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Basics

Juliann M. Paolicchi, MA, MD


BASICS

DESCRIPTION

EPIDEMIOLOGY

Incidence

Prevalence

RISK FACTORS

Genetics

ETIOLOGY

COMMONLY ASSOCIATED CONDITIONS

Diagnosis

DIAGNOSIS

HISTORY

PHYSICAL EXAM

DIAGNOSTIC TESTS AND INTERPRETATION

Lab

Initial Lab Tests

Imaging

Initial Approach

Follow-Up & Special Considerations

Diagnostic Procedures/Other

DIFFERENTIAL DIAGNOSIS

Treatment

TREATMENT

MEDICATION

First Line

Second Line

ADDITIONAL TREATMENT

General Measures

The goal of treatment is cessation of spasms and resolution of the EEG. Serial treatment trials are recommended if spasms persist, since failure of one treatment choice does not preclude success with another.

Issues for Referral

Management of IS should be referred to a pediatric neurologist. Additional consultative services may include ophthalmology and genetics.

Additional Therapies

Given association of IS and developmental delay, referral should be made for early intervention services.

COMPLEMENTARY AND ALTERNATIVE THERAPIES

SURGERY/OTHER PROCEDURES

Surgery may be indicated in malformations of cortical development and treatment refractory IS, especially due to TS and focal cortical dysgenesis. Surgical resection is focused on areas of hypometabolism identified on PET, identifiable cortical dysgenesis, or hypometabolic tubers in TS. Referral to a pediatric epilepsy center is recommended for presurgical evaluation.

IN-PATIENT CONSIDERATIONS

Initial Stabilization

If patient appears ill, focus on ABCs before treatment of spasms. ISs themselves rarely threaten vital functions.

Admission Criteria

IV Fluids

Nursing

While ACTH is initiated, patient is monitored for blood pressure, stool guiac and urine glucose.

Discharge Criteria

Discharge is determined by establishment of the diagnosis, initiation of the etiologic evaluation, patient education, and, in the case of ACTH, tolerance of initiated therapy.

Ongoing Care

ONGOING-CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

DIET

PATIENT EDUCATION

PROGNOSIS

COMPLICATIONS

Additional Reading

SEE-ALSO

Codes

CODES

ICD9

Clinical Pearls