Nervous system is divided into two parts: the CNS and the peripheral nervous system (PNS). The CNS consists of the brain and spinal cord. The PNS can be subdivided into three groups based on function: the somatic nervous system, the autonomic nervous system (ANS), and the enteric nervous system. Anatomic Units of the Nervous System and Functions Anatomic Unit Functions 1. Frontal area: Anterior—decision-making, emotions, memory, judgment, ethics, abstract thinking Broca area—speech 2. Parietal area: Sensory integration, language, reading, writing, pattern recognition 3. Temporal area: Memory storage, auditory processing, olfaction, limbic system in deep temporal lobe—arousal 4. Occipital area: Visual processing 5.Thalamus: Receives and sorts sensory input, modulates motor impulses from cortex 6.Hypothalamus: Integrates autonomic functions 7.Cerebellum: Coordination and movement; balance; smooth movements Assessment of the Nervous System History: onset, duration, pain, sensory deficits, injury, reflexes responses, motor balance changes Medical history: Prenatal history Birth history and neonatal course, injuries, infection, cardiovascular respirator system… Physical exam: growth pattern, anomalies, cardiovascular system, musculoskeletal system, tanner stage, vision, hepatomegaly/splenomegaly Tanner Stage Pubic Hair Scale (both males and females) Stage 1: No hair Stage 2: Downy hair Stage 3: Scant terminal hair Stage 4: Terminal hair that fills the entire triangle overlying the pubic region Stage 5: Terminal hair that extends beyond the inguinal crease onto the thigh Female Breast Development Scale Stage 1: No glandular breast tissue palpable Stage 2: Breast bud palpable under the areola (1st pubertal sign in females) Stage 3: Breast tissue palpable outside areola; no areolar development Stage 4: Areola elevated above the contour of the breast, forming a “double scoop” appearance Stage 5: Areolar mound recedes into single breast contour with areolar hyperpigmentation, papillae development, and nipple protrusion Cranial Nerve Nerve Mnemonic Young Child Older Child 1 Olfactory Difficult to assess, typically not present prior to 5-7 months of age; grossly assess by observing grimace/response to strong odors Differentiation of smells 2 Optic Recognition of objects introduced into visual fields; funduscopic exam if tolerated Visual acuity, visual fields, funduscopic exam 3,4,6 Oculomotor Pupillary response to light, extraocular movements with tracking object of interest, ptosis Pupillary response to light, extraocular movements, ptosis Trochlear Abducens 5 Trigeminal Grossly assess by noting response to light facial touch Sensation to light facial touch, temporalis and masseter strength 7 Facial Note symmetry of facial expressions Note symmetry of facial expressions 8 Vestibulocochlear Response to soft sounds made outside of visual fields, observe for lateralization of sounds Weber and Rinne; can use whisper test 9, 10 Glossopharyngeal Symmetrical rise of soft palate, gag reflex Symmetrical rise of soft palate, gag reflex Vagus 11 Accessory Difficult to assess if unable to follow directions but can evaluate symmetry of sternocleidomastoid (SCM) movements Turns head against resistance, shoulder shrug 12 Hypoglossal Tongue lies and/or protrudes midline Tongue protrudes midline Memnomic 1. Only = Olfactory (CN I) 2. One = Optic (CN II) 3. Of = Oculomotor (CN III) 4. The = Trochlear (CN IV) 5. Two = Trigeminal (CN V) 6. Athletes = Abducens (CN VI) 7. Felt = Facial (CN VII) 8. Very = Vestibulocochlear (CN VIII) 9. Good = Glossopharyngeal (CN IX) 10. Victorious = Vagus (CN X) 11. And = Accessory (XI) 12. Healthy = Hypoglossal (XII) This will help you remember the following: 1. Some = Olfactory (Sensory) 2. Say = Optic (Sensory) 3. Marry = Oculomotor (Motor) 4. Money = Trochlear (Motor) 5. But = Trigeminal (Both) 6. My = Abducens (Motor) 7. Brother = Facial (Both) 8. Says = Vestibulocochlear (Sensory) 9. Big = Glossopharyngeal (Both) 10. Brains = Vagus (Both) 11. Matter = Accessory (Motor) 12. Most = Hypoglossal (Motor) Infant cry High pitched with increased intracranial pressure, it resembles mewing in cri du chat syndrome, and it is hoarse with hypothyroidism). Coordination test: Tandem gait, Romberg test Children head measurement unti 36 months (CDC) Anterior fontanelle should normally be slightly depressed with very faintly perceived pulsations until its closure between 18 and 24 months. Autonomic Nervous System Alterations in blood pressure, sweating, or body temperature can be indicators of ANS problems. Meningeal irritation, such as with meningitis, includes positive Kernig and Brudzinski signs Diagnostic test CT scan, MRI PET LP, EEG medications: antiepileptic drugs (AEDs), mood stabilizers, and antidepressants HEADACHE Pediatric Migraine headache: 2-72 hr, bil or unilateral (bilateral in young children, unilateral in adolescence or adult), frontal/temporal, Occipital -need eval asap Pulsating quality. Moderate to severe intensity by routine physical activity, nausea/vomit and/or photophobia and phonophobia Tension Headache: at least 10 episode/month. 