Epidemiological and clinical studies of West syndrome in Nagasaki Prefecture, Japan

Matsuo, Atsuko; Matsuzaka, Tetsuo; Tsuru, Akira; Moriuchi, Hiroyuki; Nakashita, Y; Tanaka, Shigeki; Baba, Chikako; K, Tomimasu

doi:10.1016/s0387-7604(01)00267-4

Cited by 23 publications

(22 citation statements)

References 22 publications

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“…The present study comprised of a relatively large number of patients compared with some previous studies; 47 children by Matsuo et al, 2001; Sharma and Vishwanthan, 2008), 26 children by Sharma and Vishwanthan, 2008; Goldstein and Slomski, 2008), 28 children by Goldstein and Slomski, 2008; Cohen-Sadan et al, 2009). Criteria for definition of West syndrome were similar to previous studies and hypsarrhythmia on EEG is not considered essential for the diagnosis (Lux and Osborne, 2004).…”

Section: Discussionmentioning

confidence: 99%

“…A 16 channel EEG and neuroimaging (magnetic resonance imaging of brain – 1.5 Tesla machine or cranial CT scan – as afforded by the family) was obtained. Tests for developmental quotient (developmental Assessment of Indian Infants – DASII; Malin, 1971) and social quotient (Malin’s adaptation of Vineland Social Maturity Scale; Matsuo et al, 2001) were administered by a trained child psychologist initially and at the end of 6 months.…”

Section: Methodsmentioning

confidence: 99%

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West Syndrome: Response to Valproate

et al. 2012

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Management of West syndrome is unsatisfactory. In our clinic we observed that a significant proportion of patients respond to usual dose of valproate. Objective: To prospectively assess the efficacy of valproate in controlling infantile spasms in West syndrome. Methods: Consecutive patients presenting with West syndrome to the Pediatric Neurology Clinic or general outpatient department (OPD) were enrolled for study. Those who were not on any treatment were given valproate in a dose of 30 mg/kg/day while awaiting investigations. Patients were followed up every 2 weeks. Predefined criteria for definition of West syndrome and response were used. Those showing partial/poor response or relapse on valproate were given hormonal therapy. Results: One hundred children with West syndrome were enrolled. Ninety one children were started on valproate. Of these 36 (39.5%) showed a good response, but seven later relapsed while on same dose of valproate and three were lost to follow up. Later age at onset and typical hypsarrhythmia on EEG were associated with good sustained response to valproate while a history of delayed cry at birth was associated with partial or poor response. Sixty two patients who responded poorly to or relapsed on valproate were put on hormonal treatment in addition. Of these 36 (58.1%) had a good response but 11 later relapsed after stopping treatment and two were lost to follow up. Conclusion: Valproate may have a role in treatment of West syndrome in a selected group of patients.

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

West Syndrome: Response to Valproate

et al. 2012

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“…1 Its incidence varies between 2.9 and 4.5per 10,000 live births. [2][3][4][5] The prevalence is approximately 1/4000 -1/6000 [6], and in 70% of cases, patients are male. 2,3 West syndrome is caused by a brain dysfunction whose origins can be prenatal, neonatal and postnatal.…”

Section: Introductionmentioning

confidence: 99%

Combined Sensory Motor Approach to Enhance Participation of a Child with West Syndrome: A Follow-Up Case Study

Kunde

Ganvirs

2017

IPMRJ

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This is a follow up case report of male child with West Syndrome, brought to Physiotherapy department at the age of 1 year with a chief complaint of unable to transit to sitting, standing and walking. He had poor ability to focus and orient to environment and poor somatosensory, kinesthetic, and proprioceptive awareness throughout his body. Parents also complained that he becomes aggressive and irritated on moving surfaces. Treatment strategies were targeted towards specific impairments of the sensory, neuromuscular, and musculoskeletal systems to improve his gross motor abilities. At the early age of 1 year the score of GMFM was 3.98% and GMFCS level was V. At the age of 5 years there was increase in score of GMFM with 84.07% and GMFCS changed to level II indicating level of independence is also improved. This suggest that participation of a child with West Syndrome can be improved with combine sensory motor approach.

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“…West in 1841. It is a rare epileptic syndrome, 1 quite specific to early infancy 2,3 and considered a malignant condition 4 . The annual incidence of WS in developed countries is estimated at 1 in every 2,000 to 4,000 livebirths; 5,6 it occurs in boys 60% of the time 2 .…”

Section: Introductionmentioning

confidence: 99%

“…WS is generally classified into two groups: cryptogenic, when the spasms appear after an uneventful pregnancy with normal developmental milestones and with normal results of clinical and neurological examinations, 7,8 or symptomatic, when the origin of the infantile spasms is known 1 . The etiologies of symptomatic WS have been categorized as prenatal (toxemia during pregnancy congenital cerebral malformation, organic acidemia, intrauterine infection), perinatal (persistent neonatal hypoglycemia, hypoxic‐ischemic encephalopathy obstetric trauma), or postnatal (meningitis, herpes simplex encephalopathy phenylketonuria, cerebral hemorrhage) 3 …”

Section: Introductionmentioning

confidence: 99%

Oral findings and dental treatment in a child with West syndrome

Regis

Rocha

Torres

et al. 2009

Special Care in Dentistry

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West syndrome (WS) is a rare, severe form of epilepsy that typically manifests early in infancy. It is considered a malignant condition that combines episodes of spasms that occur in clusters (infantile spasm), hypsarrhythmia on the electroencephalogram, and neuropsychomotor delay. Although WS has been widely investigated from a medical standpoint, few reports have focused on the oral findings in patients with this syndrome. This article reports the case history of a 7-year-old child diagnosed with WS. The major clinical features were generalized tooth wear and gingival enlargement, altered chronology and sequence of dental eruption, primary canine cusp-to-cusp relationship, ectopic dental eruption, and mildly arched palate. Multiple white spot lesions were also observed, possibly associated with poor oral hygiene, due to a fermentable carbohydrate-rich diet, and continuous use of sugar-containing medications. Dental care management of patients with special needs is discussed and the dental treatment for this child with WS is described.

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Epidemiological and clinical studies of West syndrome in Nagasaki Prefecture, Japan

Cited by 23 publications

References 22 publications

West Syndrome: Response to Valproate

West Syndrome: Response to Valproate

Combined Sensory Motor Approach to Enhance Participation of a Child with West Syndrome: A Follow-Up Case Study

Oral findings and dental treatment in a child with West syndrome

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