1. NEUROLOGICAL DISORDERS

2. 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

3. TENSION-TYPE HEADACHE
Symmetrical headache of gradual onset
Described as tightness, a band or pressure
Usually no other symptoms

4. 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

5. 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

6. TRIGGERS Stress Food Menstruation Emotional Or Behavioral Problems
Alcohol, Drugs

7. HEADACHES OFTEN RAISE THE FEAR OF BRAIN TUMOURS

8. 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.

9. 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

10. RESCUE TREATMENTS
Paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs)
Anti-emetics serotonin agonists:
Sumatriptanbeta-blockers
Propranolol
Psychological Support relaxation

11. 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.

12. NON-EPILEPTIC Febrile seizures Metabolic Hypoglycemia Hypocalcaemia
Hypomagnesaemia
Hypo/Hypernatremia
Head Trauma
Meningitis / Encephalitis
Poisons / toxins

13. 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

14. 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

15. 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)

16. 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.

17. GENERALISED SEIZURES There is always a loss o consciousness No warning
Symmetrical seizures
Bilaterally synchronous seizures
Discharge on EEG or varying asymmetry

18. ABSENCE SEIZURES

19. MYOCLONIC SEIZURES

20. TONIC SEIZURES

21. TONIC-CLONIC SEIZURES

22. ATONIC SEIZURES

23. 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

24. 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)

25. 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

26. 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

27. COMMON AEDs Valproate Carbamazepine/Oxcarbazepine Vigabatrin
Lamotrigine
Ethosuximide
Topiramate
Gabapentin
Levetriacetam
Clonazepam,
Diazepam

28. SPINAL MUSCULAR ATROPHY TYPE 1 WERDNIG-HOFFMANN DISEASE

29. ACUTE POST-INFECTIOUS POLYNEUROPATHY GUILLAIN-BARRE SYNDROME

30. MYASTHEMIA GRAVIS

31. DUCHENNE MUSCULAR DYSTROPHY

32. FRIEDRICH ATAXIA

33. 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

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

35. 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

36. 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

37. 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

38. NEURAL TUBE DEFECTS
ANENCEPHALY

39. ANENCEPHALY

40. NEURAL TUBE DEFECTS

41. SPINA BIFIDA OCCULTA

42. HYDROCEPHALUS SETTING-SUN SIGN

43. VENTRICULOPERITONELA SHUNT FOR DRAINAGE

44. NEUROCUTANEUS SYNDROMES
NEUROFIBROMATOSIS
TUBEROUS SCLEROSISS
TRUGE-WEBER SYNDROME

45. 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

46. NF

47. TUBEROUS SCLEROSIS The cutaneous features consist of:
Depigmented ash leaf
Shaped patches
Shagreen patches
angiofibromatic neurological features
Infantile spasm and developmental delay
Epilepsy

48. TUBEROUS SCLEROSIS

49. 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.

50. 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

51. NEURODEGENERATIVE DISORDERS
Tay-Sachs disease
Gaucher disease
Niemann pick disease
Mucopolysaccharidosis
MPS1 = Hurler
MPS2 = Hunter
MPS3 = Sanfilippo
MPS4 = Morquio
MPS4 = Maroteaux-Lamy

52. 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

53. TAY-SACHS DISEASE

54. 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

55. GAUCHER DISEASE

56. CLINICAL FEATURES OF MUCOPOLYSACCHARIDOSES

57. 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

58. 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%

59. NIEMANN-PICK DISEASE

60. WILSON DISEASE
From the accumulation of copper, may cause changes in behavior and additional involuntary movements or a mixture of neurological and hepatic symptoms.