<%server.execute "isdev.asp"%> Profile of Worster Drought Syndrome (WDS): Unrecognized subtype of cerebral palsy—From tertiary care center in South India Patil A, Gowda VK, Shivappa SK, Benakappa N - J Pediatr Neurosci
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Year : 2022  |  Volume : 17  |  Issue : 1  |  Page : 17-22

Profile of Worster Drought Syndrome (WDS): Unrecognized subtype of cerebral palsy—From tertiary care center in South India

1 Mahadevappa Rampure Medical College (MRMC), Kalaburagi, Karnataka, India
2 Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, India
3 Department of Pediatric Medicine, Indira Gandhi Institute of Child Health, Bengaluru, India

Date of Submission18-May-2020
Date of Decision04-Jul-2020
Date of Acceptance20-Dec-2020
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Vykuntaraju K Gowda
Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_122_20

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Introduction: Worster Drought syndrome (WDS) is a type of cerebral palsy that affects the muscles around the mouth and throat. This causes problems with swallowing, feeding, talking, and dribbling. Although the impairments are predominantly motor, a range of cognitive, behavioral, and seizures also exist. These features point towards the involvement of the perisylvian area of the cerebral cortex. Purpose of the Study: In WDS, the predominant impairment is a profound expressive communication problem and feeding difficulties; hence, it is under-recognized and even under-reported. Besides, these children have a specific phenotype which when recognized early can make a significant difference in management. This study aims to know the etiology and profile of WDS. Materials and Methods: This is a prospective cohort study in a tertiary care hospital from January 2018 to December 2019. All patients who met the absolute criterion for the diagnosis of WDS were included. A detailed history, clinical examination findings, relevant investigations like CT/MRI brain, and EEG were recorded in a predesigned proforma, and results were analyzed. Results: A total of 83 children (M:F = 52:31) presented within the age group of 2 years to 18 years. Seventy (84.33%) of them were delivered by a normal vaginal route. All children had global developmental delay, expressive speech delay, and spasticity. Other features were undernutrition 75(90%), drooling 66(79.51%), seizures 44(52.60%), and prolonged feeding 42(50.60%). Birth asphyxia was present in 72 (86.70%) cases, but neuroimaging was suggestive of perisylvian gliosis in 80 (96.38%) cases. Conclusions: WDS should be considered in children with cerebral palsy if mild motor delay with a predominant expressive language delay is present without hearing impairment and autistic features. In India, birth asphyxia is the most common cause.

Keywords: Cerebral palsy, congenital supra-bulbar paresis, drooling, epilepsy, expressive speech delay, pseudobulbar, Worster–Drought syndrome

How to cite this article:
Patil A, Gowda VK, Shivappa SK, Benakappa N. Profile of Worster Drought Syndrome (WDS): Unrecognized subtype of cerebral palsy—From tertiary care center in South India. J Pediatr Neurosci 2022;17:17-22

How to cite this URL:
Patil A, Gowda VK, Shivappa SK, Benakappa N. Profile of Worster Drought Syndrome (WDS): Unrecognized subtype of cerebral palsy—From tertiary care center in South India. J Pediatr Neurosci [serial online] 2022 [cited 2023 Dec 8];17:17-22. Available from: https://www.pediatricneurosciences.com/text.asp?2022/17/1/17/320379

   Introduction Top

Worster Drought Syndrome (WDS) was first described by Worster Drought in 1956 as congenital supra-bulbar paresis. He pointed the main lesion as congenital involvement of pyramidal tract causing weakness of lips, tongue, soft palate, and laryngopharynx. WDS is characterized by speech and feeding issues like prolonged meal times due to poor chewing and swallowing with a risk of aspiration, constant dribbling, poor lip closure, dysphagia, difficulty moving tongue, and exaggerated jaw jerk,[1] while sparing involuntary activities like smiling.[2] It is also thought now that it is the same as congenital bilateral perisylvian syndrome (CBPS). Kuzniecky et al. opined that CBPS is a homogenous clinical–radiological entity and that the underlying abnormality is probably polymicrogyria. CBPS is known by many other names. The manifestations of CBPS in pediatrics are different from adults, where it is called Foix–Chavany–Marie Syndrome (FCMS) or Anterior Opercular Syndrome (AOS) which results from bilateral infarction of the anterior operculum.[3] In FCMS, there is a loss of voluntary control of facial, pharyngeal, lingual, masticatory, and sometimes ocular muscles. Nevertheless, the reflexive and automatic functions of these muscles are preserved.[3] Acquired AOS, on the other hand, results from staged stroke with the patient becoming symptomatic after the second or third stroke. So, these three clinical conditions (CBPS, WDS, and AOS) have similar profiles of symptoms, but their etiology, onset, and clinical course are different so that a clinical diagnosis can easily be made.[2] Since there are complex and multiple impairments, we need a holistic approach for management, failing which child may not attain an optimal quality of life, hence early recognition is imperative, so we planned this study. This study aims to know the etiological, clinical, and radiological profile of WDS.

