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PEDIATRIC NEUROLOGY
NEUROLOGIC EVALUATION A. HISTORY

Dr. J. Wangdali

2 = active movt, gravity eliminated 3 = active movt against gravity 4 = active movt against gravity & resistance 5 = normal power *Abnormalities of tone: 1. Spasticity = initial resistance to passive movt 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 movt 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 = childs devtal 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 devt 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 movts 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 movt or disturbance of balance *Abnormalities assoc w/ cerebellar lesions: a. Dysmetria b. Rebound c. disdiadochokinesia 2. Chorea = involuntary movts of the major joints, trunk & the face that are rapid & jerky. = hand grip contracts & relaxes (milkmaid sign), speech is explosive & inarticulate

PEDIATRIC NEUROLOGY
3. Athetosis = slow, writhing movt that is assoc often with abnormalities of muscle tone, most prominent in the distal extremities & is enhanced by voluntary activity or emotional upset. 4. Dystonia = involuntary, slow, twisting movt that primarily involves the prox muscles of the extremities, trunk & neck 5. Motor tics = sudden brief unsustained movt, suppressible & preceded by a warning sensation = eye blinking, grimacing, brief rapid movt of head & shoulders DTRs : graded from 0 (absent) to 4 (markedly hyperactive), with 2 being normal DTRs are absent or decreased in primary dso of muscle, nerve & myoneural junction & abn of cerebellum Babinski reflex = indicates an UMN lesion; characterized by extension of great toe & fanning of remaining toes

Dr. J. Wangdali

= interpreted cautiously bec they are present in normal children during various stages of neurodevelopment = variation in expression of these signs , depending on age, sex & maturation of nervous system C. DIAGNOSTIC PROCEDURES:

1. Lumbar puncture = lat recumbent position, = 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. 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. Ventricular tap = used to remove CSF in increased ICP assoc with hydrocephalus 4. Neuroradiologic procedures = skull x rays, CT scan, MRI, radionuclide brain scan 5. EEG = provides continuous recording of electrical activity with electrodes placed on the scalp CONGENITAL ANOMALIES OF THE CNS A. NEURAL TUBE DEFECTS (DYSRAPHISM) = account for most congenital anomalies of CNS; result from failure of the neural tube to close spontaneously bet the 3rd & 4th wk of in utero devt = precise cause is unknown B. 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 devt. ; their absence or persistence beyond a given time frame signifies dysfx of CNS 4. Sensory exam 5. Gait a spastic gait is charac by stiffness & by stepping like a tin soldier

- cerebellar ataxia produces a broad based unsteady gait - a waddling gait results from weakness of the prox hip girdle - 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

PEDIATRIC NEUROLOGY
= asymptomatic, lack neuro signs = patches of hair, lipoma, discoloration of skin or dermal sinus in the midline of low back signifies a malformation C. 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. 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; 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. J. Wangdali

= mortality rate 10-15%; most deaths occur before 4 y/o = 70% survivors have normal intelligence, but learning problems & seizure dso more common = chronic handicapping condition E. ENCEPHALOCELE = 2 major forms of disraphism affect the skull, resulting in protrusion of tissue through a bony midline defect, called cranium bifidum = cranial meningocele consists of a CSF-filled meningeal sac only = cranial encephalocele contains the sac plus cerebral cortex, cerebellum or portions of brainstem = occurs most commonly in the occipital region but in certain parts of the world, 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. ANENCEPHALY : = presents a distinctive appearance with a large defect of the calvarium, meninges & scalp assoc with a rudimentary brain = results from failure of closure of the rostral neuropore, opening of the anterior neural tube = primitive brain consists of portions of CT, vessels & neuroglia = die w/in several days of birth = incidence is 1/1000 LB

PEDIATRIC NEUROLOGY
= recurrence rate is 4% & increases to 10% if couple has had 2 previously affected pregnancies = etiology : genetic basis; low SES; nutritional & vitamin deficiencies; envtal & toxic factors G. 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 , particularly the 1st 2 yrs of life H. HYDROCEPHALUS = not a specific dse, 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. J. Wangdali

= (+) Macewen sign percussion of skull produce a cracked-pot, indicating separation of sutures CRANIOSYNOSTOSIS = premature closure of cranial sutures

