Neurological Disorders in Children

Cerebral Palsy

group of nonprogressive disorders of upper motor neuron impairment that result in motor dysfunction.

speech or ocular difficulties, seizures, cognitive challenges, or hyperactivity.

Children who have cerebral palsy may have

2 in every 1,000 live births.

Incidence rate of cerebral palsy

• Abnormal brain development or damage to the developing brain

• Nutritional deficiencies

• Drug use

• Maternal infections such as cytomegalovirus or toxoplasmosis

• Direct birth injury

Risk Factors of cerebral palsy

very-low birth weight infants, Head injury such as from child maltreatment or automobile accidents, infections such as meningitis or encephalitis

Cerebral palsy is commonly associated with

spastic, dyskinetic, ataxic, mixed

Types of Cerebral Palsy

Spastic (Pyramidal)

Hypertonia in involuntary muscles that results from loss of upper neurons., abnormal clonus, positive Babinski reflex. Persistent neonatal reflexes (e.g., tonic neck reflex). Poor parachute reflex. Scissors gait, toe walking (tight heel cords), hip subluxation

Scissor gait

Fail to demonstrate parachute reflex

Hemiplegia, Diplegia/Paraplegia, Quadriplegia

Subtypes of Spastic Cerebral Palsy

Hemiplegia

One side affected, affects the arms more than legs. Flexed elbow when supinating, shorter limb, smaller muscle mass, astereognosis

Quadriplegia

All limbs + speech/swallowing difficulty (pseudobulbar palsy)

Tonic neck reflex (fencer position)

when the arm and leg on the side toward which the head is turn extend, and the opposite arm and leg contract.

2nd and 3rd months of life.

Tonic neck reflex (fencer position) disappears between

Moro (startle) reflex

can be elicited with a loud noise. The newborn extend arms and legs then swings the arms into an embrace position and pulls up the legs against the abdomen.

strong for the first 8 weeks of life and then fades by the end of the fourth or fifth month.

Moro reflex disappears

Babinski Reflex

when the sole of a newborn's foot is stroked in an inverted "J" curve from the heel upward, a newborn fans the toes.

3 months of age.

Babinski Reflex remains positive until at least

dyskinetic or athetoid cerebral palsy

Abnormal involuntary movements

• Infant is limp and flaccid.

• Uncontrolled, slow, worm-like writhing or twisting movements

• Affects all four extremities and possible involvement of face, neck, and tongue

• Movements increase during periods of stress

• Dysarthria and drooling may be present

- may become irregular and jerking (choreoid) with disordered muscle tone (dyskinetic)

Ataxic Cerebral Palsy

involvement have an awkward, wide based gait. On neurologic examination, they are unable to perform fine coordinated motions, the finger-to nose test, or rapid, repetitive movements (cerebellar tests)

Mixed Cerebral Palsy

Combination of spastic, athetoid, or ataxic features. Typically results in severe physical impairment

strabismus, refractive disorders, visual perception problems, visual field defects and speech disorders such as abnormal rhythm or articulation.

All forms of CP may have sensory alterations such as

A skull Xray or ultrasound may show cerebral asymmetry. • An EEG may be abnormal, although the pattern is highly available

Diagnostic tests for Cerebral Palsy

• To prevent contractures, teach parents some passive exercises and games as part of their child's therapy and must be done consistently each day.

• Advise parents to always supervise children during bathing.

• On a high fiber diet •

Encourage parents to pursue stimulating activities and bring toys appropriate to the child's intellectual, developmental and motor levels, not the child's chronological age.

Interventions for Cerebral Palsy

Viking Speech Scale

a tool used to classify the speech performance of children with cerebral palsy (CP). It's designed to help categorize the severity of speech impairments in children with CP

VSS Level 1

Speech is not affected by motor disorder and follows usual speech development

VSS Level 2

Speech is imprecise but usually understandable to unfamiliar listeners. have speech that is affected by their motor disorder. Loudness of speech is adequate for one to one conversation. Voice may be breathy or harsh sounding but does not impair intelligibility.

VSS Level 3

Speech is unclear and not usually understandable without contextual cues. Difficulties controlling breathing for speech. Can be too loud or to quiet to be understood. Speech may be markedly hyper nasal.

VSS Level 4

No understandable speech.

Bacterial Meningitis

inflammation of the protective membranes covering the brain and spinal cord caused by various types of bacteria

Occur most frequently in children younger than 24 months of age

Bacterial Meningitis frequently occurs at what age

-Streptococcus pneumoniae

-Group B Streptococcus

-Eschericheria coli

-Pseudomonas

-Hemophilus influenzae

-Neisseria meningitidis

Causative Agents of Meningitis

blindness, hearing impairment or facial paralysis.

Invasion of the infection into cranial nerves can result in

syndrome of inappropriate antidiuretic hormone secretion (SIADH).

Brain tissue edema can put pressure on the pituitary gland causing increased production of antidiuretic hormone resulting in increased edema because the body cannot excrete adequate urine.

