Seizure Disorder/Cerebral Palsy

Dr Jimoh A.O

Consultant Paediatrician/Senior Lecturer

College of Medicine & Health Sciences,

Bingham University/Teaching Hospital, Jos. Plateau state.

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Learning Objectives

• Student should be able:
• To define and classify seizures
• To identify and distinguish the various types of seizures
• To describe the principles in management of seizure
• To define cerebral palsy and identify risk factors for CP
• To describe the clinical presentations of various types of CP
• To identify the associated deficits and describe the principles in management of CP

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Seizure Disorder

Introduction…what is a seizure ?

• Paroxysmal involuntary disturbance of brain function, characterised by abnormal discharges originating from cortical neurones, and manifestations may be motor, sensory, autonomic or psychic.

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• Convulsions: erratic involuntary muscle movements

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• Epilepsy – Recurrent seizures unrelated to fever or an acute cerebral insult (unprovoked).

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• It has frequency of 4 – 6/1000 children.

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• It is not a diagnosis but a symptom of an underlying CNS disorder that requires thorough investigation and management plan.

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Classification

• Seizures can be divided into 2:

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• Febrile Seizures

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• Afebrile Seizures ( Epilepsy)

Febrile Seizure

• Occurs in young children as a result of elevated body temperature in the absence of a pathology in the brain.

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• Affects about 2-3% of young children, between the ages of 6months and 5 years.

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• Common causes include: Malaria, URTI (Pharyngotonsilitis), Bronchopneumonia, Otitis media, UTI.

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• Febrile seizures are usually brief, generalised tonic, clonic and occur in conjunction with a rapid rise in body temperature to ≥39oC.

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• Strong positive family history suggests genetic predisposition.

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Febrile Seizure contd

• Simple FS:
• Commoner form seen
• Single attack in 24 hr period
• Seizures are generalised tonic clonic, followed by brief post ictal period
• Lasts for < 15 minutes

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• Complex FS
• Multiple attacks in 24 hr period
• Seizures are focal or have focal findings during post ictal period
• Duration > 15 minutes.

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Febrile Seizure contd

• Recurrence occurs in about 1/3 of cases after the first episode.
• Risk of recurrence increases with –

i) Young age (<9months) at first episode

ii) Onset of seizure at lower body temperature

iii) Positive family history

iv) Complex features

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• Risk of development of epilepsy in children with febrile seizure is about 1% as in the general population if no risk factors.

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• Risk of epilepsy increases with - Complex febrile seizure, family history of epilepsy, abnormal neurological examination, onset of 1^st seizure <12 months.

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Management of febrile seizure

Resuscitation

Airway- Clear & Maintain airways

Breathing- Ensure that patient is breathing or assist respiration if needed

Circulation- Assess circulatory function

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Abort the Seizure with an anticonvulsant.

Rectal Diazepam. Age <3yr give 5mg ; >3yr give 10mg

IV Diazepam – 0.1 – 0.3mg/kg/dose

IM Paraldehyde 1ml / year of life to max of 5ml. (0.1mg/kg for children less than 1 year)

Management of febrile seizure contd.

Take a detailed history and carry out a thorough physical examination to ascertain possible underlying causes of the convulsion.

Relevant investigations to rule out meningitis and identify the underlying cause of fever.

- LP for CSF analysis - Random Blood Sugar

- Blood film for malaria parasites

- Chest X ray

- Urine m/c/s

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Afebrile seizure (epilepsy)

• Epilepsy refers to recurrent afebrile seizures, i.e. more than 1

episode of afebrile seizure

• Epilepsy affects about 1% of the population worldwide but

higher prevalence in the developing countries of the world.

Aetiology

- Idiopathic

- Perinatal brain injury- Neonatal encephalopathy, Intracranial haemorrhage.

- CNS infections

- Congenital malformations of the CNS

- Trauma with head injury

- Neurocutaneous syndromes- Neurofibromatosis, Tuberous sclerosis.

- Genetic disorders e.g. Juvenille Myoclonic Epilepsy

- Brain tumours.

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Classification of Epilepsy

• Generalized seizures
• Partial seizure
• Unclassified seizures
• Specific Epileptic Syndromes – Rolandic epilepsy, Landau Kleffner syndrome, Lennox Gastaut syndrome, infantile spasms.