Classification: 1. Primary refers to closure of one or more sutures due to abnormalities of skull devt ; incidence approximates 1/2000 births; unknown cause, genetic syndromes account for 10-20% of cases 2. Secondary results from failure of brain growth & expansion Clinical manifestations:

= evident at birth, characterized by skull deformity = palpation of suture reveals prominent bony ridge = scaphocephaly premature closure of sagittal suture producing a long & narrow skull; most common form of craniosynostosis = frontal plagiocephaly next most common; unilat flattening of forehead, elevation of ipsilat orbit or eyebrow; premature fusion of coronal & sphenofrontal suture SEIZURES IN CHILDHOOD Seizure or convulsion paroxysmal, time limited change in motor activity &/or behavior that results from abn electrical activity in the brain.

= occur in approx 10% of children = less than one third of seizures in children are caused by EPILEPSY, 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, bulging ant fontanel = dilated scalp veins, broad forehead

PEDIATRIC NEUROLOGY
A. Febrile Seizures = most common seizure disorder during childhood = excellent prognosis = may signify a serious underlying acute infectious dse such as bacterial meningitis = age dependent, 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, lasts for few sec to 10 mins = followed by brief postictal period of drowsiness; occurs only once in 24 hr = viral URTI, roseola & acute otitis media are frequent causes * Complex/complicated febrile seizure: >15 min duration; 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. Recurrent seizures

Dr. J. 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. Partial Seizures = account for 40% of cases = classified as simple or complex = consciousness is maintained with simple seizures, impaired in complex seizures 1.SIMPLE PARTIAL SEIZURES : motor activity is the most common symptom asynchronous clonic or tonic movt involving the face,neck & extremities automatisms do not occur but some may complain of aura (chest discomfort,headache) versive seizures consisting of head turning & conjugate eye movts 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. 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; occurs in 5075% of cases; develop after the LOC & may persist into the postictal phase; not recalled by child

B. Unprovoked Seizures 1. 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

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. J. Wangdali

never assoc with aura; not assoc w/ postictal state; rarely persist longer than 30 seconds countless seizures daily; do not lose body tone but head may fall forward slightly

2. GENERALIZED TONIC-CLONIC SEIZURES may follow a partial seizure w/ a focal onset may be assoc w/ aura px suddenly lose consciousness, emit a shrill, piercing cry eyes roll back,entire body undergoes tonic contractions & become cyanotic in assoc w/ apnea

3. BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC) : excellent prognosis clinical features, EEG findings(rolandic foci) & lack of neuropathologic lesion are characteristic & separate BPEC from CPS occurs bet. 2-14 y/o, 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; motor signs & somatosensory symptoms are confined to face = oropharyngeal symptoms : tonic contractions & paresthesias of tongue, unilat numbness of cheek, guttural noises, dysphagia & excessive salivation = consciousness may be intact or impaired = Carbamazepine preferred drug; continued for at least 2 yr or until 14-16 yrs old,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, patients are semicomatose, remain in deep sleep from 30 mins to 2 hrs postictal phase is assoc w/ vomiting & intense bifrontal headache

3. MYOCLONIC EPILEPSIES: characterized by repetitive seizures consisting of brief , often symmetric muscular contractions w/ loss of body tone & falling or slumping forward

4. INFANTILE SPASMS: = begin bet ages 4-8 mos = characterized by brief symmetric contractions of the neck, trunk & extremities = 3 types: flexor, extensor & mixed = a cry may precede or follow an infantile spasm

D. Generalized Seizures : 1. ABSENCE SEIZURES (PETIT MAL) sudden cessation of motor activity or speech w/ a blank facial expression & flickering of eyelids uncommon before 5 y/o

PEDIATRIC NEUROLOGY
= may occur during sleep or arousal 2. Carbamazepine: Generalized Seizures:Infantile Spasms 2 groups:

Dr. J. 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; normal devtal milestones before onset of seizures normal neuro exam; normal CT/MRI scans good prognosis

3. Phenobarbital : = safe, 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. Symptomatic infantile spasms related directly to several prenatal,perinatal & postnatal factors HIE, congenital infections, inborn errors of metabolism,prematurity, CNS infections, head trauma 80-90% risk of MR

4. Phenytoin: = acts by decreasing the sustained repetitive firing of single neurons by blocking Nadependent channels & decreasing Ca uptake

