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NEUROLOGICAL DISORDERS

NEUROLOGICAL DISORDERS. Anna Kosmowska. HEDACHE.

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NEUROLOGICAL DISORDERS

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  1. NEUROLOGICAL DISORDERS Anna Kosmowska

  2. HEDACHE • Frequent reason for older children and adolescents to consult a doctorIHS:PRIMARY H.: MIGRAINE, TENSION-TYPE HEADACHE, CLUSTER HEADACHESECONDARY H.: -HEAD OR NECK TRAUMA, -CRANIAL OR CERVICAL VASCULAR DISORDER – VASCULAR MALFORMATION OR INTRACRANIAL HAEMORRHAGE-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 onsetDescribed as tightness, a band or pressureUsually no other symptoms

  4. MIGRAINE WITHOUT AURA • 90% of migreneIn children episodes may last 1-72hBilateral or unilateralPulsatile – temporal or frontal areaAccompanied – nusea, vomiting, abdominal pain, photophobia

  5. MIGRAINE WITH AURA • 10% of migraineHeadache 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 • STRESSFOODMENSTRUATIONEMOTIONAL OR BEHAVIOURAL PROBLEMS ALCOHOL, DRUGS

  7. HEADACHES OFTEN RAISE THE FEAR OF BRAIN TUMOURS

  8. RED FLAG SYMPTOMS • HEADACHE – WORSE LYING DOWN OR WITH COUGHING AND STRAININGHEADACHE – WAKES UP CHILDASSOCIATED CONFUSION, MORNING OR PERSISTANT NUSEA OR VOMITINGRECENT CHANGE IN PERSONALITY, BEHAVIOUR OR EDUCATIONAL PERFORMONACE

  9. RED FLAG PHYSICAL SIGNS – SPACE-OCCUPYING LESION • GROWTH FAILUREVISUAL FIELD DEFECTS – CRANIOPHARYNGIOMASQUINTCRANIAL NERVE ABNORMALITYTORTICOLLISABNORMAL COORDINATION – FOR CEREBELLAR SIGNSPAPILLOEDEMA OF THE SECOND CRANIAL NERVEBRADYCARDIACRANIAL BRUITS – ARTERIOVENOUS MALFORMATION

  10. RESCUE TREATMENTS • PARACETAMOL AND NON-STEROIDAL ANTI-INFLAMANTORY DRUGS (NSAIDs)ANTI-EMETICSSEROTONIN AGONISTS e.g SUMATRIPTANBETA-BLOCKERS – PROPRANOLOLPSYCHOLOGICAL SUPPORTRELAXATION

  11. SEIZURES • Is a clinical event in which there is a sudden disturbance of neurological fuction caused by an abnormal or excessive neuronal discharge.May by epileptic or non-epileptic.

  12. NON-EPILEPTIC • FEBRILE SEIZURESMETABOLIC – HYPOGLYCAEMIA, HYPOCALCAEMIA, HYPOMAGNESAEMIA, HYPO/HYPERNATRAEMIAHEAD TRAUMAMENINGITIS / ENCEPHALITISPOISONS / TOXINS

  13. EPILEPSY • IDIOPATHIC (70-80%) - CAUSE UNKNOWN BUT PRESUMED GENETICSECONDARY:-CEREBRAL DYSGENESSIA/MALFORMATION -CEREBRAL VASULAR OCCLUSION -CEREBRAL DAMAGE e.g congenital infection, hypoxic-ischaemic encephalopathy, intraventricular haemorrhageCEREBRAL TUMOURNEURODEGENERATIVE DISORDERSNEUROCUTANEOUS SYNDROMES

  14. FEBRILE SEIZURES • Affect 3% of childrenHave a genetic predispositionOccure between 6 months and 6 years of ageAre usually brief, generalised tonic-clonic seizures occurring with a rapid rise in feverIf a bacterial infection, especially meningitis, is present, it needs to be identified and treatedAdvise family about management of seizures, consider rescue therapy – rectal diazepamOral prophylactic anti-epileptic drugs are not usedAn EEG is not indicatedIf simple – does not affect intellectual performance or risk of developing epilepsyIf complex, 4-12% risk of subsequent epilepsy

  15. CHILDREN WITH PAROXYSAML DISORDERS FUNNY TURNS • BRETAH-HODING ATTACS – TEMPERREFLEX ANOXIC SEIZURES – HEAD TRAUMA, COLD FOOD, FRIGHT, FEVERSYNCOPEMIGRAINEBENIGN PROXYSMAL VERTIGOOTHER CAUSES (cardiac arrythmia, ticks, pseudoseizures)

