1PEDIATRIC NEUROLOGY NEUROLOGIC EVALUATION A. HISTORY Dr. J. Wangdali 2 = active mov’t, gravity eliminated 3 = active mov’t against gravity 4 = active mov’t against gravity & resistance 5 = normal power *Abnormalities of tone: 1. Spasticity = initial resistance to passive mov’t ff by a sudden release called the clasp-knife phenomenon, most apparent in the upper extremity flexors & LE extensor muscles = results from a lesion that involves upper motor neuron tracts, maybe unilat/bilat. 2. Rigidity = result of a basal ganglia lesion, charac by constant resistance to passive mov’t of both extensor & flexor muscles. *Children w/ spastic LE drag the legs while crawling(commando style) or walk on tiptoes. *Opisthotonus =marked spasticity & rigidity; head & heels are bent backward & body bowed forward *Decerebrate rigidity = marked extension of extremities resulting from dysfx or injury to brainstem 3. Hypotonia = abnormally diminished tone , most common abnormality of tone in neurologically compromised PT or FT Abnormalities in motility & locomotion: = most important component of the evaluation of a child with a neurologic problem = child’s dev’tal assessment is the most important component = family hx is extremely important . NEURO EXAM 1. Head – size & shape shld be documented -2 fontanels at birth: posterior fontanel is usually closed & nonpalpable after the 1st 6-8 wks of life - anterior fontanel may normally close as early as 9 months ; average time of closure is 18 mo. - fontanel is slightly depressed & pulsatile, best evaluated when infant is held upright while asleep or feeding ave. rate of head growth in PT: 0.5 cm in the 1st 2 wk, 0.75 cm during the 3rdwk & 1 cm in the 4th wk & thereafter until the 40th wk of dev’t HC of term infant: at birth – 34-35 cm 6 mos. - 44 cm 1 y/o - 47 cm 2. Cranial Nerves (review your cranial nerves) 3. Motor exam – includes an assessment of the integrity of the MSK system & search for abn mov’ts that may indicate a disorder of PNS or the CNS - Components include testing of strength, muscle bulk, tone, posture, locomotion & motility, DTRs & presence of primitive reflexes Muscle power grading: 0 = no contraction 1 = flicker or trace of contraction 1. Ataxia = incoordination of mov’t or disturbance of balance *Abnormalities assoc w/ cerebellar lesions: a. Dysmetria b. Rebound c. disdiadochokinesia 2. Chorea = involuntary mov’ts of the major joints, trunk & the face that are rapid & jerky. = hand grip contracts & relaxes (milkmaid sign), speech is explosive & inarticulate nerve & myoneural junction & abn of cerebellum Babinski reflex = indicates an UMN lesion. trunk & neck 5.cerebellar ataxia produces a broad based unsteady gait . writhing mov’t that is assoc often with abnormalities of muscle tone. Dystonia = involuntary. 4. Neuroradiologic procedures = skull x rays. Sensory exam 5. = ideal interspace is L3-L4 or L4-L5 = contraindications: a) elevated ICP 2’ suspected mass lesion of brain or SC b) s/sx of pending cerebral herniation c) critical illness d) skin infection at site of LP e) thrombocytopenia 2. suppressible & preceded by a warning sensation = eye blinking. SPINA BIFIDA OCCULTA = consists of a midline defect of vertebral bodies without protrusion of SC or meninges * Primitive reflexes : appear & disappear in sequence during specific periods of dev’t. sex & maturation of nervous system C. . Ventricular tap = used to remove CSF in increased ICP assoc with hydrocephalus 4. depending on age. most prominent in the distal extremities & is enhanced by voluntary activity or emotional upset. radionuclide brain scan 5. twisting mov’t that primarily involves the prox muscles of the extremities. their absence or persistence beyond a given time frame signifies dysfx of CNS 4. NEURAL TUBE DEFECTS (DYSRAPHISM) = account for most congenital anomalies of CNS. Subdural tap = to establish a dx of subdural effusion or hematoma = approached at lat border of ant fontanel or along the upper margin of coronal suture at least 2-3 cm from the midline 3.scoliosis may cause an abn gait & result from disorders of muscle & SC SOFT NEUROLOGIC SIGNS : = defined as a particular form of deviant performance on a motor or sensory test in the neuro exam that is abnormal for a particular age . result from failure of the neural tube to close spontaneously bet the 3rd & 4th wk of in utero dev’t = precise cause is unknown B. brief rapid mov’t of head & shoulders DTRs : graded from 0 (absent) to 4 (markedly hyperactive).2 PEDIATRIC NEUROLOGY 3. Motor tics = sudden brief unsustained mov’t. Wangdali = interpreted cautiously bec they are present in normal children during various stages of neurodevelopment = variation in expression of these signs . Athetosis = slow. CT scan. with 2 being normal DTRs are absent or decreased in primary dso of muscle. J. EEG = provides continuous recording of electrical activity with electrodes placed on the scalp CONGENITAL ANOMALIES OF THE CNS A. DIAGNOSTIC PROCEDURES: 1. Gait – a spastic gait is charac by stiffness & by stepping like a tin soldier . MRI. characterized by extension of great toe & fanning of remaining toes Dr. slow.a waddling gait results from weakness of the prox hip girdle . grimacing. Lumbar puncture = lat recumbent position. frontal or nasofrontal are more prominent = infants with cranial encephalocele are at increased risk for hydrocephalus = (+) small sac w/ pedunculated stalk or large cystlike structure that may exceed size of cranium = lesion may be completely covered w/skin = UTZ – most helpful in determining contents of the sac F. but learning problems & seizure dso more common = chronic handicapping condition E. ANENCEPHALY : = presents a distinctive appearance with a large defect of the calvarium. lack neuro signs = patches of hair. vessels & neuroglia = die w/in several days of birth = incidence is 1/1000 LB . meninges & scalp assoc with a rudimentary brain = results from failure of closure of the rostral neuropore. cerebellum or portions of brainstem = occurs most commonly in the occipital region but in certain parts of the world. resulting in protrusion of tissue through a bony midline defect. Wangdali = mortality rate – 10-15%. MYELOMENIGOCELE = most severe form of dysraphism involving the vertebral column. incidence of 1/4000 LB = unknown cause = genetic predisposition = maternal periconceptional use of folic acid reduces incidence by at least 50% = folic acid supplementation shld be initiated before conception & continued until 12th wk AOG = produces dysfx of many organs = lesion in low sacral region causes bowel & bladder incontinence with anesthesia in the perineal area. called cranium bifidum = cranial meningocele – consists of a CSF-filled meningeal sac only = cranial encephalocele – contains the sac plus cerebral cortex. lipoma. most deaths occur before 4 y/o = 70% survivors have normal intelligence. ENCEPHALOCELE = 2 major forms of disraphism affect the skull.3 PEDIATRIC NEUROLOGY = asymptomatic. opening of the anterior neural tube = primitive brain consists of portions of CT. J. no impairment of motor function = lesion in the midlumbar region produces lower motor neuron signs = increasing neuro deficit as the lesion extends higher into the thoracic region *Hydrocephalus in assoc w/ type II Chiari defect develops in at least 80% of patients TREATMENT : = multidisciplinary team approach PROGNOSIS : Dr. MENINGOCELE = formed when meninges herniate through a defect in the post vertebral arches = SC usually normal = (+) fluctuant midline mass that transilluminate along the vertebral column = well covered with skin & pose no threat to the patient D. discoloration of skin or dermal sinus in the midline of low back signifies a malformation C. time limited change in motor activity &/or behavior that results from abn electrical activity in the brain. bulging ant fontanel = dilated scalp veins. unknown cause. Secondary – results from failure of brain growth & expansion Clinical manifestations: = evident at birth. unilat flattening of forehead. indicating separation of sutures CRANIOSYNOSTOSIS = premature closure of cranial sutures Classification: 1. J. HYDROCEPHALUS = not a specific dse. env’tal & toxic factors G. Primary – refers to closure of one or more sutures due to abnormalities of skull dev’t . MICROCEPHALY = HC that measures more than 3 standard deviations below the mean for age & sex = common among developmentally delayed children = 2 main groups : * primary microcephaly – group of conditions that usually have no other malformations & follow a mendelian pattern of inheritance or are assoc w/ a specific genetic syndrome * secondary microcephaly – results from a large number of noxious agents that may affect a fetus in utero or an infant during periods of rapid brain growth .4 PEDIATRIC NEUROLOGY = recurrence rate is 4% & increases to 10% if couple has had 2 previously affected pregnancies = etiology : genetic basis. incidence approximates 1/2000 births. genetic syndromes account for 10-20% of cases 2. premature fusion of coronal & sphenofrontal suture SEIZURES IN CHILDHOOD Seizure or convulsion – paroxysmal. a condition in w/c seizures are triggered recurrently from w/in the brain = EPILEPSY – considered when 2 or more unprovoked seizures occur at an interval greater than 24 hrs apart Clinical manifestations: = accelerated rate of head enlargement = wide open. particularly the 1st 2 yrs of life H. broad forehead . low SES. most common form of craniosynostosis = frontal plagiocephaly – next most common. represents a diverse group of conditions that result from impaired circulation & absorption of CSF or from increased production by a choroid plexus papilloma = normal CSF prodn – 20 ml/hr = total volume – 50 ml in infants Obstructive or noncommunicating hydrocephalus – hydrocephalus resulting from obstruction within the ventricular system Nonobstructive or communicating hydrocephalus – hydrocephalus resulting from obliteration of the subarachnoid cisterns or malfunction of the arachnoid villi I. = setting-sun eye sign Dr. = occur in approx 10% of children = less than one third of seizures in children are caused by EPILEPSY. characterized by skull deformity = palpation of suture reveals prominent bony ridge = scaphocephaly – premature closure of sagittal suture producing a long & narrow skull. nutritional & vitamin deficiencies. Wangdali = (+) Macewen sign – percussion of skull produce a cracked-pot. elevation of ipsilat orbit or eyebrow. First seizure = LP is of limited value in a child w/ a 1st nonfebrile seizure = EEG recommended in a child w/ an apparent 1st unprovoked seizure = anticonvulsants generally not recommended after a single seizure . Partial Seizures = account for 40% of cases = classified as simple or complex = consciousness is maintained with simple seizures. not recalled by child B. Febrile Seizures = most common seizure disorder during childhood = excellent prognosis = may signify a serious underlying acute infectious dse such as bacterial meningitis = age dependent. develop after the LOC & may persist into the postictal phase. repeated convulsions occur w/in 24 hr or when focal sz activity or focal findings are present during postictal period LP should be strongly considered in children <12 mo of age & in those 12-18 mo of age especially if seizures are complex or sensorium remains clouded after a short postictal period 2. occurs in 5075% of cases.5 PEDIATRIC NEUROLOGY A. Recurrent seizures Dr.SIMPLE PARTIAL SEIZURES : motor activity is the most common symptom asynchronous clonic or tonic mov’t involving the face.headache) versive seizures consisting of head turning & conjugate eye mov’ts are common 10-20 sec Partial Seizures : SPS distinguishing characteristic: px remain conscious & may verbalize during the seizure no postictal phenomenon EEG – spikes or sharp waves unilat or bilat or multifocal spike pattern 2. rare before 9 mos & after 5 y/o = genetic predisposition = peak age of onset – 14-18 mo of age = febrile seizure gene – chromosomes 19p & 8q13-21 Clinical manifestations : = GTC seizure. occurs only once in 24 hr = viral URTI.neck & extremities automatisms do not occur but some may complain of aura (chest discomfort. impaired in complex seizures 1. Wangdali = 2 unprovoked seizures greater than 24 hrs apart suggest presence of an epileptic dso w/in the brain that will lead to future recurrences C. J. roseola & acute otitis media are frequent causes * Complex/complicated febrile seizure: >15 min duration. lasts for few sec to 10 mins = followed by brief postictal period of drowsiness. Unprovoked Seizures 1. COMPLEX PARTIAL SEIZURES (CPS) may begin with a SPS with or w/o aura ff by impaired consciousness presence of an aura always indicates a focal onset of the seizure automatisms – common feature. emit a shrill. piercing cry eyes roll back. motor signs & somatosensory symptoms are confined to face = oropharyngeal symptoms : tonic contractions & paresthesias of tongue. BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC) : excellent prognosis clinical features. rarely persist longer than 30 seconds countless seizures daily. often symmetric muscular contractions w/ loss of body tone & falling or slumping forward 4. guttural noises. dysphagia & excessive salivation = consciousness may be intact or impaired = Carbamazepine – preferred drug.6 PEDIATRIC NEUROLOGY Partial Seizures : CPS = ave duration – 1-2 min = prolonged & repetitive alimentary automatisms assoc with blank stare or with lack of responsiveness almost always indicate CPS in an infant = tends to be