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Classification is important for-

i) Choice of appropriate treatment

ii ) Prognosis

Classification Of Epilepsy contd

• Generalized seizures

Non-motor - Absence seizures

- typical

- atypical

Motor - Myoclonic seizures

- Clonic

- Tonic

- Tonic – Clonic

- Atonic (drop attacks)

- Unclassifiable

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Classification Of Epilepsy contd

• Focal (Partial)

A. focal aware seizure (formerly simple partial)

- motor

- sensory

- autonomic

- psychic

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B. focal impaired awareness (formerly complex partial)

- Simple partial followed by impaired consciousness

- with impaired consciousness at onset

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C. focal to bilateral tonic-clonic (formerly partial seizures with secondary generalization)

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Classification Of Epilepsy contd

Generalised tonic clonic seizures,

• Most common form of generalised seizure, formerly Grand mal epilepsy
• Characterised by loss of consciousness, generalised body stiffening (tonic component), then jerky movements of the limbs (clonic component).
• Loss of bowel and bladder control is common.
• There may be associated cyanosis due to irregular respiration & hypoxia.
• Last few minutes & is typically followed by post ictal sleep

Absence seizures, formerly petit mal

• Involves a brief period of loss of or diminution in normal activity. The child appears to be daydreaming or switching off and is totally unaware of what’s happening.

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• Commonly manifests as starring and transient loss of responsiveness and sometimes manifests as repeated eye blinking.

• Attacks are brief, last for about 5-20 secs, occur several times in a day.

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• May interfere with the child’s academic performance.

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• Age of onset : 4-8 yrs. More common in girls.

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• Most outgrow the disease by teenage years.

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• Typically, child does not fall and there are no post ictal phenomena.

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Atonic seizure

• These are characterised by sudden loss of muscle tone which causes the child to fall to the ground.

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• May manifest as head nodding or sagging at the knees.

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• There is a high risk of injury, especially head injury from the repeated falls.
• A.k.a Drop attack

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• Treatment -Anti-epileptic drugs- Na Valproate

-Protective helmets & face guard.

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Myoclonic Jerks

• These are brief, forceful jerks which may affect the whole body or a part of it.

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• The jerks may be severe enough to cause a fall.

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• Usually brief accidents are common e.g. spilling drinks.

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Partial seizures

Simple Partial Seizure (Focal Aware Seizure)

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• Seizure affects only a part of the brain. There is no impairment of consciousness.

• May manifest as numbness, tingling sensation, numbness, altered taste in the mouth, disturbances of hearing or vision, sweating, dizziness.

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• Attacks not noticeable to on-lookers.

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Partial seizures contd

COMPLEX PARTIAL SEIZURE (focal impaired awareness)

• Characteristically manifests as sensory, motor or behavioural alterations.
• May be preceded by an aura, e.g. foul odour, bitter taste.
• Automatism is a feature- plucking at clothes, wandering around, dancing, running, lip smacking, chewing.
• Child is not aware of self during attacks.
• Transient somnolence may follow an attack.

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Specific Epileptic Syndromes

• Rolandic epilepsy

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• Landau Kleffner syndrome

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• Lennox Gastaut syndrome

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• Infantile spasms.

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Neonatal seizures

• Pattern

- Apnea with tonic stiffening of the body

- Focal clonic movements of one limb or both limbs on one side

- Multifocal clonic limb movements

- Myoclonic limb jerking

- Tonic deviation of the eyes upwards or to one side

- Tonic stiffening of the body

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Status epilepticus

• It is defined as a continuous convulsion lasting longer than 20–30 min or the occurrence of serial convulsions between which there is no return of consciousness.

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• It is a medical emergency that requires an organized and skillful approach to minimize the associated mortality and morbidity.

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• Produces potentially life-threatening disturbances in physiologic function, and the mortality rate .,is ≈5%

Causes of status epilepticus

• Prolonged febrile seizures (febrile siezure >30 minutes)
• Sudden withdrawal of anticonvulsant
• Metabolic abnormality
• Sleep deprivation
• Epileptic children not regular on anticonvulsant or who are noncompliant

Diagnosis of seizures

• Diagnosis of epilepsy is clinical

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• Seldom witness by doctor

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• Witness spells on video made by parent or caregiver is vital.