• Children usually have had 2 or 3 days upper respiratory infection

• Irritable (intense headache)

• Poor sucking, weak cry, or lethargy (newborn)

• Back arched and their neck hyperextended

• Will not follow a light through visual fields

• Papular or purple petechial skin rash may occur

• Positive Brudzinski and Kernig signs

• Sudden cardiovascular shock, seizures, nuchal rigidity, or apnea

Signs and Symptoms of Meningitis

-Antibiotic therapy may be prescribed prophylactically for the child's immediate family members

-Respiratory precautions for 24hrs after the start of antibiotic

-Administer Corticosteroids or diuretics to reduce ICP and prevent hearing loss

- Assess Neurologic sequelae in the weeks to come

Therapeutic Management of Meningitis

Corticosteroids such as dexamethasone or the osmotic diuretic mannitol. Antibiotics such as Cefotaxime, Ceftriaxone; Ampicillin (for H. influenzae)

Medications used in Meningitis

↑ WBCs and protein

↓ Glucose (bacteria consume glucose)

Normal CSF glucose ≈ 60% of serum

Lumbar Puncture results to diagnose meningitis

Paroxysmal Disorders

occurs suddenly and recurrently. Seizures, headaches, and breath-holding spells are the most frequent types seen in childhood

Seizure

an involuntary contraction of muscle caused by abnormal electrical brain discharges. 50% are idiopathic, they can also be attributed to infection, trauma, or tumor growth as well as Familial or polygenic inheritance.

epilepsy

comes from a Greek word meaning "to take hold of". preferred term today is "recurrent seizures"

Partial Seizures:

Localized to one area of the brain. May not involve loss of consciousness.

Generalized Seizures

Involves the entire brain. Loss of consciousness is common.

• Twitching of the head, arms or eyes

• Smacking of the lips

• Slight cyanosis

• Respiratory difficulty or apnea

• Limp & flaccid

Signs and Symptoms of Seizures in newborn

• Trauma & anorexia (intrauterine life or birth)

• Metabolic disorders (hypoglycemia,

hypocalcemia, or lack of pyridoxine)

• Neonatal infection

• Acute bilirubin encephalopathy (blood

incompatibility)

Cause of Seizures in newborn

Infantile Spasms

Rapid trunk movements, flexion, adduction of limbs, often seen in the first 6 months of life. This occur slightly more often in males than females (2-3 per 10,000 live births)

may be caused from trauma, metabolic disease such as phenylketonuria, viral invasion such as herpes or cytomegalovirus.

Cause infantile spasms

Infantile Spasms

Seizures from Poisoning or Drugs

Seizures in Infants and Toddlers:

Febrile Seizures

Complex Partial (Psychomotor) Seizures:

Partial (Focal) Seizures:

Absence Seizures:

Tonic-Clonic Seizures:

Seizures in Children Over 3 Years:

Febrile Seizures

Caused by High fever (102-104°F), often post-immunization. usually generalized tonic-clonic pattern which lasts for 15-20 seconds. and the seizure only lasts 1 to 2 minutes or less.

Antipyretic treatment (acetaminophen), monitoring, and if needed, diazepam for recurrent seizures. Tepid sponge bath (do not apply alcohol or cold water). Don't attempt to give oral medications after the seizure (drowsy) and might aspirate

Management for febrile seizures

• Laceration of the brain tissue from automobile

accident or fall

• Enlarging brain tumor

• Hemorrhage due to a blood dyscrasia

• Infection

• Anoxia

• Lead poisoning

Causes of Complex Partial (Psychomotor) Seizures:

• Sudden change in posture such as arm dropping

suddenly to the side

• Automatism (lip smacking, fumbling hand

movements, intense running or screaming)

• Slump to the ground unconscious

• Circumoral pallor

Signs and Symptoms of Complex Partial (Psychomotor) Seizures

Valproic acid, phenytoin, or phenobarbital. Carbamazepine (Tegretol) can lead to neutropenia (monitor WBC count) during therapy

Management of Complex Partial (Psychomotor) Seizures:

Partial (Focal) Seizure

A seizure affecting a limited portion of the brain. Motor or sensory signs originating from a specific brain area (e.g., tingling, muscle contractions).

Absence seizures (petit mal)

Occurs during childhood, Staring spells lasting 1-5 seconds, possibly accompanied by rhythmic blinking..occur more often in girls than boys (4-12 y.o)

Ethosuximide, sodium valproate, lamotrigine.

Treatment for Absence Seizures

tonic-clonic seizure (grand mal seizure)

Involves the entire body. In the tonic phase of the seizure, the body becomes rigid, and in the clonic phase, there is uncontrolled jerking.

Prodromal, aura, tonic, clonic, postictal.

Phases of tonic-clonic seizures

Prodromal Period

may consists of drowsiness, dizziness, malaise, lack of coordination, or tension.

Aura

may reflect portion of the brain in which the seizure originates.

Smelling unpleasant odors denotes activity in the medial portion of the temporal lobe.

Seeing flashes of lights suggests the occipital area;

repeated hallucinations arise from temporal lobe;

numbness of an extremity relates to the opposite parietal lobe,

a "Cheshire-cat grin" relates to the frontal lobe.