observed during waking hours prevalent in girls Dr. EEG findings(rolandic foci) & lack of neuropathologic lesion are characteristic & separate BPEC from CPS occurs bet. trunk & extremities = 3 types: flexor. do not lose body tone but head may fall forward slightly 2.when spontaneous remission of BPEC usually occurs Generalized Seizures:GTC Seizures clonic phase is heralded by rhythmic clonic contractions alternating w/ relaxation of all muscle grps (+) loss of sphincter control postictally. MYOCLONIC EPILEPSIES: characterized by repetitive seizures consisting of brief . J. not assoc w/ postictal state. GENERALIZED TONIC-CLONIC SEIZURES may follow a partial seizure w/ a focal onset may be assoc w/ aura px suddenly lose consciousness. patients are semicomatose. unilat numbness of cheek. INFANTILE SPASMS: = begin bet ages 4-8 mos = characterized by brief symmetric contractions of the neck. Generalized Seizures : 1.entire body undergoes tonic contractions & become cyanotic in assoc w/ apnea 3. ABSENCE SEIZURES (PETIT MAL) sudden cessation of motor activity or speech w/ a blank facial expression & flickering of eyelids uncommon before 5 y/o . Wangdali never assoc with aura. continued for at least 2 yr or until 14-16 yrs old. peak age of onset is 9-10 y/o occurs in normal children w/ unremarkable hx & normal neuro exam (+) FHx of epilepsy occurs during sleep in 75% of px = seizures usually partial. remain in deep sleep from 30 mins to 2 hrs postictal phase is assoc w/ vomiting & intense bifrontal headache 3. extensor & mixed = a cry may precede or follow an infantile spasm D. 2-14 y/o. Symptomatic infantile spasms related directly to several prenatal. normal dev’tal milestones before onset of seizures normal neuro exam. determined by the hx & EEG findings 7. Benzodiazepines: exert its activity by binding to a specific GABA site = Diazepam & lorazepam IV are used for initial mgt of status epilepticus . ACTH: = preferred drug for infantile spasms 6. Phenobarbital : = safe. Ketogenic Diet = for mgt of recalcitrant seizures = restricts the quantity of COH & CHON. most calories are provided as fat = fatty. J. useful for GTC seizures = 25% undergo severe behavioral changes = affect the cognitive performance of children treated on a long term basis = acts on the GABA receptor to increase the chloride channel open duration 2. mean onset of 5 ½ yr = characterized by loss of language skills in a previously normal child = 70% have assoc seizure disorder = Valproic acid – drug of choice 5. unpalatable diet = MOA is unknown but some evidence shows that it exerts an anticonvulsant effect sec to elevated levels of B-hydroxy-butyrate & acetoacetate resulting from ketosis TREATMENT OF EPILEPSY: 1st step – ensure that patient has a seizure disorder 2nd step – choose an anticonvulsant depending on the classif of seizure.prematurity. Carbamazepine: Generalized Seizures:Infantile Spasms 2 groups: Dr. congenital infections. Phenytoin: = acts by decreasing the sustained repetitive firing of single neurons by blocking Nadependent channels & decreasing Ca uptake 5. normal CT/MRI scans good prognosis 3. Surgery: shld be considered for children w/ intractable seizures unresponsive to anticonvulsants 1.perinatal & postnatal factors HIE. CNS infections. inborn errors of metabolism.7 PEDIATRIC NEUROLOGY = may occur during sleep or arousal 2. Wangdali = effective in the mgt of GTC & partial sz = acts by decreasing the sustained repetitive firing of neurons by blocking Na-dependent channels & by decreasing depolarizationdependent Ca uptake 1. Cryptogenic infantile spasms has uneventful pregnancy & birth hx. head trauma 80-90% risk of MR 4.LANDAU-KLEFFNER SYNDROME (LKS) : = rare condition of unknown cause = more common in boys. Vagal Nerve Stimulation: animal experiments have shown that electrical stimulation of left vagal nerve will interrupt or prevent seizures HEADACHES Dr. boys are in slight majority among children younger than 10 yr = more than half undergo spontaneous prolonged remission after the 10th birthday * Cortical Spreading Depression (CSD) =phenomenon thought to be responsible for the aura in migraine.assoc aura(visual. bicycling or pedaling mov’ts * HIE – most common cause of neonatal seizures STATUS EPILEPTICUS continuous convulsion lasting longer than 30 min or the occurrence of serial convulsions bet w/c there is no return of consciousness medical emergency! most common cause: febrile seizure lasting > 30 min. particularly those of extremities & face 2.unilat location. Tonic