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Management

• If child is convulsing at time of presentation, first abort the seizure +ABC of resuscitation.

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• History
• Age
• Onset
• Duration
• Description of ictal event
• Reponse to tactile and verbal stimulation
• Post ictal phase
• Family history
• Response to therapy if any

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Investigations

• Blood glucose
• Lumbar puncture should be considered for children with repeated seizures and other evidence of neurodevelopmental disability

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• LP can help in detecting low CSF glucose in the glucose transporter disorder; alterations in amino acids, neurotransmitters, or cofactors in metabolic disorders; or evidence of chronic infection

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• Electrolytes including Ca²⁺ and PO₄²⁺

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• EEG- to determine the type of epilepsy and the future prognosis

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• Neuroimaging
• CT scan, MRI, Positron emission tomography (PET)
• Magnetoencephalography (MEG )

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ELECTROENCEPHALOGRAPGH

Treatment

• Pharmacotherapy – mainstay of management

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• Examples: Phenobarbitone, phenytoin, Ethosuximide,

Diazepam, Carbamazepine, Sodium Valproate ,

Clonazepam, Gabapentin, Topimarate etc

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• The goal is to ensure the best quality of life by maximizing seizure control and minimizing drug toxicity.

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• Treatment is started with one drug, at a small dose, then gradually increased to the lowest effective maintenance to minimize adverse effects

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• The child's serum anticonvulsant level should be monitored at intervals, and the dose should be altered accordingly.

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Treatment contd.

Surgery

• Temporal lobectomy
• Topectomy – removal of the cortex
• Lesionectomy – removal of identified lesions
• Hemispherectomy
• Corpus callostomy

Surgeries that involve implanting devices:

- Vagus nerve stimulation

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Treatment contd.

• Counselling

- explain condition thoroughly

- emphasize drug compliance

- dispel stigmatization and wrong believes

- interact with school – teachers/pupils

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• Monitor – drug level, complications etc

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Cerebral Palsy

What is Cerebral Palsy?

• A disorder of posture and movement, resulting from a permanent, non-progressive lesion to the immature or developing brain.

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• First described by William Little in the 19^th century. Also known as Little’s disease or static encephalopathy.

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• Incidence : 1.2-2.5 /1000 in the developed countries.

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What Are the Causes of Cerebral Palsy

PRENATAL PERINATAL POSTNATAL

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• Radiation

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• Anoxia

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• Infection (Intrauterine)

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• Developmental anomaly-encephalocoele,

Cerebral cysts

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• Drugs – alcohol, narcotics

• Birth weight

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• Asphyxia**

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• Trauma

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• Prematurity

• Metabolic – PKU, hypoglycaemia, hypothyroidism.

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• Infections** –meningitis, encephalitis

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• Trauma-head injury.

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• Toxins – Kernicterus**

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• Cerebral Hypoxia-FB aspiration,

near-drowning

What are the Risk Factors for CP?

• Multiple gestation
• Low birth weight or preterm
• Intrauterine death of a co-twin/triplet
• Infections during pregnancy
• Black race
• Rhesus incompatibility
• Exposure to toxic chemicals
• Maternal medical conditions like thyroid disorders, seizure disorders, mental disorders
• Difficult delivery
• Neonatal seizures

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What are the Clinical Features?

• Microcephaly
• Delayed developmental milestones (commonest)

Early pointers to CP include-

• Feeding difficulties
• Hypotonia ( floppy infant)
• Head lag
• Neonatal seizures

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Later in the 1^st year of life-

• Persistence of primitive reflexes e.g. grasp, Moro, sucking
• Abnormal posturing- spastic grasp, scissoring, tongue thrusting, persistent thumb adduction
• Hand-preference in the 1^st year of life
• Hypertonia

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Classification of CP

• Aetiologic - Prenatal, Perinatal, Postnatal

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• Physiologic - Spastic, Dyskinetic, Ataxic, Hypotonic, Atonic, Mixed
• Topographic - Monoplegia, Hemiplegia, Triplegia, Quadriplegia, Double Hemiplegia, Spastic Diplegia

