West Syndrome: A Case Report

 

Sanjay S Shinde

Professor, Child Health Nursing Department, Sumandeep Nursing College, Sumandeep Vidyapeeth, Vadodara.

*Corresponding Author E-mail: mscshinde@gmail.com

 

ABSTRACT:

Infantile spasms (IS), formerly known as West syndrome, are a type of seizure associated with the epileptic disorder known as West syndrome. Infants and children are affected by West syndrome. Mental deficits are associated with infantile spasms. A particular kind of seizure that starts in the first year of life is known as infantile spasms. Loss of developmental milestones or developmental regression. However, an electroencephalogram is required to confirm the diagnosis of west syndrome (EEG). We describe the unusual West syndrome case.

 

KEYWORDS: West syndrome.

 

 


INTRODUCTION:

A disorder known as epileptic encephalopathy, or West syndrome, is one in which epileptic seizures may directly cause cognitive and behavioral problems. For a child to be diagnosed with West syndrome, they must exhibit all three of these characteristics. The onset of infantile spasms in West syndrome normally occurs between 4 and 8 months of age.1 The youngster may experience brief forward bending of the body and stiffening of the arms and legs during the one to two second long seizures. Some kids extend their arms and legs while arching their backs. The transitions between waking and sleeping, as well as right after eating, are when spasms most frequently happen. They may occur in clusters of many spasms at a time and affected children can have dozens of these spasm clusters in a day2.

 

 

Patient and observation:

A 3-years 8-month-old child who have experienced involuntary head movements, giddiness, and inability to speak since birth. Past history when a 2-year-old boy fell down while playing, he suffered a head injury in the occipital region. He was taken to a private clinic and had

a CT scan of his head, but nothing was found. Children's giddiness after awakening from sleep lasts 15-20 minutes and necessitates frequent visits to a specialty hospital.

 

 

Clinical findings:

The physical examination was marked by anthropometric measurements. All the body movements were present, understanding the speaking command, but unable to speak. A complete blood count test was performed, and everything was normal. The electroencephalogram (EEG) reveals every common multifocal epileptic form abnormality, as well as a moderate degree of nonspecific, diffuse electrophysiological dysfunction and hypsarrythmia pattern3.  

 

 

Therapeutic intervention:

The paediatric department determined that the child's medical care was adequate. Injection: ACTH 60 Iu/mL;  0.7 cc IM BD, Syrup-Valporate 15 mg twice a day;  syrup-Rantac 3.5cc per oral dose twice a day;  and syrup-Calcium 500mg/10ml three times a day. A paediatrician recommended speech therapy, physical therapy, and occupational therapy. Therefore, the diagnosis of West syndrome was made according to the clinical features. The EEG (electroencephalogram) shows a hypsarrhythmia pattern. After the treatment, a reduction in giddiness and head nodding was observed4.

 

Causes and Risk Factors:

This condition is most common in infants and young children. The condition most commonly begins between 3 and 8 months of age. The condition usually stops at 4 years old.

 

Follow-up and outcomes:

A child was admitted to the hospital for one month; the child reported clinical improvement, and the paediatrician advised her on follow-up and proper home care.

 

Consent:

Informed oral consent was taken from child parents.

 

DISCUSSION:

The epilepsy syndrome known as "West syndrome" is defined by the infantile spasms, age of onset, and EEG pattern. This illness is named after Dr. William West, who first identified it in his 4-month-old son in 1841 and originally documented it. A child will have West syndrome in one out of every 2,500 to 3,000. According to this, 350–400 children in the UK will be diagnosed with West syndrome per year. Infantile epileptic spasm syndrome has an unique subtype called West syndrome5.

 

Clinical spasms are characterized by brief, synchronized movements of the head, trunk, or limbs that may be flexor, extensor, or a combination of both, lasting about one second, with the head nod being the most subtly expressive.

