Congenital Suprabulbar Palsy
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Introduction[edit | edit source]
Congenital suprabulbar palsy describes an assortment of symptoms occurring in infants and children, which can be caused by a variety of cerebral disorders. The aetiology might be related to "anoxia or ischaemia to the developing brain"[1]. Disorders associated with congential suprabulpar palsy include the Congenital Bilateral Perisylvian Syndrome (CBPS) and Worster-Drought Syndrome (WDS; see below). Congenital suprabulbar palsy diseases are very rare.
Clinic[edit | edit source]
Researchers found that in addition to CBPS, which is a neuronal migration disorder, symmetrical infarcts in the perisylvian regian might also be a possible cause of congenital suprabulbar palsy.[1]
Symptoms[edit | edit source]
Key symptoms are severe dysarthria, drooling, problems of feeding and swallowing. [1] Affected infants and children are not following the normal speech development stages resulting in articulating problems, which is the lead symptom to be found for congenital suprabulbar palsy.
Differential diagnostics[edit | edit source]
The non-congenital form of suprabulbar palsy (progressive suprabulbar palsy or PSP) is a rare disease which can be mistaken for a Parkinson's syndrome, as it occures later in life but shows similar symptoms. [2]
Treatment[edit | edit source]
Treatment should be realized multidisciplinary between paediatrician, speech therapist, psychological education therapists and physiotherapists. There is no specific pharmaceutical treatment, as the impairment can be caused by a multitude of cerebral diseases.
Worster-Drought Syndrome (WDS)[edit | edit source]
One form of congenital suprabulbar palsy is Worstser-Drought Syndrome. It is recognized by "agenesis or hypogenesis of corticobular tracts, affecting voluntary movements of the lips, tongue, soft palate, pharyngeal, and laryngeal muscles, while sparing involuntary activities such as smiling"[1]. With a slight predominance in boys, the prevalence ranges around 1/25,000 to 1/30,000 with a mean diagnosis age of 6.[3] Very early diagnosis proves to be difficult as the impaired development of speech is one of the symptoms.
Relevance in physiotherapy[edit | edit source]
References[edit | edit source]
- ↑ 1.0 1.1 1.2 1.3 Poovathinal A Suresh, Cherukunnathu Deepa. Congenital suprabulbar palsy: a distinct clinical syndrome of heterogeneous aetiology. Developmental Medicine & Child Neurology. 2004; 46:617–625.
- ↑ Johns Hopkins Medicine. Progressive Suprabulbar Palsy. Available from: https://www.hopkinsmedicine.org/health/conditions-and-diseases/progressive-supranuclear-palsy (accessed 2 November 2021).
- ↑ Orphanet, the portal for rare diseases and orphan drugs. Worster-Drought syndrome. Available from:https://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=3046&Disease_Disease_Search_diseaseType=ORPHA&Disease_Disease_Search_diseaseGroup=3465&Disease(s)/group%20of%20diseases=Congenital-suprabulbar-paresis&title=Congenital-suprabulbar-paresis&search=Disease_Search_Simple (last accessed 2 November 2021).