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• Functional - I - No limitation of activity

II - Slight to moderate limitation

III - Moderate to severe limitation

IV- No useful activity

• Gross Motor Function Classification System (GMFCS) I-V

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Spastic Diplegia

• Usually in preterms due to high incidence of IVH & periventricular leukomalacia.
• About 80% of preterm infants with CP have spastic diplegia
• Child presents with delayed walking, scissoring gait, commando crawl.
• Scissoring posture of lower limbs, disuse atrophy of lower limbs
• Brisk reflexes, ankle clonus present
• Toe walking in older children. 50% learn to walk by 3yrs
• Minimal involvement of upper limbs.
• IQ usually normal. Seizures are rare.

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Spastic diplegia

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Spastic Quadriparesis

• Most severe form of CP.
• Seen in infants/ children with diffuse brain injury
• There is weakness of all 4 limbs, as well as the neck and trunk muscles. Lower limbs more involved & more severe than upper limbs
• Opisthotonic posturing is a feature in infancy
• Movement of the head often initiates forced extension of the arms and legs
• Swallowing difficulties and drooling. Predisposition to recurrent pneumonia
• Seizures occur in about 50% and severe mental retardation is common.

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Spastic Quadriparesis

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Dyskinetic CP

• Usually in infants with bilirubin encephalopathy.

• Characterized by athetosis & chorea, tongue thrusting , feeding difficulties.

• There may be associated dental enamel dysplasia, impairment of upward gaze and deafness.

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• Seizures & mental retardation uncommon.

Ataxic CP

• Results from damage to the cerebellum. Individual has problems with balance & depth perception

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• It sometimes co-exists with spastic diplegia

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• Child experiences difficulties with rapid movements and is often unsteady.

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• Motor difficulties are often not apparent until late in the first year of life

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• Writing skills are compromised & other skills that demand good fine motor coordination. This adversely affects educational endeavours

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Associated Deficits

• These other neurological problems may pose problems that are more severe than the impaired motor function.
• They include:
• Mental Retardation
• Epilepsy
• Hearing Impairment
• Visual Defects- strabismus, optic atrophy, nystagmus, refractive errors, cortical visual impairment
• Speech Defects- delayed, poor-articulation, loss of voice modulation
• Learning disability
• ADHD
• Failure to thrive, feeding problems
• Gait abnormalities
• Hip subluxation, other skeletal deformities, dental problems
• Bladder and bowel control problems

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DIAGNOSIS

Mainly clinical and can be made from history and thorough physical examination

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HISTORY: to determine risk factors, psychosocial factors and family adapatation

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EXAMINATION: to evaluate degree of deformity and other associated problems

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INVESTIGATIONS-May include:

• Skull X ray- intracranial calcification, skull defects.
• Chromosomal studies- to r/o Down syndrome & others
• CT scan of the brain - cerebral atrophy, dysgenesis etc
• MRI
• Developmental assessment tools to get developmental age

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Differential Diagnosis

• Differential Diagnosis
• Brain tumours
• Muscular dystrophy
• Down syndrome
• Spinal cord injury
• Peripheral Neuropathies
• Myelomeningocoele

MANAGEMENT

• General care: nutrition, personal care

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• Pharmacological: muscle relaxants, anticonvulsants

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• Surgical: rhizotomy

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• Physiotherapy/physical aid: walking aid, splints, braces, wheelchairs

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• Special education

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• Rehabilitation services

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• Family support services

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MANAGEMENT contd�

• Requires a multi-disciplinary approach, involving a team of specialists.�These include the following:
• Paediatric Neurologist
• Ophthalmologist
• Audiologist
• Speech Therapist
• Clinical Psychologist
• Physiotherapist
• Occupational Therapist
• Educator
• Social worker
• Orthopaedic Surgeon.
• NB: Involve parents in the management of the child

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Prognostic factors in CP

• Clinical type of CP

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• The degree of delay in meeting expected milestones noted at presentation

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• Pathologic reflexes present

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• Degree of associated deficits in intelligence, sensation & emotional adjustment

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Thank you

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Questions?