   Materials and Methods Top

We recruited all children, presented to a neurology clinic in a tertiary care pediatric center, in whom major feature was upper motor neuron type of bulbar palsy from January 2018 to December 2019. We followed the criteria suggested by Clark et al.[1] which is described below: Delay in gross motor function beyond the expected by cognitive level, mild pyramidal features, expressive language level significantly below that of receptive language, drooling inappropriate for developmental age, history of feeding problems, learning impairment, glue ear, recurrent respiratory infections /aspiration, gastroesophageal reflux, neuropsychiatric problems, epilepsy, contractors, and bilateral perisylvian pathology. We considered birth asphyxia as etiology in the presence of delayed cry or documented hypoxic–ischemic encephalopathy in the birth records, delay in milestones, static encephalopathy, pyramidal signs, and CT or MRI of the brain showing gliosis in the perisylvian region. We excluded other causes based on history, examination, and investigation like metabolic and genetic testing wherever indicated.

A detailed history was taken according to the pre-designed proforma which included a history of pseudobulbar paresis like prolonged time for feeding, difficulty in chewing, swallowing, drooling, speech status, and presence of seizures along with their semiology, the response to treatment, and the number of drugs used. Details of neonatal intensive care admission and developmental history were taken. Clinical examination findings of anthropometry, dysmorphic features, presence of poor lip closure, and restricted movements of the tongue were noted. The detailed neurological examination was done with special emphasis on jaw jerk and gag reflex. Head size, grading was done according to WHO charts, and less than 3Z scores were labeled as microcephaly. Grading of weight was done based on the Indian academy of pediatrics (IAP) classification. They were then investigated for CT/MRI and EEG. Ethical clearance was obtained from the institutional ethical committee. We had taken 83 children with cerebral palsy without a perisylvian syndrome as a control group and compared with cases of CP with the perisylvian syndrome.

   Results Top

A total of 83 children (M:F = 52:31) were presented within the age group of 2 years to 18 years. [Table 1] shows various risk factors in the study population. [Table 2] shows a comparison of various risk factors in cerebral palsy with and without the perisylvian syndrome. All children had global developmental delay and expressive speech delay. Seizures were noted in 44/83 (52%) cases. The various subtypes of seizures were 20/44 (45.4%) generalized tonic-clonic, 14/44 (31.8%) generalized tonic, and 10/44 (22.7%) focal type. The most common features of seizures are that they were precipitated while eating on 25/44 (57%). One case developed myoclonic jerks and epileptic encephalopathy. There was a family history of speech delay in two families, one in the mother and another in the father, but other features of WDS were not present. The indications for Cesarean section were obstructed labor in three (27.2%), oligohydramnios in four (36.3%), and in the rest four (36.3%), the indication was not known. In birth asphyxia, the average neonatal intensive care unit (ICU) stay was 7.1 days. On examination, spasticity was present in all, of which 19 (22.8%) had left more than right and 13 (15.66%) had right more than left. Three (3.61%) children were dysmorphic but did not fit into any specific syndrome. [Table 3] shows clinical findings and neuroimaging findings in the study population. The choice of the neuroimaging technique was based on the financial affordability status of the patient. In situations where the child had difficulty in getting sedated, where CT was done instead of MRI. The gliosis was bilateral in all cases except in two children only one-sided gliosis was noted. Compared to the bilateral lesions, the features of supra bulbar paresis were milder in unilateral cases. EEG was done in all 44 children with epilepsy and was abnormal in 39 (88.63%). [Table 4] shows the treatment outcomes in the study population. Thirty (68.18%) of them responded to a single drug, 13 (29.54%) of them needed two drugs, and only two (4.54%) needed three drugs. Four (4.81%) were born of seconded-degree consanguinity, nine (10.84%) were born of third-degree consanguinity, and only one child had an affected sibling with the perisylvian syndrome. [Figure 1]A shows clinical photograph of child with drooling and open mouth, and [Figure 1]B is the MRI of brain which shows gliosis in the bilateral perisylvian region.
Table 1: Risk factors of the study population

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Table 2: Comparison of cerebral palsy with and without the perisylvian syndrome

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Table 3: Clinical and neuroimaging findings in the study population

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Table 4: Treatment outcome in the study population

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Figure 1: (A) A clinical photograph of child with drooling and open mouth, (B) FLAIR and (C) T2WI sequence of MRI of the brain, respectively, showing gliosis in the bilateral perisylvian region (Informed consent was obtained from parents to publish the clinical photograph of a child.)

