Worster-Drought syndrome

Worster-Drought syndrome (WDS) is a mild perisylvian form of cerebral palsy , the main feature of which is pseudobulbar paresis . It is also accompanied by nervous, speech, mental and movement disorders. The associated manifestations also include frustrum spastic quadriparesis, epilepsy , speech disorders, significant cognitive and behavioral disorders.  It is a non-progressive syndrome described by British physician Dr Cecil Charles Worster-Drought.

WDS is not rare and is easily diagnosed. The first symptoms that parents of children with WDS notice are usually related to difficulties in feeding (sucking, biting, chewing disorders), swallowing and controlling the swallowing of saliva (sialoroea). Later, speech problems begin to manifest. Although the first symptoms can be observed from the first year of life, the diagnosis of WDS is usually made much later. The estimated prevalence is 2–3 per 100,000 children, but this number is probably an underestimate.

Etiology

The cause of Worster-Drought syndrome is an abnormality in the perisylvian region, that is, the part of the brain that controls the muscles of the mouth and pharynx . This abnormality is related to the early stage of pregnancy between 12 to 16 weeks. One possible factor appears to be a problem with the blood supply to this area of ​​the brain. The familial incidence is up to 15%.

Clinical picture

Clinical manifestations of WDS include:

• Problems with sucking, swallowing, biting, chewing , which is manifested by difficult feeding with a spoon or drinking from a cup. In more severe forms, there are also problems with the intake of liquid or mushy food (pooping during feeding, aspiration).
• Malnutrition due to feeding difficulties.
• Gastroesophageal reflux .
• Oromotor dysfunction , which includes problems with movements of the tongue, lips, jaw, and palate. Children cannot purse their lips, do not stick their tongue out of their mouths, and have a pathological swallowing pattern.
• Permanent salivation ( sialoroea ) on the basis of difficult control of saliva swallowing.
• Delay in speech development (delay in expression compared to reception), some children do not manage oral communication at all.
• Developmental learning disabilities. These are mainly dyslexia , dysgraphia , dysortography . Intellect is not reduced.
• Mild impairment of gross motor skills - frustum spastic quadriparesis . Children with WDS sit and walk later, are clumsy, and have problems coordinating and organizing hand movements.
• Attention deficit hyperactivity disorder .
• Epilepsy.

The phenotype of WDS overlaps with that of congenital bilateral perisylvian polymicrogyria (CBPP) syndrome.

Diagnostics

An anamnesis and clinical multidisciplinary examination are carried out (by a pediatric neurologist, a clinical speech therapist, a clinical psychologist, a radiologist, a rehabilitation doctor and a physiotherapist). Free paresis of the orbicularis oris muscle , the muscles of the tongue , the muscles of the larynx and pharynx is objectively examined. Increased masseter reflex, lively myotatic reflexes and frustum pyramidal irritation. Furthermore, speech impairment, sialoroea, dysphagia, but a preserved smile.

The doctor also assesses the ability to swallow solid, fragile, mushy and liquid food and possibly an examination of the act of swallowing or an electromyographic examination of swallowing. Other diagnostic methods include EEG and MRI : bilateral perisylvian polymicrogyria, heterotopia, partial agenesis of the corpus callosum , mild brain atrophy, moderate cerebellar atrophy, imperfect differentiation of gray and white matter, increased signal in the capsula externa in T2, small pons and cerebellar asymmetry hemisphere, abnormal signal left frontally at the head of the caudate nucleus.

Therapy

Multidisciplinary comprehensive care focuses on sensory and motor stimulation of the orofacial area, correction of swallowing disorders, speech stimulation and communication training (e.g. adding signs to speech).

Links

References

1. Clark M, Carr L, Reilly S, Neville BGR. Worster-Drought syndrome, a mild tetraplegic perisylvian cerebal palsy. Brain 2000; 123: 2160–2170
2. ↑ Skočit nahoru k:a b c d e TOMANOVÁ, E, J KRAUS a M BROŽOVÁ. Worster-Droughtův syndrom – starý známý?. Neurológia pre prax [online]. 2011, roč. 12, vol. 4, s. 249–250, dostupné také z <http://www.solen.sk/index.php?page=pdf_view&pdf_id=5209>. 
3. ↑ . Worster-Drought C. Congenital suprabulbar paresis. J Laryngol Otol 1956; 70: 453–463.
4. ↑ Skočit nahoru k:a b Clark M, Hartus R, Jollef N, Price K, Neville BGR. Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech. Dev Med Child Neurol 2010; 52(1): 27–32.
5. ↑ Clark M, Chiny WK, Cox T, Neville BGR. Congenital perisylvian dysfunction – is it a spectrum?Dev Med Child Neurol 2010; 52(1): 33–39.
6. ↑ Clark M, Neville BGR. Familial and genetic associations in Worster-Drought syndrome and perisylvian disorders. Am J Med Genet 2007; 146A: 35–42.