" |
SCA1 |
ATXN1 |
164400 |
| OPCA type 5 |
"OPCA with dementia and extrapyramidal signs" |
none known |
? |
164700 |
References
- ^ http://www.nzhis.govt.nz/publications/newsletters/coders36.pdf
- ^ [1]
|
Nervous system pathology, primarily PNS (G50-G99, 350-359) |
Nerve, nerve root
and plexus disorders |
cranial nerve: V (Trigeminal neuralgia) - VII (Facial nerve paralysis, Bell's palsy, Melkersson-Rosenthal syndrome, Central seven) - XI (Accessory nerve disorder)
nerve root and plexus: Brachial plexus lesion - Thoracic outlet syndrome - Phantom limb
mononeuropathy: Carpal tunnel syndrome - Ulnar nerve entrapment - Radial neuropathy - Causalgia - Meralgia paraesthetica - Tarsal tunnel syndrome - Morton's neuroma - Mononeuritis multiplex |
Polyneuropathies
and other disorders of the PNS |
Hereditary and idiopathic (Charcot-Marie-Tooth disease, Dejerine Sottas syndrome, Refsum's disease, Morvan's syndrome) - Guillain-Barré syndrome - Alcoholic polyneuropathy - Neuropathy |
Diseases of myoneural junction
and muscle |
Myasthenia gravis - Primary disorders of muscles (Muscular dystrophy, Myotonic dystrophy, Myotonia congenita, Thomsen disease, Neuromyotonia, Paramyotonia congenita, Centronuclear myopathy, Nemaline myopathy, Mitochondrial myopathy) - Myopathy - Periodic paralysis (Hypokalemic, Hyperkalemic) - Lambert-Eaton myasthenic syndrome |
| Autonomic |
Familial dysautonomia - Horner's syndrome - Multiple system atrophy (Shy-Drager syndrome, Olivopontocerebellar atrophy) |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Olivopontocerebellar_atrophy". A list of authors is available in Wikipedia.
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