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Worster-Drought Syndrome

Congenital Suprabulbar Paresis


Presentation

  • While no significant cortical 11C-PiB retention was present, a high degree of cerebellar 11C-PiB retention was observed in a genetically confirmed carrier of the WDS gene.[ncbi.nlm.nih.gov]
  • Two children with WDS are presented, emphasizing the clinical symptoms, natural history of the disorder, etiology and imaging.[ncbi.nlm.nih.gov]
  • 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]
  • Mild pyramidal tetraplegia, glue ear, recurrent respiratory infections and aspiration, and gastroesophageal reflux may be present in up to 40% of patients. WDS is associated with bilateral lesions of the corticobulbar tract.[orpha.net]
Movement Disorder
  • AIM: Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech, and saliva control owing to a non-progressive disturbance[ncbi.nlm.nih.gov]
  • Photo by kind permission of Breda Oxley Worster-Drought syndrome (WDS) is a type of cerebral palsy (movement disorder) that affects the muscles around the mouth and throat.[laoispeople.ie]
  • Neville Published 2010 in Developmental medicine and child neurology DOI: 10.1111/j.1469-8749.2009.03475.x AIM Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent[semanticscholar.org]
  • Entry H01837 Disease Name Congenital suprabulbar paresis; Worster Drought syndrome Description Congenital suprabulbar paresis (Worster- Drought syndrome) is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing[genome.jp]
  • Developmental Medicine & Child Neurology, v52 n1 p27-32 Jan 2010 Aim: Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech[eric.ed.gov]
Failure to Thrive
  • They tended to experience failure to thrive, gastro-oesophageal reflux (usually requiring surgery) and aspiration and went on to have major expressive language problems when older, needing high levels of augmentative communication support.[doi.org]
Abnormal Eye Movement
  • Six (6/46, 13%) children were thought to have abnormal eye movements (not including those with simple squints). No other additional cranial nerve abnormality was noted.[doi.org]
Long Arm
  • One (1/21) had an abnormal chromosome (fragile site on long arm of chromosome 2).[doi.org]
Contusion
  • Journal of Child Neurology 20(2): 163-165, 2005 Foix-Chavany-Marie syndrome after unilateral anterior opercular contusion: a case report .[eurekamag.com]
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]
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]
Clonus
  • He has generally increased tone with brisk reflexes and ankle clonus. MRI and chromosomes were unremarkable, and EEG showed paroxysmal bursts.[doi.org]
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]
Quadriplegia
  • Reye Síndrome de Rubinstein-Taybi Síndrome de Satos Síndrome de Tourette Síndrome de X frágil Síndrome Di George Síndrome de Wolff Síndrome de Worster-Drought Sclerodermis de Smith-Magenis Smith-Lemli-Opitz-Spastic Diplegia Spastic Paralysis Spastic Quadriplegia[dolphin-therapy.org]
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]
  • 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]
  • Preoperative communication may be difficult, although adult patients with WDS should not be assumed to be incompetent to give consent for treatment [ 5 ].[journals.lww.com]

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]
  • 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]
  • In infants, hydrocephalus presents with head enlargement and symptoms ranging from irritability to seizures, and the prognosis ranges from normal development to severe developmental delay1,2.[the-medical-dictionary.com]

Etiology

  • Two children with WDS are presented, emphasizing the clinical symptoms, natural history of the disorder, etiology and imaging.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • The etiology of the disorder is unknown. Although several families with a high prevalence of the disease have been reported, 1 many other cases appear to be sporadic.[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]
  • Late language emergence at 24 months: an epidemiological study of prevalence, predictors, and covariates. Journal of Speech Language and Hearing Research, 50, 1562-1592.[fhi.no]
Sex distribution
Age distribution

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]
  • 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]
  • Children can develop marked avoidance strategies to prevent having to speak. Stuttering that begins in adulthood is identified with its own diagnostic code.[fhi.no]
  • . & Cole, M.D.(2000) Alstrom syndrome the case for secondary prevention. Diabetes Research and Clinical Practice , 50 (Suppl. 1), S202.Satman, I., Yilmaz, M.T., Gursoy, N., Karsidag, K., Dinccag, N., Ovali, T.[docslide.com.br]

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