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   Discussion Top

The WDS is a poorly recognized form of cerebral palsy. It presents with predominant expressive speech delay, mild intellectual disability, drooling, and epilepsy. There were no studies from India on WDS. In this study, we analyzed 83 children with WDS. The mean age at diagnosis in our study was 7.3 years compared to 6 years in Clark et al.,[1] 7.9 years in Kuzniecky et al.,[4] and 2.25 years in Gropman et al.[5] The youngest child in our study was 2 years old, while Gropman has reported in a child as young as 1 week. The sex ratio in the current study was comparable to Clark et al.[1] group. In this study, the unilateral lesion is less severe, which is consistent with Kuzniecky et al.[4]

Clark et al.[1] reported that 60% of them had feeding difficulty, whereas we recorded 42 (50.6%) had feeding difficulties in the form of taking a long time to swallow. Twenty-six percent in the above-quoted study required tube feeding whereas none of the children in our study group required tube feeding. This is probably because, in India, tube feeding is rarely advised by a physician and also is not easily accepted by parents because of social stigma. Gastroesophageal reflux disease (GERD) was documented in 41%, but none of our children had GERD, probably because in India parents give less importance to these symptoms. However, poor nutrition was noted in 90% of our study population.

Expressive language delay was present in all children, similar to Clark et al.[1] study where 98% had a language delay. In this study, we found that the predominant complaints were expressive speech delay, so much that even in our oldest patient who was 18 years old, he could speak words that were intelligible only to familiar people. Secretory otitis media in Clark et al.[1] study occurred in 61% of children, but in our study, only two (2.4%) of children had ear infections probably because of lack of awareness and diagnosis of secretory otitis media in India Dribbling was reported in 61% of cases by Clark et al., while we report 66 (79.51%) of cases.

Clark et al. reported 73% of children with developmental delay, but all our cases had developmental delay. Brisk jaw jerk was seen in 90% of cases, while in our study, only 30 (36.1%) cases had this finding. Also, only 50% had increased tone, while all our cases had increased tone, although mild. This is probably because our cases are more severe and late presentation to the hospital. Only 7 (18%) of their patients were microcephalic, while 80 (96.3%) had microcephaly in our study. The reasons for microcephaly are, no specific norms for head size for Indian children, the inclusion of more severe cases, and more severe growth failure in these children. Thirty-one (66%) had congenital defects while none of our patients had any congenital anomalies. In Worster Drought et al.’s[6] original paper of 82 children, 11% had congenital defects and 27% had learning disabilities. In our study, only one child had a family history of similar complaints and none had congenital defects.

Only 13 (28%) children in Clark et al.[1] study had epilepsy, while it was 87% in Kuzniecky[4] study. In the current study, 44 (53.01%) have epilepsy. Of those children with epilepsy, none of them had atypical absence or atonic seizures as described in certain studies.[7] Also, most epilepsies were responsive to a single antiepileptic drug, with only one child having status epilepticus and another child developing epileptic encephalopathy. The CBPS study group reported intractable seizures in half of the group. Eighty-eight percent of our cases had abnormal EEG whereas only 50% of children in the Kuzniecky et al. study had abnormal EEG.

Kuzniecky et al.[4] reported the presence of bilateral perisylvian and perirolandic region abnormalities in all (31/31) cases, similar to our study. Kuzniecky et al. recruited patients based on clinical features alone or by the presence of clinical and imaging findings.

Birth asphyxia was present in 72 (86.7%) of cases, while none of the children had birth asphyxia in a study done by Clark et al.[1] and only 3/31 children in Kuzniecky[4] study had perinatal asphyxia. We compared various risk factors in cerebral palsy with and without perisylvian syndrome as shown in [Table 2]. Birth asphyxia, prolonged duration of labor, and feeding problems are more common in children with the perisylvian syndrome with CP compared to CP without the perisylvian syndrome; however, they are not statistically significant. It appears that birth asphyxia is contributing to the clinical features. In 11 cases, there was no history of birth asphyxia but an MRI of the brain was suggestive of gliosis, suggesting that there may be intrauterine hypoxia. We do not have the birth records in all cases, so it is difficult to categorically tell that birth asphyxia was absent. The informant was father and grandparents in some cases where the history was unreliable since if the grandparents were paternal or if it was father, they could be unaware of the events in the delivery room, which would have happened in the mother’s parents’ house, when either of the above may have not have been present. Also, there were not any consistent causes in the antenatal or intranatal period. Suresh et al.[2] in their study have pointed out that anoxia or ischemia of the developing brain could be a common plausible etiology. Also, the dissociation between voluntary and involuntary movements such as smiling or crying indicates that the disorder is of supranuclear type.[2] Our study differs in having a high incidence of birth asphyxia and the presence of gliosis more than polymicrogyria[8],[9],[10] and our children did not have any congenital anomalies.[11],[12] However, Takano et al.,[13] who described six cases including four infants, have expressed that the presence of perisylvian polymicrogyria does not have to be associated with oropharyngeoglossal dysfunction. But, as Clark et al.[1] have elucidated, these symptoms may appear as the child grows up, and since most were infants, the children have the possibility of developing these features later.

