NEUROLOGICAL DISORDERS

HEDACHE

Frequent reason for older children and adolescents to consult a doctor

(International Headache Society)

Primary

•Migraine

•Tension-type headache

•Cluster headache

Secondary

•Head or neck trauma,

•Cranial or cervical vascular disorder

•Vascular malformation or intracranial hemorrhage

•Non-vascular intracranial disorder

•Raised intracranial pressure, idiopathic intracranial hypertension-alcohol, solvent, drug abuse-infection

•Meningitis, encephalitis-hypercapnia, hypertension-acute sinusitis-psychiatric disorder

TENSION-TYPE HEADACHE

• Symmetrical headache of gradual onset

• Described as tightness, a band or pressure

• Usually no other symptoms

MIGRAINE WITHOUT AURA

• 90% of migraine children episodes may last 1-72h

• Bilateral or unilateral

• Pulsatile – temporal or frontal area

• Accompanied – nausea, vomiting, abdominal pain, photophobia

MIGRAINE WITH AURA

• 10% of migraine Headache is preceded by an aura (visual, sensory, motor)

• Last for a few hours, during which time children prefer to lie down in a quiet, dark place

TRIGGERS

• Stress

• Food

• Menstruation

• Emotional Or Behavioral Problems

• Alcohol, Drugs

HEADACHES OFTEN RAISE THE FEAR OF BRAIN TUMOURS

RED FLAG SYMPTOMS

Headache:

• Worse lying down or with coughing and straining headache

• Wakes up child associated confusion, morning or persistent nausea or vomiting recent change in personality, behavior or educational performance.

RED FLAG PHYSICAL SIGNS (SPACE-OCCUPYING LESION)

• Growth failure visual field defects:

Craniopharyngioma squint cranial nerve

• Abnormality torticollis

• Abnormal coordination

For cerebellar signs

• Papilledema of the second cranial nerve

• Bradycardia cranial bruits

• Arteriovenous malformation

RESCUE TREATMENTS

• Paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs)

• Anti-emetics serotonin agonists: Sumatriptanbeta-blockers

Propranolol

Psychological Support relaxation

SEIZURES

Is a clinical event in which there is a sudden disturbance of neurological function caused by an abnormal or excessive neuronal discharge.

May by epileptic or non-epileptic.

NON-EPILEPTIC• Febrile seizures

• Metabolic

Hypoglycemia

Hypocalcaemia

Hypomagnesaemia

Hypo/Hypernatremia

• Head Trauma

• Meningitis / Encephalitis

• Poisons / toxins

EPILEPSY

Idiopathic (70-80%) cause unknown but presumed genetic

Cerebral dysgenessia/malformation

Cerebral Vasular Occlusion

Cerebral damage:

Congenital infection,

Hypoxic-ischemic encephalopathy,

Intraventricular hemorrhage

Cerebral tumor

Neurodegenerative disorders

Neuro cutaneous syndromes

FEBRILE SEIZURES

• Affect 3% of children

• Have a genetic predisposition

• Occur between 6 months and 6 years of age

• Are usually brief, generalized tonic-clonic seizures occurring with a rapid rise in fever

• If a bacterial infection, especially meningitis, is present, it needs to be identified and treated

• Advise family about management of seizures, consider rescue therapy

Rectal diazepam

Oral prophylactic anti-epileptic drugs are not used

• An EEG is not indicated

• If simple – does not affect intellectual performance or risk of developing epilepsy

• If complex, 4-12% risk of subsequent epilepsy

CHILDREN WITH PAROXYSAML DISORDERS

• Breath-holding attacks – temper reflex anoxic seizures

• Head trauma

• Cold food

• Fright

• Fever syncope migraine

• Benign proximal vertigo

• Other causes (cardiac arrhythmia, ticks, pseudo seizures)

EPILEPSIES OF CHILDHOOD

Is a chronic neurological disorder characterised by recurrent unprovoked seizures, consisting of transient signs and/or symptoms associated with abnormal, excessive or synchronous neuronal activity in the brain.

GENERALISED SEIZURES

• There is always a loss o consciousness

• No warning

• Symmetrical seizures

• Bilaterally synchronous seizures

• Discharge on EEG or varying asymmetry

ABSENCE SEIZURES

MYOCLONIC SEIZURES

TONIC SEIZURES

TONIC-CLONIC SEIZURES

ATONIC SEIZURES

FOCAL SEIZURES

Onset in neural network limited to one cerebral hemisphere.

Begin in relative small group of dysfunctional neurons in one of the cerebral hemispheres

May be heralded by an aura which reflects the site of origin

May or may not be associated with change in consciousness or more generalized tonic-clonic seizure

FOCAL SEIZURES

• Frontal seizures

• Motor phenomena temporal lobe seizures

• Auditory or sensory (smell or taste) phenomena occipital

• Positive or negative visual phenomena parietal lobe seizures

• Contralateral altered sensation (dysaesthesia)

INVESTIGATION OF SEIZURES

Many children with epilepsy have a normal initial EEG

And many children who will never have epilepsy have EEG abnormalities

EEG is just a supportive evidence

ANTI-EPILEPTIC DRUG THERAPY(AED)

It is common practice not to institute treatment after a single unprovoked seizure

Not all seizures require AED therapy

The decision should be based on the seizure type, frequency and social and educational consequences of seizures set against the possibility of unwanted effects of the drug