  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 consciousnessNo warningSymmetrical seizuresBilaterally 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 hemisphereBegin in relative small group of dysfunctional neurones in one of the cerebral hemispheresMay be heralded by an aura which reflects the site of originMay or may not be associated with change in consciousness or more generalised tonic-clonic seizure

  24. FOCAL SEIZURES • FRONTAL SEIZURES – MOTOR PHENOMENATEMPORAL LOBE SEIZURES – AUDITORY OR SENSORY (SMELL OR TASTE) PHENOMENAOCCIPITAL – POSITIVE OR NEGATIVE VISUAL PHENOMENAPARIETAL LOBE SEIZURES – CONTRALATERAL ALTERED SENSATION (DYSAESTHESIA)

  25. INVESTIGATION OF SEIZURES • EEG but …Many children with epilepsy have a normal initial EEGAnd many children who will never have epilepsy have EEG abnormalitiesEEG as add suportive evidenceStandard/ a sleep/ sleep-deprived record/ 24h-EEG/ videoEEGMRICTMetabolic investigationsGentic studies

  26. ANTI-EPILEPTIC DRUG THERAPY(AED) • …to treat or not to terat...It is common practise not to institute treatment after a single unprovoked seizureNot all seizures require AED therapyThe 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 drugAll AEDs have potential unwanted effects

  27. COMMON AEDs • ValproateCarbamazepine/oxcarbazepineVigabatrinLamotrigineEthosuximideTopiramateGabapentinLevetriacetamClonazepam, diazepam

  28. SPINAL MUSCULAR ATROPHY TYPE 1WERDNIG-HOFFMANN DISEASE

  29. ACUTE POST-INFECTIOUS POLYNEUROPATHYGUILLAIN-BARRE SYNDROME

  30. MYASTHEMIA GRAVIS

  31. DUCHENNE MUSCULAR DYSTROPHY

  32. FRIEDRICH ATAXIA

  33. ATAXIA TELAGIECTASIA • This disorder of DNA repairARGene ATM has been identifiedMild delay in motor development in infancyOculomotor problemsDifficulty with balance and coordination becoming evident at school ageDeterioration with a mixture of dystonia and cerebellar signsMany children require a wheelchairTelangiectasia develops in the conjunctiva, neck and shouldersIncreased susceptibility to infectionMalignant 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 TRAUMASKULL FRACTUREARTERIAL OR VENOUS BLEEDING INTO THE EXTRADURAL SPACELUCID INTERVAL UNTIL THE CONSCIOUS LEVEL DETERIORATES, WITH SEIZURESFOCAL NEUROLOGICAL SIGNSDILATATION OF THE IPSILATERAL PUPILPARESIS OF THE CONTRALATERAL LIMPSCTEVACUATION OF THE HAEMATOMAARREST OF THE BLEEDING

  36. SUBDURAL HAEMATOMA • THIS IS RESULTS FROM TEARING OF THE VEINS AS THEY CROSS THE SUBDURAL SPACESUBDURAL HAEMATOMA AND RETINAL HAEMORRHAGES IN AN INFANT – CONSIDER NON-ACCIDENTAL INJURY CAUSED BY SHAKING OR DIRECT TRAUMA

  37. SUBARACHNOID HAEMORRHAGE • Much more common in adultsAcute onset of head painNeck stiffnessOccasionally feverCT scan – blood in CSFThe 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. HYDROCEPHALUSSETTING-SUN SIGN

  43. VENTRICULOPERITONELA SHUNT FOR DRAINAGE

  44. NEUROCUTANEUS SYNDROMES • NEUROFIBROMATOSISTUBEROUS SCLEROSISSTRUGE-WEBER SYNDROME

  45. NF1 • This affects 1 in 3000 live birthsAD or new mutation6 or more cafe-au-lait spots > 5mm in size before puberty, >15mm after pubertyMore than one neurofibromaAxillary frecklesOptic gliomaOne Lisch nodule, a hamartoma of the iris (slit-lamp examination)Bony lesions from sphenoid dysplasiaA first degree relative with NF1

  46. NF

  47. TUBEROUS SCLEROSIS • THE CUTANEOUS FEATURES CONSIST OF:-DEPIGMENTED ASH LEAF-SHAPED PATCHES-SHAGREEN PATCHES-ANGIOFIBROMATANEUROLGICAL 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 ENCEPHALOPATHYGENETIC – DOWN SYNDROME, PRADER-WILLI SYNDROMEMETABOLIC – HYPOTHYROIDISM, HYPOCALCAEMIANEUROMUSCULAR – SPINAL MUSCULAR ATROPHY, MYOPATHY, MYOTONIA, CONGENITAL MYASTHENIA

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