Neurological Emergencies &

Stabilization

By Dr. Akshay

Objectives

General pediatric neurological assessment

Nonsurgical neurological emergencies

Neurosurgical nontraumatic emerencies

Neurosurgical traumatic injuries

Initial stabilization and emergency management

Symptoms of presentation

Moves Spontaneously & purposefully

Withdraws In Response To Touch

Withdraws In Response To Pain

Decorticate Posturing In Response To Pain

Decerebrate Posturing In Response To Pain

None

Seizures

– Febrile seizures

– Afebrile Seizures (Childhood epilepsy syndromes)

CNS Infections

Approach to Febrile Seizures

Febrile seizures are defined as seizures that occur in association with fever, in the

absence of CNS infection (meningitis, encephalitis) and in patients with no history of

previous afebrile seizures

Occur in 3-4% of children between 6 months – 5 years (peak age 18-24months)

High recurrence (30-40%)

Etiology – genetic predisposition

– 40% concordance rate for monozygotic twins versus 7% for dizygotic twins

– 8% if sibling with febrile seizures, 22% if sibling + parent

– Mode of inheritance: polygenic vs autosomal dominant with variable penetrance

Risk of developing epilepsy in general population: ~1%

Simple (typical)

•Generalized

•<15min in duration

•No recurrence in a

24hr period

•Normal

neurological status

before seizure

Complex (atypical)

•Focal

•>15min in duration

(status epilepticus)

•Multiple episodes in

a 24hr period

•Abnormal

preexisting

neurological status

before seizure

Classification

Approach to Febrile Seizures

Treatment of febrile seizures and prevention of recurrences does not alter risk of later

possible epilepsy - routine use of AEDs not recommended.

Parents can be advised to use anti-pyretics for comfort care, but there is no evidence

that it prevents recurrence of febrile seizures

Intermittent prophylaxis can be used when recurrence is expected; excessive parental

anxiety

– Clobzam (0.5 to 1 mg/kg/day) (Minimum 3days or until fever subsides)

What investigations are necessary:

– Simple febrile seizures: nothing

– Complex febrile seizures: EEG ± neuroimaging

Approach to Febrile Seizures

What do parents want to know:

o Is this harmful

oWill it happen again

o Can I prevent it?

oWill my child develop epilepsy

oWill it go away?

Approach to Febrile Seizures

Intermittent prophalyxis

Clobzam (0.5 to 1 mg/kg/day)(Minimum 3days or until fever subsides)

Approach to Afebrile Seizures

Seizure

Provoked

•Electrolyte abnormalities

•Infection (meningitis)

•Trauma

•Toxic ingestion

•Vasculitis

•Inborn error of metabolism

•CNS tumour

Unprovoked

•History

•Exam

•Investigations: lytes (Glc, Ca, P, Mg);

CBC; LP; tox screen, etc)

•Neuroimaging

Does it fit any of the Childhood Epilepsy

Syndromes?

•Semiology of seizures

•Age of onset

•EEG features

•Clinical features/progression

•Response to Rx

•Prognosis

History

•Antenatal History

•Birth history

•Developmental history

•Family history

•Head trauma

•Seizure description (aura,

trigger, eyewitness

description)

Serologies/TORCH

Preeclampsia/GDM/Infections

Substance abuse/meds

Antenatal USG

Fetal distress

Need for postnatal resuscitation

Normal vs delayed vs regressed

Consanguinity, hx of febrile

seizures, epilepsy, developmental

delay, recurrent miscarriages, IEM

Approach to Afebrile Seizures

Physical Exam

•Dysmorphism

•Neurocutaneous Markers

•Neurological exam including

•HC

• developmental

•?Liver, heart involvement (IEM)

Approach to Afebrile Seizures

Physical Exam

•Dysmorphism

Physical Exam

•Neurocutaneous Markers

Hypopigmented macule

(Tuberous sclerosis)

Shagreen patch (Tuberous

Sclerosis)

Café -au -lait macule

(Neurofibromatosis)Port Wine Stain (Sturge Weber)

Feinichel

Neonatal Period (<28 days)

Seizures in newborns are often difficult to distinguish from normal activity

Most commonly occur within the first week of life

– 2/3 of neonatal seizures are due to Hypoxic-ischemic encephalopathy (HIE)

– Other causes: infection, electrolyte abnormalities, inborn errors of metabolism,

structural

The clinical and electroencephalographic features of neonatal seizures differ

considerably from those in older children and adults.

