Corticobasal degeneration
The corticobasal (or corticobasal ) degeneration (CBD ) is a slowly progressive neurodegenerative disorder characterized by accumulation of hyperphosphorylated tau protein in the brain ( tauopathy ). The main symptoms are Parkinson's symptoms and loss of cognitive abilities.
Classification
Atypical parkinsonian syndromes are diseases that are easily confused with Parkinson 's disease. The Corticobasal degeneration after multiple system atrophy (MSA ) and progressive supranuclear palsy (PSP ) is the third most common atypical parkinsonian syndrome.
Frequency and Causes
The CBD is a rare disease. It is estimated that less than 1 in 100,000 people suffering from this disease. As one of the trigger the gene coding for the tau protein MAPT gene could be identified.
Symptoms / Diagnosis
On the one dominating complex cortical ( cerebral cortex concerned ) symptoms such as apraxia. It comes to cognitive decline, which leads into a subcortical dementia. The tendon reflexes are typically increased and myoclonus before coming. As a very characteristic sign a strangeness experience of one's own limbs ( alien limb) is described.
Among the extrapyramidal motor symptoms are usually unilateral onset of Parkinson's symptoms such as rigidity and hypokinesia, rare determine a resting tremor. It comes to dystonia of the neck or limbs and a severe gait disturbance.
Optional Symptoms include eye movement disorders and often in the context of dementia judgmental mood disorder such as depression or generalized anxiety.
The diagnosis is made ( " clinical " ) based on the symptoms. To their backup a histological (histological ) examination of the brain would be necessary. However, since there is no sufficient consequence for the patient's treatment, a biopsy is not performed in his lifetime. In specialized centers, a further allocation by means of special SPECT is possible. However, this has practically only makes sense in the context of research and not for the care of patients.
Neuropathology
On examination of the brain an often asymmetric atrophy of the cerebral cortex, especially the Regiones is neuropathologically precentral and postcentral detectable. The substantia nigra is paler. Microscopically, the affected cortex appears narrowed and has nerve cell sunsets on. Typical are ballooned neurons. Immunohistochemistry deposits of hyperphosphorylated tau protein in nerve and glial cells are detectable. In addition to neurofibrillary tangles are found neuropil, oligodendroglial " coiled bodies " and astrocytic plaques.
Treatment course
The disease can not be cured or caused to stop. Levodopa sometimes leads to an improvement in Parkinson's symptoms, but the response is usually unsatisfactory. The clinical course is usually characterized by increasing movement disorders with akinesia. Elapse from diagnosis to death between 1-10 years; the cause of death is frequently a ( by lack of exercise favored ) pneumonia.