Reflected portions in the brain and its consequences during tonic-clonic seizures

Tonic Stage

During a seizure, the child's muscles contract, causing them to fall and stiffen with a distorted face. This phase lasts about 20 seconds, during which breathing muscles contract, leading to possible hypoxia and cyanosis. Saliva collects due to an inability to swallow, and the child may bite their tongue. A guttural cry occurs as air is forced through the glottis.

Postictal Period

sleep soundly for 1-4 hours and will rouse only to painful stimuli during this time. When awoken, experiences a severe headache and will have no memory of the seizure

Phenobarbital

has the advantage of being an inexpensive anticonvulsant. However, drowsiness and sleepiness may interfere with the child's ability to perform in school. dosages should be tapered, never stopped suddenly because the body becomes dependent on it.

Phenytoin sodium (Dilantin), valproic acid (Depakene), carbamazepine (Tegretol), Phenobarbital.

Anticonvulsants

painless hypertrophy of the gums.

One side effect of phenytoin

2-3 years.

Medications are usually continued until the child has been seizure free for

Ketogenic Diet

Some children are prescribed what diet because it is believed to decrease myoclonic or tonic-clonic seizure activity.

Status Epilepticus

Seizure that lasts continuously for longer than 30 minutes or series of seizures from which the child does not return to his or her previous level of consciousness. an emergency situation requiring immediate treatment. otherwise, exhaustion, respiratory failure, permanent brain injury or death may occur.

IV benzodiazepines (e.g., diazepam, lorazepam) halts seizures dramatically

Oxygen therapy to relieve cyanosis

Treatment for Status Epilepticus

Imaging tests such as CT scans, MRIs, skull radiographs, or EEGs may be indicated.

Diagnostic tests for seizures

Remain Calm

Move away any sharp object

Turn the child gently on their side

Don't restrain other than the keeping the head turned to the side

Do not attempt to place a stick or padded tongue blade between the child's teeth.

Try to keep onlookers from crowding the area

if the child should pass rapidly from one seizure into another (status epilepticus) she may need supplemental oxygen

Safety During Seizure

Guillain-Barre Syndrome

Inflammatory polyradiculoneuropathy. perplexing syndrome that involves both motor and sensory portions of peripheral nerves. Immune-mediated, often triggered by upper respiratory or GI infections or immunizations

Demyelination of peripheral nerves

• Peripheral neuritis several days after infection

• Decreased or absent tendon reflexes

• Muscle paralysis and paresthesia begin first in

the legs and then spread to involve the arms,

trunk, and head.

• Facial weakness and difficulty in swallowing

Assessment of Guillain-Barre Syndrome

Elevated CSF production level, EEG may show denervation and decreased nerve conduction velocity

Diagnostic tests for Guillain-Barre Syndrome

• Supportive until process runs it course; paralysis. Monitoring of cardiac and respiratory function

peaks at 3 weeks and is followed by gradual recovery

• Prednisone to halt autoimmune response

• Plasmapheresis or transfusion of immune serum globulin may shorten the course of the illness

• Indwelling urinary catheter is usually inserted to monitor urine output

• Enteral or total parenteral nutrition

• Analgesia

• Passive range of motion every 4 hours to prevent muscle contractures

• Turning and repositioning every 2 hours to protect skin integrity

Management of Guillain-Barre Syndrome

Headache

may occur as a result of conditions as simple as eye strain and sinusitis, as serious as brain tumor. The pain is because of meningeal vascular irritation not from the brain itself because brain tissue is insensitive to pain.

has both vomiting and a headache

Common signs of brain tumor in toddlers

Tension or Stress Headache

often occur when children are concentrating or studying, causing muscle contraction in the neck and temporary ischemia. experienced as a dull, steady pain in the head. Relieved by simple analgesics or by sleep or application of a cool compress

Sinus Headache:

Often associated with sinusitis, sinus headaches result from inflammation and possible obstruction of the sinuses. Symptoms include facial pain, nasal congestion, and pressure around the eyes or forehead.

Migraine Headache:

may be preceded by an aura (e.g., visual disturbances like diplopia or a zigzag pattern). Pain is usually unilateral, throbbing, and moderate to severe. Aggravated by routine physical activity and may be accompanied with nausea and vomiting and intolerance to bright lights and noise.

- Onset and frequency of headaches

- Any preceding events (e.g., aura)

- Duration, intensity, and associated symptoms (e.g., nausea, light sensitivity)

- Treatments taken and their effectiveness

- Conduct a complete physical exam, including funduscopic examination to rule out papilledema. Measure blood pressure to rule out hypertension.

- If an aura is present, an EEG may be ordered.

Assessment for Migraines

• Ergotamine tartrate (Cafergot) which constricts

cerebral arteries

• Propranolol and flunarizine

• Beta blockers or calcium channel blockers that

results in vasodilation may be prescribed

prophylactically.

• Sleep or lying down may be necessary to relieve

the pain and vomiting

Management of Migraines