We have managed all children with a multidisciplinary team of various specialists and as shown in [Table 4]. Significant improvement was noted in most of the comorbidities in these children. Some intervention that should not have been done was tongue-tie release surgery by surgeons as most of the parents consult surgeon, with the common problem in these children being expressive speech delay with spastic tongue, they attribute tongue-tie as a reason for a speech problem. In this series, eight children underwent tongue-tie release surgery but no improvement was noted in any of them. This shows that, before advising tongue-tie surgery in any child with suspected expressive speech delay, the perisylvian syndrome should be ruled out. [Table 4] also shows that traditional speech and language intervention are not much helpful compared to augmentative and alternative communication (AAC) technique.

We noticed that visual impairment was also common findings but none of the other studies have reported this finding. A probable explanation for this is that the occipital area is also involved in our study group as perinatal asphyxia is the commonest cause compared to malformations in other studies. The limitation of our study is that we have not done formal intelligence quotient assessment in this study group, and the strength of our study is the largest cohort reporting from India.

   Conclusion Top

WDS should be considered in children with cerebral palsy if mild motor delay with predominant expressive language delay without hearing impairment and autistic features. In Indian subcontinent, birth asphyxia is the most common cause compared to malformation in developed countries. Early recognition is important for prognostication, identification of specific comorbidities, and multidisciplinary management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

Contribution of each author

AP was involved in the management of the child and the preparation of manuscript. VK supervision, guidance and reviewed the manuscript. SK was involved in the diagnosis and management of the child. NB has given valuable inputs in the management of this child.

   References Top

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Suresh PA, Deepa C Congenital suprabulbar palsy: A distinct clinical syndrome of heterogeneous aetiology. Dev Med Child Neurol 2004;46:617-25.  Back to cited text no. 2
Mariani C, Spinnler H, Sterzi R, Vallar G Bilateral perisylvian softenings: Bilateral anterior opercular syndrome (Foix–Chavany–Marie syndrome). J Neurol 1980;223:269-84.  Back to cited text no. 3
Kuzniecky R, Andermann F The congenital bilateral perisylvian syndrome: Imaging findings in a multicenter study. CBPS study group. AJNR Am J Neuroradiol 1994;15:139-44.  Back to cited text no. 4
Gropman AL, Barkovich AJ, Vezina LG, Conry JA, Dubovsky EC, Packer RJ Pediatric congenital bilateral perisylvian syndrome: Clinical and MRI features in 12 patients. Neuropediatrics 1997;28:198-203.  Back to cited text no. 5
Worster-Drought C Congenital suprabulbar paresis. J Laryngol Otol 1956;70:453-63.  Back to cited text no. 6
Kuzniecky R, Andermann F, Guerrini R Congenital bilateral perisylvian syndrome: Study of 31 patients. The CBPS multicenter collaborative study. Lancet 1993;341:608-12.  Back to cited text no. 7
Baykan-Kurt B, Sarp A, Gökyiğit A, Tunçay R, Calişkan A A clinically recognizable neuronal migration disorder: Congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up. Seizure 1997;6:487-93.  Back to cited text no. 8
Jansen A, Andermann E Genetics of the polymicrogyria syndromes. J Med Genet 2005;42:369-78.  Back to cited text no. 9
Margari L, Presicci A, Ventura P, Buttiglione M, Andreula C, Perniola T Congenital bilateral perisylvian syndrome with partial epilepsy. Case report with long term follow up. Brain Dev 2005;27:53-7.  Back to cited text no. 10
Yamanouchi H, Ota T, Imataka G, Hagiwara Y, Nakagawa E, Eguchi M Congenital bilateral perisylvian syndrome associated with congenital constriction band syndrome. J Child Neurol 2002;17:448-50.  Back to cited text no. 11
Poduri A, Chitsazzadeh V, D’Arrigo S, Fedrizzi E, Pantaleoni C, Riva D, et al. The syndrome of perisylvian polymicrogyria with congenital arthrogryposis. Brain Dev 2010;32:550-5.  Back to cited text no. 12
Takano T, Matsuwake K, Yoshioka S, Takeuchi Y Congenital polymicrogyria including the perisylvian region in early childhood. Congenit Anom (Kyoto) 2010;50:6467.  Back to cited text no. 13


  [Figure 1]

  [Table 1], [Table 2], [Table 3], [Table 4]


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