Congenital Suprabulbar Palsy: Difference between revisions
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== Introduction == | == Introduction == | ||
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"<ref name=":0">Poovathinal A Suresh, Cherukunnathu Deepa. Congenital suprabulbar palsy: a distinct clinical syndrome of heterogeneous aetiology. Developmental Medicine & Child Neurology. 2004; 46:617–625.</ref>. 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 == | |||
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.<ref name=":0" /> | |||
==== Symptoms ==== | |||
Key symptoms are severe dysarthria, drooling, problems of feeding and swallowing. <ref name=":0" /> 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 ==== | |||
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. <ref>Johns Hopkins Medicine. Progressive Suprabulbar Palsy. Available from: https://www.hopkinsmedicine.org/health/conditions-and-diseases/progressive-supranuclear-palsy (accessed 2 November 2021).</ref> | |||
==== Treatment ==== | |||
== | == Worster-Drought Syndrome (WDS) == | ||
One form of congenital suprabulbar palsy is Worstser-Drought Syndrome. It is recognised 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"<ref name=":0" />. With a slight predominance in boys, the prevalence ranges around 1/25,000 to 1/30,000 with a mean diagnosis age of 6.<ref>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). </ref> Very early diagnosis proves to be difficult as the impaired development of speech is one of the symptoms. | |||
== | == Relevance in physiotherapy == | ||
== Resources == | == Resources == | ||
Revision as of 12:42, 2 November 2021
Original Editor - User Name
Top Contributors - Anna Fuhrmann, Kim Jackson and Mila Andreew
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]
Worster-Drought Syndrome (WDS)[edit | edit source]
One form of congenital suprabulbar palsy is Worstser-Drought Syndrome. It is recognised 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]
Resources[edit | edit source]
- bulleted list
- x
or
- numbered list
- x
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).
