Neuro

Seizure Disorders

Definitions

  • Seizure: A sudden discharge of excessive electrical energy from nerve cells in the brain.

  • Epilepsy: A condition characterized by two or more unprovoked seizures, potentially resulting from various pathological processes in the brain.

Classification of Seizures

  1. Idiopathic: No apparent cause; believed to have a genetic origin.

  2. Symptomatic: Associated with identifiable pathologic abnormalities in the brain.

  3. Provoked: Triggered by systemic factors such as medications or environmental stimuli (e.g., flashing lights).

  4. Cryptogenic: No clear cause but suspected underlying issues.

Types of Seizures

  • Partial Seizures (Focal Seizures): Involve one area of the brain without spreading throughout.

  • Generalized Seizures: Begin in one area and rapidly involve both hemispheres of the brain.

Clinical Presentation of Seizures

Generalized Seizures

  • Tonic Phase: Abrupt onset with an aura; the child loses consciousness, muscles stiffen, and may fall to the ground.

  • Clonic Activity: Followed by muscle spasms; tongue biting and involuntary urination are common. This phase lasts approximately 20 to 40 seconds.

Absence Seizures

  • Short transient losses of consciousness; may present with eye rolling or head drooping. Can be triggered by hyperventilation or bright lights.

Myoclonic Seizures

  • Involve sporadic, brief muscle contractions and may last several minutes. They are relatively rare and often secondary.

Status Epilepticus

  • Defined by frequent seizures lasting too long or occurring in succession, posing a medical emergency due to risks of brain damage from decreased oxygen supply.

Complex Partial Seizures

  • Involve complex sensory changes, emotional alterations, and a series of reactions, often with a loss of consciousness and may include motor changes such as urination or chewed motions. These usually begin in late adolescence.

Causes of Seizures

  • Primary Seizures: Abnormal neurons that are sensitive to stimulation.

  • Secondary Seizures: Related to factors like head injury, drug overdose, or environmental toxins.

Treatments for Seizure Disorders

General Management

  • Child must be eased onto the floor during a seizure, with tight clothing loosened to prevent harm. Maintaining airway by positioning the head to the side is crucial.

  • Patients may actively participate in non-hazardous activities but require supervision.

Use of Anti-Convulsant Drugs

  • Medicines are tailored based on seizure type and severity, and alterations should be made only after 1-2 weeks due to delayed maximal effects.

  • Continuous medication is required for 4-5 years post-last seizure; abrupt discontinuation is problematic, especially during puberty.

Specific Medications

  • For Grand Mal:

    • Phenobarbital: First-line due to its safety.

    • Dilantin (Phenytoin): Associated with gum hypertrophy; oral hygiene is critical.

  • For Petit Mal (Absence):

    • Zarontin: Lower blood dyscrasia risks.

    • Trimethadione (Tridione): Reduces cortical excitability but has potential significant side effects.

Meningitis

Overview

  • Inflammation of the meninges can be caused by various pathogens, most commonly bacteria such as H. influenza, pneumococcus, and others.

Symptoms

  • Signs include fever, headache, nuchal rigidity, and positive Kernig's and Brudzinski's signs. Children may exhibit irritability, vomiting, and increased intracranial pressure.

  • Anterior fontanel bulging is an important sign in children under 18 months.

Diagnosis

  • Diagnosis is based on clinical signs and lumbar puncture, which may show cloudy cerebrospinal fluid with elevated protein levels and reduced glucose.

Management

  • Maintain a quiet environment, monitor vital signs, and place on a non-constipating diet to prevent increased ICP. Isolation precautions are essential to prevent infection spread.

Hydrocephalus

Introduction

  • Characterized by an excess of cerebrospinal fluid within the ventricles and subarachnoid spaces.

Symptoms

  • Visible signs include increased head size, bulging fontanelles, and prominent scalp veins; may present alongside irritability or a high-pitched cry.

Diagnosis and Treatment

  • Diagnosed through imaging such as sonograms or MRIs. Treatment often includes managing ICP and possibly placing a ventriculoperitoneal shunt to drain excess fluid.

Neural Tube Defects (NTDs)

Overview

  • NTDs are congenital defects resulting from incomplete closure of the neural tube during fetal development.

Types

  • Spina Bifida Occulta: Failure of the posterior vertebrae to fuse; may present as a dimple or tuft of hair.

  • Meningocele: Herniation of the meninges through the vertebrae; presents as a protruding mass.

  • Myelomeningocele: Involves both the spinal cord and meninges, leading to severe complications.

Management

  • Early closure of defects is critical; ongoing monitoring for complications like hydrocephalus, urinary, and bowel management issues.

Down Syndrome

Definition and Genetics

  • A congenital disorder caused by an extra 21st chromosome (trisomy 21), leading to various physical and cognitive challenges.

Prevalence

  • Occurs in approximately 1 in 691 live births, with frequency increased in older maternal age.

Physical Characteristics

  • Individuals typically show facial features such as a flat forehead, slanted eyes, and hypotonia.

Cognitive and Developmental Impact

  • Ranges from mild to severe intellectual disability. Supportive care is necessary to address developmental and feeding challenges.

Associated Health Issues

  • Individuals may also experience congenital heart defects and gastrointestinal issues, necessitating holistic management throughout development.