5.LANDAU-KLEFFNER SYNDROME (LKS) : = rare condition of unknown cause = more common in boys; mean onset of 5 yr = characterized by loss of language skills in a previously normal child = 70% have assoc seizure disorder = Valproic acid drug of choice

5. ACTH: = preferred drug for infantile spasms

6. Ketogenic Diet = for mgt of recalcitrant seizures = restricts the quantity of COH & CHON, most calories are provided as fat = fatty, 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, determined by the hx & EEG findings

7. Surgery: shld be considered for children w/ intractable seizures unresponsive to anticonvulsants

1. Benzodiazepines: exert its activity by binding to a specific GABA site = Diazepam & lorazepam IV are used for initial mgt of status epilepticus

PEDIATRIC NEUROLOGY
8. Vagal Nerve Stimulation: animal experiments have shown that electrical stimulation of left vagal nerve will interrupt or prevent seizures HEADACHES

Dr. J. Wangdali

common problem in children may occasionally indicate a severe underlying dso (brain tumor) most impt causes : migraine, increased ICP and psychogenic factors or stress less common causes: EOR; strabismus; sinusitis & malocclusion of teeth often an associated manifestation of common head & neck infections in children A. MIGRAINE = recurrent headache w/ symptom free intervals & at least 3 of the ff symptoms : abd pain, N/V, throbbing headache,unilat location,assoc aura(visual,sensory,motor),relief ff sleep & a (+) FHx = most impt & frequent type of headache in pediatrics = girls are more likely to develop migraine as adolescents; 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 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. Focal seizures = rhythmic twitching of muscle grps, particularly those of extremities & face

2. Multifocal clonic seizures = similar to focal clonic sz but many muscle grps are involved, frequently several simultaneously

3. Tonic seizures = rigid posturing of the extremities & trunk, sometimes assoc w/ fixed deviation of the eyes

4. Myoclonic seizures = brief, focal or generalized jerks of the extremities or body that tend to involve distal muscle grps

5. Subtle seizures consists of chewing motions, excessive salivation & alterations in the RR incldg apnea, blinking, nystagmus, bicycling or pedaling movts

* 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 in a child younger than 3 y/o

CLASSIFICATION OF MIGRAINE: 1. Migraine Without Aura = not assoc w/ aura = most prevalent type

PEDIATRIC NEUROLOGY
= throbbing or pounding; unilateral at onset or throughout its duration = located in the bifrontal or temporal regions = persists for 1-3 hrs,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, photophobia, light-headedness,phonophobia, osmophobia & paresthesias of hands & feet

Dr. J. Wangdali

pain is described as dull, may be moderate to severe; persist from 1-72 hr; usually midline child must complain at the time of the abdominal pain of at least 2 of the ff: anorexia, N/V, or pallor

TREATMENT OF MIGRAINE: = avoid initiating stimuli = most common precipitators : stress, fatigue, anxiety = AVOIDANCE of certain foods: nuts, chocolate, cola drinks, hotdog, spicy meats, chinese food, citrus fruits, fried foods, cheese, yogurt, pocessed meats = mgt of acute attacks : analgesics & antiemetics = Acetaminophen (15mg/kg) or Ibuprofen (7.510 mg/kg) = Naproxen, ketorolac, codeine, butorphanol & meperidine for severe headache = Ergotamine prep for older children w/ severe, classic migraine, most efficacious during early stages of migraine attacks

2. 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, scotoma, photopsia, fortification spectra or irregular distortion of objects = after the aura, patient develops typical symptoms of migraine

B. 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, generalized = more prominent over the frontal & occipital regions = insidious onset = causes: brain tumors, hydrocephalus, meningitis, encephalitis, cerebral abscess,

3. Childhood Periodic Syndromes that are Commonly Precursors of Migraine a. Cyclic Vomiting : recurrent, 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. Abdominal Migraine: recurrent disorder characterizedby mid-abdominal pain w/ pain-free periods between each attack

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PEDIATRIC NEUROLOGY
subdural hematoma, chronic lead poisoning, pseudotumor cerebri C. TENSION OR STRESS HEADACHE common in pediatric age group, 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, wt loss, anorexia, disturbed sleep, fatigue & withdrawal from social activities depressed child occasionally presents w/ severe headaches

Dr. J. Wangdali