Neurologic Evaluation

Transcript

1 Dr. J. Wangdali NEUROLOGIC EVALUATION  2 = active mov’t, gravity eliminated A. HISTORY 3 = active mov’t against gravity = most important component of the evaluation evaluation of a child with a neurologic problem 4 = active mov’t against gravity & resistance 5 = normal power = child’s dev’tal assessment is the most important component *Abnormalities of tone: = family hx is extremely important 1. Spasticity = initial resistance to passive mov’t mov’t ff by a sudden release called the clasp-knife phenomenon,  . NEURO EXAM most apparent in the upper extremity flexors & LE extensor muscles 1. Head – size & shape shld be documented   -2 fontanels at birth: posterior fontanel is st usually closed & nonpalpable after the 1 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 st ave. rate of head growth in PT: 0.5 cm in the 1 2 wk, 0.75 cm during the 3rdwk & 1 cm in the th th 4 wk & thereafter until the 40 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 t he 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 = 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 le gs 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: 1. Ataxia = incoordination 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 2 Dr. J. Wangdali 3. Athetosis = slow, writhing mov’t 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 mov’t that primarily involves the prox muscles of the extremities, trunk & neck 5. Motor tics = sudden brief unsustained mov’t, suppressible & preceded by a warning sensation = eye blinking, grimacing, brief rapid mov’t 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 * Primitive reflexes : appear & disappear in se quence during specific periods of dev’t. ; their absence or persistence beyond a given time frame signifies dysfx o f  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 = 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, r adionuclide 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 t o close spontaneously bet the 3rd & 4th wk of in utero dev’t = precise cause is unknown B. SPINA BIFIDA OCCULTA = consists of a midline defect of vertebral bodies without protrusion of SC or meninges 3 Dr. J. Wangdali = 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 PROGNOSIS : = 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 = (+) fluctuant midline mass that transilluminate along the vertebral column = 2 major forms of disraphism affect the skull, re sulting in protrusion of tissue through a bony midline defect, called cranium bifidum = well covered with skin & pose no t hreat to the patient = cranial meningocele – consists of a CSF-filled meningeal sac only D. MYELOMENIGOCELE = cranial encephalocele – contains the sac plus cerebral cortex, cerebellum or portions of brainstem = 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 th conception & continued until 12 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 = 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 c ystlike 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 t ube = primitive brain consists of portions of CT, vessels & neuroglia = die w/in several days of birth = incidence is 1/1000 LB 4 Dr. J. Wangdali = recurrence rate is 4% & increases to 10% if couple has had 2 previously affected pregnancies   = etiology : genetic basis; low SES; nutritional & vitamin deficiencies; env’tal & toxic factors G. MICROCEPHALY = HC that measures more than 3 standard deviations below the mean for age & sex I. = premature closure of cranial sutures Classification: 1. Primary – refers to closure of one or more sutures due to abnormalities of skull dev’t ; incidence approximates 1/2000 births; unknown cause, genetic syndromes account for 10-20% of cases = 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 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 Clinical manifestations:  = accelerated rate of head enlargement  = wide open, bulging ant fontanel  = dilated scalp veins, broad forehead = (+) Macewen sign – percussion of skull produce a cracked-pot, indicating separation of  sutures CRANIOSYNOSTOSIS = common among developmentally delayed children * 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 st the 1 2 yrs of life = setting-sun eye sign 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 5 Dr. J. Wangdali A. Febrile Seizures 2. Recurrent 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 = 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 : = febrile seizure gene – chromosomes 19p & 8q13-21  motor activity is the most common symptom  asynchronous clonic or toni c mov’t involving the face,neck & extremities  = followed by brief postictal period of drowsiness; occurs only once in 24 hr automatisms do not occur but some may complain of aura (chest discomfort,headache)  = viral URTI, roseola & acute otitis media are frequent causes versive seizures consisting of head turning & conjugate eye mov’ts are common  10-20 sec Clinical manifestations : = GTC seizure, lasts for few sec to 10 mins * 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 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) B. Unprovoked Seizures  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 1. First seizure = LP is of limited value in a c hild w/ a 1 nonfebrile seizure st = EEG recommended in a child w/ an apparent st 1 unprovoked seizure = anticonvulsants generally not recommended after a single seizure 6 Dr. J. Wangdali 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 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 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  prevalent in girls  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 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 7 Dr. J. Wangdali  = may occur during sleep or arousal Generalized Seizures:Infantile Spasms  2 groups: 2. Carbamazepine:  1. Cryptogenic infantile spasms  has uneventful pregnancy & birth hx; normal dev’tal milestones before onset of seizures  normal neuro exam; normal CT/MRI scans  good prognosis 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 = rare condition of unknown cause  = more common in boys; 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 TREATMENT OF EPILEPSY: st  1 step – ensure that patient has a seizure disorder  2 step – choose an anticonvulsant depending on the classif of seizure, determined by the hx & EEG findings  = 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 4. Phenytoin:    exert its activity by binding to a specific GABA site = Diazepam & lorazepam IV are used for initial mgt of status epilepticus = acts by decreasing the sustained repetitive firing of single neurons by blocking Nadependent channels & decreasing Ca uptake 5. ACTH:  = preferred drug for infantile spasms 6. Ketogenic Diet     nd 1. Benzodiazepines: = acts by decreasing the sustained repetitive firing of neurons by blocking Na-dependent channels & by decreasing depolarizationdependent Ca uptake 3. Phenobarbital : 5.LANDAU-KLEFFNER SYNDROME (LKS) :  = effective in the mgt of GTC & partial sz  = 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 7. Surgery:  shld be considered for children w/ intractable seizures unresponsive to anticonvulsants 8 Dr. J. Wangdali 8. Vagal Nerve Stimulation:  animal experiments have shown that electrical stimulation of left vagal nerve will interrupt or prevent seizures NEONATAL SEIZURES:   common problem in children  may occasionally indicate a severe underlying dso (brain tumor)  most impt causes : migraine, increased I CP 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 th prolonged remission after the 10 birthday Five seizure types: 1. Focal seizures  HEADACHES = 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 & tr unk, 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 mov’ts   =phenomenon thought to be responsible for the aura in migraine; assoc w/ e levation 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 * 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 * Cortical Spreading Depression (CSD) CLASSIFICATION OF MIGRAINE: 1. Migraine Without Aura  = not assoc w/ aura  = most prevalent type 9 Dr. J. Wangdali  = throbbing or pounding; unilateral at onset or throughout its duration  pain is described as dull, may be moderate to severe; persist from 1-72 hr; usually midline  = located in the bifrontal or temporal regions   = persists for 1-3 hrs,although pain may last as long as 72 hr child must complain at the time of t he abdominal pain of at least 2 of the ff: anorexia, N/V, or pallor    = 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 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 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 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 & o ccipital regions = insidious onset = causes: brain tumors, hydrocephalus, meningitis, encephalitis, cerebral abscess, 10 Dr. J. Wangdali 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