All AEDs have potential unwanted effects

COMMON AEDs• Valproate

• Carbamazepine/Oxcarbazepine

• Vigabatrin

• Lamotrigine

• Ethosuximide

• Topiramate

• Gabapentin

• Levetriacetam

• Clonazepam,

• Diazepam

SPINAL MUSCULAR ATROPHY TYPE 1 WERDNIG-HOFFMANN DISEASE

ACUTE POST-INFECTIOUS POLYNEUROPATHY

GUILLAIN-BARRE SYNDROME

MYASTHEMIA GRAVIS

DUCHENNE MUSCULAR DYSTROPHY

FRIEDRICH ATAXIA

ATAXIA TELAGIECTASIA

This disorder of DNA repair

ARGene ATM has been identified

Mild delay in motor development in infancy

Oculomotor problems

Difficulty with balance and coordination becoming evident at school age

Deterioration with a mixture of dystonia and cerebellar signs

Many children require a wheelchair

Telangiectasia develops in the conjunctiva, neck and shoulders

Increased susceptibility to infection

Malignant disorders – ALL

CEREBELLAR ATAXIA

Causes – medication, drugs, varicella infection, posterior fossa lesion or tumors, genetic and degenerative disorders e.g friedrich ataxia and ataxia telangiectasia

EXTRADURAL HAEMORRHAGE

Head trauma

Skull fracture

Arterial or venous bleeding into the extradural space

Lucid interval until the conscious level deteriorates, with seizures

Focal neurological signs

Dilatation of the ipsilateral pupil

Paresis of the contralateral limps

Arrest of the bleeding

SUBDURAL HAEMATOMA

This is results from tearing of the veins as they cross the subdural space

Subdural hematoma and retinal hemorrhages in an infant – consider non-accidental injury caused by shaking or direct trauma

SUBARACHNOID HAEMORRHAGE

Much more common in adults

Acute onset of headpain

Neck stiffness

Occasionally fever

CT scan – blood in CSF

The cause is often an aneurysm or AVMMR angiography, CT or convetional angiography

NEURAL TUBE DEFECTS

ANENCEPHALY

ANENCEPHALY

NEURAL TUBE DEFECTS

SPINA BIFIDA OCCULTA

HYDROCEPHALUS SETTING-SUN SIGN

VENTRICULOPERITONELA SHUNT FOR DRAINAGE

NEUROCUTANEUS SYNDROMES

NEUROFIBROMATOSIS

TUBEROUS SCLEROSISS

TRUGE-WEBER SYNDROME

NF1This affects 1 in 3000 live births

AD or new mutation

6 or more cafe-au-lait spots > 5mm in size before puberty, >15mm after puberty

More than one neurofibroma

Axillary freckles

Optic Glioma

Lisch nodule, a hamartoma of the iris (slit-lamp examination)

Bony lesions from sphenoid dysplasia

A first degree relative with NF1

NF

TUBEROUS SCLEROSIS

The cutaneous features consist of:

•Depigmented ash leaf

•Shaped patches

•Shagreen patches

•angiofibromatic neurological features

•Infantile spasm and developmental delay

•Epilepsy

TUBEROUS SCLEROSIS

STURGE-WEBER SYNDROME

Sporadic disorder with a haemangiomatous facial lesion (a port-wine stain) in the distribution of the trigeminal nerve associated with a similar lesion intracranially.

CAUSES OF FLOPPY INFANT

• CorticalHypoxic-ischemic encephalopathy

• Genetic Down syndromePrader-willi syndrome

• Metabolic Hypothyroidism Hypocalcaemia

• Neuromuscular Spinal muscular atrophy Myopathy MyotoniaCongenital myasthenia

NEURODEGENERATIVE DISORDERS

• Tay-Sachs disease

• Gaucher disease

• Niemann pick disease

• Mucopolysaccharidosis

MPS1 = Hurler

MPS2 = Hunter

MPS3 = Sanfilippo

MPS4 = Morquio

MPS4 = Maroteaux-Lamy

TAY-SACHS DISEASE

Enzyme defect – Hexosaminidase are disorder

Most common among Ashkenazis Jews

Develop mental

Regression in late infancy

Severe hypotonia, enlarging head

Cherry red spot at the macula

Death by 2-5 years

Diagnosis measurement of the specific enzyme activity

Prenatal detection is possible in high-risk couples

TAY-SACHS DISEASE

GAUCHER DISEASE• Enzyme defect:

Beta – Glucosidase occurs in 1 in 500 Ashkenazi's jaws • Chronic childhood form:

Splenomegaly, bone marrow suppression, bone involvement, normal enzyme replacement therapy is available

• Acute infantile form:Splenomegaly, neurological degeneration with seizures

GAUCHER DISEASE

CLINICAL FEATURES OF MUCOPOLYSACCHARIDOSES

CLINICAL FEATURES OF MUCOPOLYSACCHARIDOSES

• Corneal clouding, retinal degeneration, glaucoma

• Thickened skin

• Valvular lesion, cardiac failure

• Developmental regression

• Thickened skull, broad ribs, thoracic kyphosis, lumbar lordosis

• Hepato-splenomegaly

• Conductive deafness

• Umbilical and inguinal hernias

NIEMANN-PICK DISEASEEnzyme defect:

Sphingomyelinase at 3-4 months, feeding difficulties, hepatosplenomegaly, developmental delay, hypotonic and deterioration of hearing and vision cherry red spot in macula affect 50%

NIEMANN-PICK DISEASE

WILSON DISEASEFrom the accumulation of copper, may cause changes in behavior and additional involuntary movements or a mixture of neurological and

hepatic symptoms.