Transcript tension-type headache

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
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Head or neck trauma,
•
Cranial or cervical vascular disorder
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Vascular malformation or intracranial hemorrhage
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Non-vascular intracranial disorder
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Raised intracranial pressure, idiopathic intracranial hypertension-alcohol, solvent, drug abuse-infection
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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
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Have a genetic predisposition
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Occur between 6 months and 6 years of age
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Are usually brief, generalized tonic-clonic seizures occurring with a rapid rise in fever
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If a bacterial infection, especially meningitis, is present, it needs to be identified and treated
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Advise family about management of seizures, consider rescue therapy
Rectal diazepam
Oral prophylactic anti-epileptic drugs are not used
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An EEG is not indicated
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If simple – does not affect intellectual performance or risk of developing epilepsy
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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
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Valproate
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Carbamazepine/Oxcarbazepine
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Vigabatrin
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Lamotrigine
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Ethosuximide
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Topiramate
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Gabapentin
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Levetriacetam
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Clonazepam,
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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
NF1
This 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
• Cortical
Hypoxic-ischemic encephalopathy
• Genetic
Down syndrome
Prader-willi syndrome
• Metabolic
Hypothyroidism
Hypocalcaemia
• Neuromuscular
Spinal muscular atrophy
Myopathy
Myotonia
Congenital 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 DISEASE
Enzyme 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 DISEASE
From the accumulation of copper, may cause changes in behavior and
additional involuntary movements or a mixture of neurological and
hepatic symptoms.