seizures = rigid posturing of the extremities & trunk. Multifocal clonic seizures = similar to focal clonic sz but many muscle grps are involved. blinking. assoc w/ elevation of CNS H+ & K+ ions w/ release of glutamate & nitrous oxide = inherited physiologic response to a variety of stimuli that are responsible for triggering migraine process NEONATAL SEIZURES: Five seizure types: 1. sometimes assoc w/ fixed deviation of the eyes 4. throbbing headache. Myoclonic seizures = brief. J. Subtle seizures consists of chewing motions. focal or generalized jerks of the extremities or body that tend to involve distal muscle grps 5. N/V. Focal seizures = rhythmic twitching of muscle grps. increased ICP and psychogenic factors or stress less common causes: EOR.sensory. nystagmus. Wangdali common problem in children may occasionally indicate a severe underlying dso (brain tumor) most impt causes : migraine. Migraine Without Aura = not assoc w/ aura = most prevalent type . sinusitis & malocclusion of teeth often an associated manifestation of common head & neck infections in children A.relief ff sleep & a (+) FHx = most impt & frequent type of headache in pediatrics = girls are more likely to develop migraine as adolescents. frequently several simultaneously 3.motor). particularly in a child younger than 3 y/o CLASSIFICATION OF MIGRAINE: 1. strabismus. MIGRAINE = recurrent headache w/ symptom free intervals & at least 3 of the ff symptoms : abd pain. excessive salivation & alterations in the RR incldg apnea.8 PEDIATRIC NEUROLOGY 8. sometimes monthly bouts of severe vomiting vomiting during attacks occurs at least 5x/hr for at least 1 hr w/ complete resolution of symptoms between attacks b. hotdog. meningitis. hydrocephalus. Cyclic Vomiting : recurrent. N/V. most efficacious during early stages of migraine attacks 2. scotoma. photopsia. butorphanol & meperidine for severe headache = Ergotamine prep for older children w/ severe. ketorolac. generalized = more prominent over the frontal & occipital regions = insidious onset = causes: brain tumors. Migraine with an Aura = an aura precedes onset of headache = (+) sensory symptoms like perioral paresthesias & numbness of hands & feet = visual auras take the form of blurred vision. classic migraine. chinese food. J. anxiety = AVOIDANCE of certain foods: nuts.9 PEDIATRIC NEUROLOGY = throbbing or pounding. osmophobia & paresthesias of hands & feet Dr. cola drinks. chocolate. unilateral at onset or throughout its duration = located in the bifrontal or temporal regions = persists for 1-3 hrs. cerebral abscess.510 mg/kg) = Naproxen. encephalitis.phonophobia. photophobia. citrus fruits. ORGANIC HEADACHES = headache may be the earliest symptom of increased ICP = headache results from tension or traction of cerebral bld vessels & dura = occurs in the early hrs in the morning or shortly after the patient arises = headache is diffuse. usually midline child must complain at the time of the abdominal pain of at least 2 of the ff: anorexia. patient develops typical symptoms of migraine B. pocessed meats = mgt of acute attacks : analgesics & antiemetics = Acetaminophen (15mg/kg) or Ibuprofen (7. Abdominal Migraine: recurrent disorder characterizedby mid-abdominal pain w/ pain-free periods between each attack . persist from 1-72 hr. codeine. spicy meats. yogurt. fatigue. fried foods. light-headedness. Wangdali pain is described as dull. or pallor TREATMENT OF MIGRAINE: = avoid initiating stimuli = most common precipitators : stress. cheese.although pain may last as long as 72 hr = inhibit daily activity =(+) FHX particularly on maternal side is present in 90% of children = characteristic feature of childhood migraine – intense N/V w/c may be more bothersome than the headache = vomiting may be assoc w/ abdominal pain & fever = additional symptoms : extreme paleness. fortification spectra or irregular distortion of objects = after the aura. 3. Childhood Periodic Syndromes that are Commonly Precursors of Migraine a. may be moderate to severe. anorexia. disturbed sleep. J.10 PEDIATRIC NEUROLOGY subdural hematoma. Wangdali . particularly after the onset of puberty most apparent during the school day tend to wax & wane & build in intensity during the day not assoc w/ nausea & vomiting! patients also complain of mood changes. fatigue & withdrawal from social activities depressed child occasionally presents w/ severe headaches Dr. chronic lead poisoning. wt loss. TENSION OR STRESS HEADACHE common in pediatric age group. pseudotumor cerebri C.