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.

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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]

v • d • e 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)

Category: Neurological disorders

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.