Progressive bulbar palsy
| Classification according to ICD-10 | |
|---|---|
| G12.2 |
|
| ICD-10 online (WHO version 2019) | |
Progressive bulbar paralyses (also PBP) are a group of neurological diseases whose common feature is damage to the motor cranial nerve nuclei . The progressive bulbar paralyses belong to the group of so-called spinal muscular atrophies with a special distribution pattern .
A distinction is made between 3 forms: progressive bulbar paralysis in childhood (Fazio-Londe syndrome) , progressive bulbar paralysis with deafness ( Brown-Vialetto-van-Laere syndrome ) and progressive bulbar paralysis in adulthood . The latter is often viewed as a special form of amyotrophic lateral sclerosis .
Nuclear lesions cause bulbar palsy, supranuclear lesions cause pseudobulbar palsy.
Symptoms
The muscles innervated by the cranial nerves ( V , VII , IX , X , XII ) and corticobulbar tracts required during the act of swallowing are predominantly affected, which causes increasing difficulties in chewing, swallowing and speaking . The prognosis is poor, particularly because of the dysphagia . Airway complications from aspiration often result in death within 1 to 3 years.
literature
- Peter Berlit: Clinical Neurology. Springer, Heidelberg 2006, ISBN 978-3-540-01982-4 , p. 554.
Individual evidence
- ^ TAT Hughes, CM Wiles: Neurogenic dysphagia: the role of the neurologist. In: Journal of Neurology, Neurosurgery & Psychiatry. 64, No. 5, 1998, pp. 569-572, doi : 10.1136 / jnnp.64.5.569 , PMID 9598667 .
- ↑ M. Rubin: Amyotrophic Lateral Sclerosis (ALS) and Other Motor Neuron Diseases (MNDs) , in Merck Manual, Professional Version.
- ↑ progressive bulbar paralysis. In: Lexicon of Neuroscience.