Neurology Notes

Neurology

Cerebral Palsy

• Abnormality of movement or posture attributed to non progressive disturbances that occurred in the developing fetal or infant brain

• Presents in early life (<2 years)

• Accompanied by disturbances of cognition, communication, perception, sensation, behaviour and seziure disorder and secondary MSK problems

• Affects 2 in 1000 live births

• Althought the underlying cause is static, the resulting motor disorder may evolve, giving the impression of deterioration

Causes

• 80% are antenatal in origin

• 10% due to hypoxic-ischaemic injury during delivery

• 10% are postnatal after the age of 2 it is more appropriate to use acuired brain injury

  • Meningitis/encephalitis/encephalopathy

  • Trauma

  • Symptomatic hypoglycaemia

  • Hydrocephalus

  • Hyperbilirubinaemia

Clinical Presentation (see pg 55 of Illustrated)

• Early features

  • Abnormal limb/trunk posture and tone

    • Delayed motor milestones

  • Feeding difficult

    • Oromotor incoordination, slow feeding, gagging and vomiting

  • Abnormal gait

  • Asymmetric hand function before 12 months

• Functional ability described using the Gross Motor Function Classification System

  • I; walks without limtations

  • II; walks with limitations

  • III; uses handheld mobility device

  • IV; self mobility with limitations; powered mobility

  • V; manual wheelchair

• Diagnosis made by clinical examination 3 clinical subtypes

  • Spastic (90%)

  • Dyskinetic

  • Ataxic

Spastic

• UMN damage (pyramidal or corticospinal tract)

• Clinically

  • Increased limb tone

    • Velocity dependent stretch muscle faster = greater resistance

    • May suddenly yield under pressure catch phenomenon

  • Brisk deep tendon reflexes

    • Extensor plantars

• 3 main types

  • Hemiplegia

    • 4-12 months of age

    • Fisting of affected hand

    • Flexed arm and pronated forearm

    • Hip flexion and internal rotation

    • Toe-heel walk on affected side

  • Quadriplegua

    • Opisothonus (extensor posturing)

    • Severe hence associated features

  • Diplegia

    • All 4 limbs but legs are worse

    • Associated with preterm birth and periventricular damage

Dyskinetic

• Damage in the basal ganglia

• Previous common cause was hyperbilirubinaemia (kernicerus)

• Involuntary, uncontrolled, stereotyped movements

• Most evident with active movement or stress

• May be described as

  • Chorea irregular, sudden, brief, non repetative

  • Athetosis slow writhing

  • Dystonia simultaneous agonist and antagonist contraction

• Present with

  • Floppiness

  • Poor trunk control

  • Delayed devlopment

Ataxic (hypotonic)

• Trunk and limb hypotonia

• Poor balance

• Delayed development

• Incoordinate movements, intention tremor and ataxic gait

Seizures

• Sudden disturbance of neurological function caused by adnormal or excessive discharge

• May be EPILEPTICAL

  • Idiopathic (70-80%)

  • Secondary

    • Cerebral dysgenesis/malformation

    • Cerebral vascular occlusion

    • Cerebral damage

  • Tumour

  • Neurodegenerative disorders

  • Neurocutaneous syndromes

• NON EPILEPTIC

  • Febrile

  • Syncopal (anoxic)

  • Brainstem (hydrocephalic, coning)

  • Emotional (psychogenic, pseudo-)

  • Metabolic

    • Hypoglycaemia. Etc.

  • Head trauma

  • Meningitis / encephalitis

  • Poisons / toxins

Febrile Seizures

• Seizure accompanied by fever in the ABSENCE of intracranial infection due to bacterial meningitis or viral encephalitis

• Often associated with URTI

• 3% of children aged between 6 months and 6 years

  • Unusual after >4 years

• Family history often present (?genetic)

• Seizures are typically brief

• Generalised tonic-clonic seizures

• 30% recur

• Does not cause brain damage

• Investigations lumbar puncture – low threshold

• If there is history of a prolonged seizure (>10 minutes) rescue therapy with diazepam PR of midazolam (buccal)

Paroxysmal Disorders

• “Funny turns”

Breath Holding Attacks

• In toddlers when upset

• Child may lose consciousness but rapidly recover

Reflex Anoxic Seizure

• Infants and toddlers

• Family history

• Triggers

  • Pain (esp. from minor head trauma)

  • Cold food

  • Fright

  • Fever

• After the event the child becomes pale and falls to the floor due to cardiac asystole from vagal inhibition

• Hypoxia may induce a generalised tonic clonic seizure

• Brief and child recovers

• NB; ocular compression under controlled conditions may lead to asystole and paroxysmal slow wave discharge on ECG

Others

• Benign Paroxysmal Vertigo

• Due to viral labyrinthitis

• Associated with nystagmus, unsteadiness and loss of balnce

• Cardiac Arrhythmia

  • Prolonged QT interval may rarely cause collapse or cardiac syncope

• Syncope

• Migraine

• Self-gratification

• Sleep disorders sleep myoclonus, parasomnias

• Daydreaming

• Pseudoseizures

Epilepsy

• Largely classified as generalised or focal seizures

Generalised

• ALWAYS a loss of consciousness

• No warning

• Symmetricaly

• Absence

  • Transient loss of consciousness

  • Abrupt onset and termination

  • No motor phenomena

• Myoclonic

  • Brief, repetitive, jerking movement of the limbs, neck or trunk

  • Physiological stage II sleep sleep myoclonus

• Tonic

  • Generalised increase in tone

• Tonic-clonic

  • Rhythmical contraction following the tonic phase

  • Rigid tonic phase children do not breath may become cyanosed

  • Clonic phase breathing is irregular, cyanosis persists and saliva may accumulate, biting of toungue and incontinence

  • Lasts seconds to minutes

  • Followed by unconsciousness or deep sleep for up to several hours

• Atonic

  • Loss of muscle tone

Focal

• May be preceded by an aura

• May or may not be associated with a change in consciousness or more generalised tonic-clonic seizure

• Frontal motor

• Temporal auditory / sensory (smell or taste). Automatisms (lip smacking. Etc.) occur after spread to the frontal lobe. Deja-vu associated

• Occipital visual

• Parietal contralateral altered sensation (dysaesthsia)

Diagnosis

• Diagnosis primarily based upon history

• Clinical examination should involve checking for skin markings suggesting neurocutaneous syndrome or neurological abnormalities

• EEG

• Imaging

  • Structural not required unless there are neurological signs between seizures or seizures are focal

    • MRI FLAIR (fluid attenuated inversion recovery) sequences better detct mesial temporal sclerosis

  • Functional

    • ...

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