 

West syndrome often affects children between the ages of 4 months and 2 years, however it can affect children much older than that. When untreated, west syndrome in children has a bad prognosis. Infantile spasm, or IS, has two different etiological subgroups: cryptogenic and symptomatic. Prenatal insults such as perinatal hypoxia, preterm, kernicterus, hypoxic ischemic encephalopathy, and cerebral haemorrhage are the most common causes of west syndrome in children6.

 

ACTH and oral steroids are used in the treatment of West syndrome. Vigabatrin is a second-line therapeutic option. Sodium valproate, levetiracetam, topiramate, clonazepam, and zonisamide are further medications that have been shown to be beneficial in treating west syndrome. For those who don't respond to first treatment, the modified Atkins diet and the ketogenic diet are helpful.All children were subjected to minimum of 1.5 Tesla magnetic resonance imaging of brain. Their findings were recorded in the sheet. Similarly, children were also subjected to electroencephalography (EEG). The EEG was recorded using standard 10-20 system with 21 electrodes. EEG would be repeated after clinical cessation of spasm to document resolution of Hypsarrhythmia7.

 

To rule out latent tuberculosis, Mantoux chest X-rays were administered to all children exhibiting symptoms of West syndrome. Children were given oral prednisolone or ACTH after being screened for latent TB. In the event that neither medication worked, vigabatrin was started. Those who did not respond to vigabatrin and steroids were given clonazepam and a valproate trial. Those kids who didn't succeed with either of the aforementioned options were given the modified Atkins diet. The success of the treatment was measured by the cessation of spasms, which was defined as the absence of spasms for a string of 28 days within 14 days of the start of the treatment.

 

Surgery management: Your infant may require surgery if a brain scan reveals problems there. An operation known as a corpus callosotomy, often known as split-brain surgery, may be recommended by your doctor. The band of nerve fibres that links the two parts of the brain is known as the corpus callosum. The surgeon will make a cut during this treatment to stop the flow of seizure signals from one side of the brain to the other. Although they won't be as severe, your baby may still experience seizures. Tuberous sclerosis complex, a prevalent cause of West syndrome, can also be treated with this operation. The brain of your infant develops malignant tumours as a result of this illness. Resection surgery is one sort of treatment that helps some persons with this problem; It involves the surgeon removing the seizures-causing brain tissue. Other therapy and assistance may be beneficial if your kid has developmental difficulties. Postural assistance and occupational therapy are examples of these7.

 

CONCLUSION:

Children suffering with West syndrome affect their milestone development and risk for epilepsy, and the role of health care staff is important in providing immediate treatment and care.

 

COMPETING INTERESTS:

The authors declare no competing interests.

 

AUTHOR’S CONTRIBUTIONS:

All authors involved in data collection, manuscript drafting and manuscript revision and have read and agreed to the final manuscript

 

REFERENCES:

1.     West WJ. On a peculiar form of infantile convulsions. Lancet. 1841; 35:724–725.

2.     Commission on Classification and Terminology of the International League against Epilepsy Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia. 1989; 30:389–399.

3.     Commission on Classification and Terminology of the International League against Epilepsy Workshop on infantile spasms. Epilepsia. 1992; 33:195.

4.     Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: consensus statement of the West Delphi group. Epilepsia. 2004; 45:1416–1428.

5.     Fisher RS, Cross JH, French JA, Higurashi N, Hirsch E, Jansen FE, Lagae L, Moshé SL, Peltola J, Roulet Perez E, Scheffer IE, Zuberi SM. Operational classification of seizure types by the international league against epilepsy.

6.      Scheffer IE, Berkovic S, Capovilla G, position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017; 58:512–521.

7.     Mackay MT, Weiss SK, Adams-Webber T, American Academy of Neurology and the Child Neurology Society. Neurology. 2004; 62:1668–1681.

 

 

 

 

Received on 20.10.2023        Modified on 30.10.2023

Accepted on 08.11.2023       ©A&V Publications All right reserved

A and V Pub J. of Nursing and Medical Res. 2023; 2(4):141-143.

DOI: 10.52711/jnmr.2023.35