Olivopontocerebellar atrophy

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Olivopontocerebellar atrophy Classification and 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 thanks 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. ^[1]

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

External links

• -113967058 at GPnotebook - "olivopontocerebellar atrophy"
• -429195218 at GPnotebook - "lethal olivopontocerebellar atrophy"
• synd/1903 at Who Named It - "Dejerine-Thomas atrophy"
• opca at NINDS
• OPCA Awareness

v • d • e Nervous system pathology, primarily PNS (G50-G99, 350-359)
Nerve, nerve root and plexus disorders (neuropathy, radiculopathy, plexopathy)	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: upper limb (Carpal tunnel syndrome, Ulnar nerve entrapment, Radial neuropathy) - Causalgia - lower limb (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
Diseases of myoneural junction	autoimmune: Myasthenia gravis - Lambert-Eaton myasthenic syndrome
Diseases of muscle (myopathy)	Muscular dystrophy myotonic (Myotonic dystrophy, Myotonia congenita, Thomsen disease, Neuromyotonia, Paramyotonia congenita) congenital myopathy (Centronuclear myopathy, Nemaline myopathy, Central core disease) Mitochondrial myopathy periodic paralysis: Hypokalemic - Hyperkalemic
Autonomic	Familial dysautonomia - Horner's syndrome - Multiple system atrophy (Shy-Drager syndrome, Olivopontocerebellar atrophy)

ja:オリーブ橋小脳萎縮症

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .