Olivopontocerebellar atrophy

Olivopontocerebellar atrophy
Other names	Multiple system atrophy – cerebellar subtype[1]
Sagittal section through right cerebellar hemisphere. The right olive has also been cut sagittally.
Specialty	Neurology

Olivopontocerebellar atrophy (OPCA) is the degeneration of neurons in specific areas of the brain – the cerebellum, pons, and inferior olivary nucleus.^[2] OPCA is present in several neurodegenerative syndromes, including inherited and non-inherited forms of ataxia (such as the hereditary spinocerebellar ataxia known as Machado–Joseph disease) and multiple system atrophy (MSA), with which it is primarily associated.^[2]

OPCA may also be found in the brains of individuals with

The term was originally coined by Joseph Jules Dejerine and André Thomas.^[3][4]

Signs and symptoms

OPCA is characterized by progressive

A few non-hereditary diseases formerly categorized as olivopontocerebellar atrophy have been reclassified as forms of multiple system atrophy^[10] as well as to four hereditary types, that have been currently reclassified as four different forms of spinocerebellar ataxia:

Diagnosis

A diagnosis of olivopontocerebellar atrophy (OPCA) may be based on a thorough medical exam; the presence of signs and symptoms; imaging studies; various laboratory tests; and an evaluation of the family history.^[11]

MRI of the brain may show characteristics of OPCA, such as specific changes in the size of affected parts of the brain. This is more likely as the disease progresses; it is possible to have OPCA and have a normal brain MRI (especially within the first year of symptom onset).^{[citation needed]}

Hereditary OPCA may be suspected based on having a family history, and may be diagnosed by genetic testing (when available) for the condition suspected or known to be present in the family. Sporadic OPCA may be diagnosed if hereditary forms of OPCA, and other conditions associated with OPCA, have been ruled out.^{[citation needed]}

Treatment

Overground harness systems may be used to allow OPCA patients to challenge their balance without chance of falling. Furthermore, home exercise programs and/or aquatic exercises are used to allow more repetitions to facilitate balance learning. Treatment programs should be frequently monitored and adjusted based on a patient's progress. Outcome measures such as the Berg Balance Scale, Dynamic Gait Index and activities-specific balance confidence scales are useful to assess patient's progress over time.^[5]

See also

• Olivopontocerebellar atrophy-deafness syndrome

References