Corticobasal degeneration
| Classification according to ICD-10 | |
|---|---|
| G31.0 | Localized brain atrophy |
| ICD-10 online (WHO version 2019) | |
The corticobasal (or corticobasal) degeneration is (CBD) is a slowly progressive neurodegenerative disease hyperphosphorylated with collection of tau protein in the brain ( tauopathy ). The main symptoms are Parkinson 's and deterioration in cognitive abilities .
classification
Atypical Parkinson's syndromes are diseases that can easily be confused with Parkinson's disease. After multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), corticobasal degeneration is the third most common atypical Parkinson's syndrome.
Frequency and causes
CBD is a rare disease. It is estimated that less than 1 in 100,000 people will get it. As one of the triggers for the could tau protein coding MAPT - gene are identified.
Symptoms / diagnosis
On the one hand, complex cortical ( affecting the cerebral cortex ) symptoms such as apraxia dominate . There is a decline in cognitive performance, which leads to subcortical dementia . The muscle reflexes are typically increased and myoclonus occur. An experience of strangeness in one's own extremities ( alien limb ) is described as a very characteristic sign .
Among the extrapyramidal motor symptoms are usually unilateral Parkinson's symptoms such as rigor and hypokinesis , and more rarely a resting tremor . It comes to dystonia of the neck or limbs and severe gait disturbance.
Facultative symptoms are eye movement disorders and affective disorders such as depression or generalized anxiety that must often be assessed in the context of dementia .
The diagnosis is based on symptoms ("clinical"). A histological examination of the brain would be necessary to confirm this. However, since there is no sufficient consequence for the treatment of the patient, a biopsy is not carried out during his lifetime. In specialized centers, further allocation is possible using a special SPECT . In practice, however, this only makes sense in the context of research and not for patient care.
Neuropathology
Examination of the brain reveals an often asymmetrical atrophy of the cerebral cortex, especially of the pre-central and post-central regions. The substantia nigra is pale. Microscopically, the affected cerebral cortex appears narrowed and shows nerve cell losses. Ballooned nerve cells are typical. Deposits of hyperphosphorylated tau protein in nerve and glial cells can be detected immunohistochemically . In addition to neurofibrillary tangles, there are neuropil threads, oligodendroglial “coiled bodies” and astrocytic plaques.
Treatment, course
The disease cannot be cured or stopped. Levodopa sometimes improves Parkinson's symptoms, but the response is usually unsatisfactory. The clinical course is usually characterized by increasing movement disorders with akinesia . It takes between 1 and 10 years from diagnosis to death; The cause of death is often pneumonia (favored by a sedentary lifestyle) .
Individual evidence
- ↑ UniProt P10636
- ↑ TH Bak, LM Crawford, VC Hearn, PS Mathuranath, JR Hodges: Subcortical dementia revisited: similarities and differences in cognitive function between progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and multiple system atrophy (MSA). In: Neurocase. 2005; 11 (4), pp. 268-273.
- ^ M. Tolnay, W. Paulus: Systematrophien. In: W. Paulus, JM Schröder (Ed.): Neuropathology. 3. Edition. Springer Verlag, Heidelberg 2012, ISBN 978-3-642-02323-1 .
- ↑ GK Wenning, I. Litvan, J. Jankovic, R. Granata, CA Mangone, A. McKee, W. Poewe, K. Jelling, K. Ray Chaudhuri, L. D'Olhaberriague, RK Pearce: Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. In: Journal of Neurology, Neurosurgery, and Psychiatry . Volume 64, Number 2, February 1998, pp. 184-189, ISSN 0022-3050 . PMID 9489528 . PMC 2169933 (free full text).
