Presentation
It concerns a child of 12 years, diagnosed as having congenital suprabulbar paresis (Worster-Drought syndrome) who presents with severe developmental dysarthria; his speech is unintelligible, with hypernasality and glottalised articulation. [ncbi.nlm.nih.gov]
Another large group present predominantly with speech delay. Persistent dribbling is very common. [doi.org]
The signs and symptoms of Worster-Drought Syndrome may include: Drooling Dysarthria EEG abnormality Very frequently present symptoms in 80-99% of the cases: Abnormality of the cranial nerves Neurological speech impairment Frequently present symptoms in [dovemed.com]
Jaw & Teeth
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Periodontitis
Such children are often prone to teeth and periodontal problems. Even worse is the development of Addressee for Correspondence: Dr. Praveen Kumar Jayakumar. MDS Dr. [docksci.com]
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Poor Oral Hygiene
The patient discussed here too had such signs and a resultant poor oral hygiene. The soft tissue tumor on the commissure was an additional issue to the existing problems such as lip biting and drooling of saliva due to uncoordinated jaw movements. [docksci.com]
Urogenital
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Vaginal Bleeding
Possible aetiological factors Four (4/45, 9%) of the mothers had small vaginal bleeds in early pregnancy and 3/45 (7%) had some hypertension towards the end. However, 41/45 (91%) went on to have a normal vaginal delivery. [doi.org]
Neurologic
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Nasal Speech
He stated that the child should always have a normal smile, but would have variable difficulty in moving the tongue, soft palate and lips, with dysarthric slurred nasal speech, dribbling and a brisk jaw jerk. [doi.org]
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Clonus
He has generally increased tone with brisk reflexes and ankle clonus. MRI and chromosomes were unremarkable, and EEG showed paroxysmal bursts. [doi.org]
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Neonatal Seizures
Seizures Three (3/47, 6%) had possible neonatal seizures (one secondary to apnoeas, one due to hypocalcaemia, and one had an unexplained eye-rolling episode at 12 days). Four (4/47, 9%) had febrile fits. [doi.org]
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Neglect
Ph: 91 94449 43502 Email: [email protected] 2015 JMLL, Tokyo, Japan pathological lesions in such neglected mouth, because they are seldom noticed early and even if identified are difficult to be treated in an unconditioned child. [docksci.com]
Treatment
GH has been successful in the treatment of her short stature. [ncbi.nlm.nih.gov]
Treatment : How to Treat "worster-drought syndrome"? Treatment of the syndrome depends on the degree of the feeding & speech problems. Therapies are also advised. [signssymptoms.org]
Treatment Treatment of the syndrome depends on the degree of the feeding and speech problems. Therapies are also advised. [medigest.uk]
and in some cases, due to treatment also. [dovemed.com]
The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]
Prognosis
Speech prognosis is particularly poor. Early diagnosis with appreciation of the underlying neurology would encourage critical evaluation of interventions and long-term planning to improve outcome. [ncbi.nlm.nih.gov]
(Outcomes/Resolutions) The prognosis of Worster Drought Syndrome is dependent upon the severity of the signs and symptoms and associated complications, if any Individuals with mild conditions have better prognosis than those with severe symptoms and complications [dovemed.com]
Prognosis WDS has a non-progressive course. The overall prognosis is good when nutritional problems, epilepsy and respiratory complications are properly managed. The documents contained in this web site are presented for information purposes only. [orpha.net]
Other inconsistencies and contradictions in the literature are regularly found, particularly in regard to age of onset, predilection for one sex, course, prognosis, and treatment. [jamanetwork.com]
Etiology
Worster-Drought syndrome is a clinical entity resulting from cortico-nuclear tract or cerebral cortex injury, without defined etiology, characterized by alteration of the muscles that control the lips, jaw, tongue, soft palate and pharynx. [pubfacts.com]
Two children with WDS are presented, emphasizing the clinical symptoms, natural history of the disorder, etiology and imaging. [ncbi.nlm.nih.gov]
(Etiology) The exact cause of Worster Drought Syndrome is not known at the present time Research scientists believe that the disorder may develop from both genetic and environmental factors that are currently unknown The inherited form of Worster Drought [dovemed.com]
Various etiologies of opercular syndrome are suggested, including perinatal insult, cardiovascular disease, infections, and other inflammatory causes. [ajnr.org]
The literature on the etiology of meralgia paresthetica is particularly obfuscating. [jamanetwork.com]
Epidemiology
WDS is startlingly absent from epidemiological studies of the cerebral palsies and rarely diagnosed, presumably because of lack of clinical awareness of the condition and lack of major gross motor impairments. [ncbi.nlm.nih.gov]
Authors: Camila Vila-Nova Division of Neurology and Epidemiology (DINEP)-Federal University of Bahia Brazil Arq Neuropsiquiatr 2004 Sep 5;62(3B):906-10. Epub 2004 Oct 5. Universidade Federal da Bahia, Salvador BA, Brazil. [pubfacts.com]
Summary Epidemiology Prevalence is estimated at around 1/25,000-30,000, with a male predominance (male to female sex ratio: 3:1). Clinical description Mean age at diagnosis is 6 years. [orpha.net]
Epidemiology of cerebral palsy in England and Scotland 1984–9. Arch Dis Child Fetal Neonatal Ed 1998 ; 79 : F21 –5. Reilly S, Skuse D, Poblete X. [doi.org]
Pathophysiology
Overview In this article, the author explains the clinical presentation and pathophysiology of Foix-Chavany-Marie syndrome (bilateral anterior opercular syndrome). [medlink.com]
It is possible that our patients had both communicating and obstructive hydrocephalus pathophysiology; however, we did not assess CSF outflow resistance. [the-medical-dictionary.com]
The aphasia appears to be the result of an underlying pathophysiology causing the a... more Acquired aphasia with convulsive disorder is an unusual condition in childhood, characterized by loss of language function associated with a paroxysmal electroencephalogram [scinapse.io]
Report of threecases with further delineation of the clinical, pathophysiological, andgenetic aspects of the disorder. Medicine, 52, 5371.Hearn, T., Renforth, G.L., Spalluto, C., Hanley, N.A., Piper, K. [docslide.com.br]
Prevention
A child with WDS usually has a severe speech delay which can even prevent them from developing all early speech. They may have persistent dribbling (although there is usually some improvement.) [specialeducationalneeds.co.uk]
It prevents them from having normal Nanobots injected into their bodies because they are seen as an infection and they are attacked by the body. [jahikuyujuhes.ga]
We hope that awareness of SHYMA will increase the likelihood of early diagnosis and treatment, and prevent loss of employment or disability. [the-medical-dictionary.com]
Training in closing the mouth will help prevent deformities of the lower jaw. [neuroradiologycases.com]