Approach to Neonatal Seizures

Hypoxic-Ischemic Encephalopathy

Infection

Electrolyte abnormalities

Structural

Inborn errors of metabolism

Stroke

Epilepsy syndromes

Benign Epileptic encephalopathies

•Sudden jerking movements

during sleep only

•Can be stopped with gentle

restraint

•Normal EEG

•No Rx

•Excessive response to

stimulation

•Low frequency, high

amplitude shaking of limbs

and jaw in response to touch,

noise or motion

•Low threshold for Moro reflex

•Almost never a seizure

manifestation unless associated

with eye deviation, tonic stiffening

•Prolonged apnea without

bradycardia & with tachycardia is

a seizure until proven otherwise

•Often associated with

HIE

MENINGITIS

Signs & Symptoms in Infants

• Fever or hypothermia

• Poor Feeding

• Irritability or lethargy

• Seizures

• Rash

• Tachypnea or apnea

• Jaundice

• Bulging fontanelle (late)

• Vomiting or diarrhea

• Altered Sleep Pattern

Other Clinical Features

Focal Neurologic Deficit ( 33 % )

Rash ( 26 % )

Petechiae

Palpable purpura

Coma ( 14 % )

Seizure ( 5 % )

Differential Diagnosis

• Encephalitis• Brain Inflammation

• HSV – 1 Most common cause

• Aseptic Meningitis• Viral: Enterovirus, HIV, Mumps, HSV - 2

• Fungal: Coccidioidomycosis, Cryptococcus

• Tuberculosis

• Parasites

• Neoplasm of the leptomeninges

• Drug - Induced: NSAIDs, Allopurinol etc.

• Intracranial Abscess

Treatment

Inpatient ( preferably in ICU )

Appropriate Antibiotic Therapy

Supportive Care(hemo-dynamic ,respiratory, renal &

electrolytes, myocardial support)

Treat coexisting conditions(seizures ,Raised ICT)

Prevent hypothermia and dehydration

Status Epilipticus

status epilepticus is defined as a generalized

convulsion lasting 30 minutes or longer or when

successive convulsions occur that the patient does

not recover consciousness between.

However, it is usual practice to start anti-

convulsive treatment when the episode has lasted

5 or more minutes

Stroke

Specific Causes of raised ICP

“Sunsetting Eyes”: clinical sign of

increased intracranial pressure

Traumatic Brain Injuries

Disorders of motor function or

weakness

Approach to Hypotonia

Central Nervous System

Spinal Cord

Anterior Horn cell

Peripheral Nerve

Neuromuscular junction

Muscle

Central Nervous System

•Benign congenital hypotonia

•Genetic syndromes (Prader-Willi,

trisomies)

•Hypoxic-ischemic encephalopathy

•Cerebral malformations

•Cortical migration abnormalities

(lissencephaly, schizencephaly)

•Inborn errors of metabolism

(leukodystrophies)

•History (traumatic birth HIE)

•Seizures

•Cognitive delay

Approach to Hypotonia

•Dysmorphism

•Hemiparesis

•Hyperreflexia

Spinal cord

•Trauma

•Spinal cord ischemia

Approach to Hypotonia

Anterior Horn Cell

•Spinal muscle atrophy

•Neonatal poliomyelitis

•Profound weakness

•Areflexia

•Feeding difficulties

•Respiratory difficulties

•Tongue fasciculations

•No sensory deficits

•Alert, interactive

Approach to Hypotonia

Peripheral Nerve

•Hereditary motor and sensory

neuropathies

•Acute inflammatory demyelinating

polyneuropathy

•Familial dysautonomia syndromes

•Giant axonal neuropathy

•Inborn errors of metabolism

•Distal > proximal weakness

•Hyporeflexia

•Facial weakness unusual

•Sensory deficits

Approach to Hypotonia

Neuromuscular Junction

•Infantile botulism

•Transient neonatal

myasthenia gravis

•Congenital myasthenia gravis

•Generalized weakness

•Fatiguability

•Hyporeflexia

•Feeding problems

•Respiratory compromise

•Ptosis

Approach to Hypotonia

Muscle

•Congenital myopathy

•Congenital muscular dystrophy

•Syndromic and non-

syndromic

•Congenital myotonic dystrophy

•Metabolic myopathy

•History of poor fetal movement/polyhydramnios

•Proximal muscle weakness

•Hyporeflexia

•Feeding problems

•Respiratory compromise

•Facial diplegia

•Arthrogryposis/bilateral club feet

•Other organ involvement (ex. Heart in Pompe disease)

Approach to Hypotonia

• Head/spine imaging

• Genetic testing

• Karyotype

• Prader-Willi (methylation 15q11-13)

• SMA testing

• CSF evaluation

• EMG/NCS

• Muscle/nerve biopsy

• Tensilon testing

• Metabolic w/u:

• Lactate/pyruvate

• Plasma a.acids

• Urine organic acids

• Acyl carnitine profile

• VLCFA