30 min to 7 days, bilateral location, non-pulsating, mild.moderate intensity, not trigger by physical, no nausea/vomit and no photophobia, phonophonia (sound). The pain is dull and bifrontal or occipital, with nausea and vomiting occurring only rarely; there is no prodrome. Chronic tension headache: tension headache criteria + occur 15 days per month and more than 3 months Migraine with aura: 1/3 patient, prodome precede headache up to 24 hr (irritability, mood swing, food, craving/anorexia, water retention, sleep disturbance) Severe, unilateral often throbbing headache follows aura >30 min later; lasts 5-20 min; may generalize About 5% of children with classic migraine do not have headache with their auras Migraine without aura: 2/3 patient, same prodrome more profound commonly bilateral, orbital, or frontotemporal; pain may radiate to face, occiput, neck Headache throbbing or pulsating; typically lasts <4 h but can last up to 72 h; moderate or severe intensity ctivity aggravates, and sleeps relives headache Nausea or vomiting or both, Sensitivity to light, sound, and movement Secondary headache: immediate referral: headache awaken from sleep, worst headache, vision change, pain, neck pain, irritability, fever, family or patient hx neurologic disorder. Diagnostic: MRI (1 choice), CT, Pet, EEG, cbc, iron study, vitamin d.Image within 2-4 weeks . Medication: NSAID (1 line), apap, Zofran, triptans (only sumatriptan and zolmitriptan safe in children) Frequent migraine: β-blockers (propanol), antidepressants( amitriptyline), anticonvulsants (depakote, topiramate), or calcium channel blockers Magnesium, Coenzyme Q10, Riboflavin, butterbur, omega-3 fish oil, and Migraleif (riboflavin, puracol, magnesium). Education: avoid trigger, rest, sleep, increase fluid intake, decrease stress. Head injury/ Concussion Various types of head injuries can result in pathologic conditions: skull fracture, concussion, posttraumatic seizure, cerebral contusion, epidural hematoma, subdural hematoma, cerebral edema, and penetrating injury. Children can also experience subtle symptoms of TBI that may not appear until days or weeks after the injury. History: how the injury occur, lost of conscious, vision change, numbness, alter mental status Physical exam: vital sign, throughout physical exam, CNS involment Diagnostic: Children with moderate (GCS 9 to 12) and severe (GCS 3 to 8) acute trauma should have a cranial CT scan. CT require: • Penetrating trauma or depressed skull fracture or signs of basilar injury • Altered level of consciousness , Loss of consciousness (exceeding 1 minute) • Amnesia about the injury, Focal neurologic signs or deficit, Persistent vomiting or seizures, History of coagulopathy Management: level of consciousness is the key determinant Management of the Child With Minor Closed Head Injury and No Loss of Consciousness Observation in the clinic, office, ED, or home, under the care of a competent caregiver Closed Head Injury and Brief Loss of Consciousness/worrisome symptom: hospital Complication Second impact syndrome (SIS) occurs when the brain swells catastrophically, after a person suffers a second concussion prior to resolution of symptoms from an earlier injury. Post concussive syndrome in adolescents is manifested by headache, dizziness, irritability, and impaired ability to concentrate. In younger children, it is manifested as aggression, disobedience, behavioral regression, inattention, and anxiety. Education point: wake 2-4 hr first 24 hr, move arm, leg, ONLY APAP. Change status ER Macrocephaly: measure parent head. Benign macrocephaly is genetic. Metabolic etiology- CT scan Hydrocephalus: symptom increased intracranial pressure, gait disturbance, n/v, esotropia or diplopia cause: trauma, infection, mass, type 1 chiari Malformation Microcephaly: Cause: hypoxic-ischemic Treatment: supportive, brain MRI Craniosynostosis: skull malformation Congenital (or “true” or “primary”) craniosynostosis involves premature fusion of one or more cranial sutures. Physical exam: look down at the top of the head Secondary synostosis results when outside forces put pressure on the growing cranium, causing the skull to become misshapen, referred to as deformational plagiocephaly. Symmetry of neck rotation should also be included in the examination to rule out torticollis. Infants with torticollis typically have some limitation of neck rotation away from the side of their occipital flattening and may require physical therapy for symptom management and parental education. Diagnostic Studies: CT scan, neuroimaging with MRI In about 5% of young infants, the frontal metopic suture may normally be prominent and identified as “frontal bossing. Management If craniosynostosis is suspected, refer the child to an experienced pediatric neurosurgeon or craniofacial plastic surgeon. Epilepsy: cause by unprovoked seizures. seizures are generalized (arising across the cortex) and focal (arising from one specific area of the cortex) Physical Examination: focus on weakness, seizure activity, HTN, cardiovascular, disorder, head trauma, transillumination of the skull in infants. Diagnostic Studies: CBC, platelet, LFTs, metabolic, blood glucose, urine, toxicology, genetic, LP, EEG, MRI, CT scan, polysomnography Management: The goal for treating epilepsy should always be two-fold: no seizures and no side effects Common: Levetiracetam (side effect aggression or depression) Lacosamide, Valproic acid Lamotrigine, Oxcarbazepine, , carbamazepine, phenobarbital (cause StevensJohnson syndrome) Antiepileptic Drug Withdrawa: must taper medication if the child is able to go 2 years without a seizure, over 6-8 weeks, one AED taper each time 6 months off medication, the child should be placed back on AED therapy, typically the same medication and dose prior to withdrawal. Epilepsy can be considered resolved when a child is able to go for 10 years without a seizure, with 5 of those years being off medication Ketogenic diet: considered for children with intractable epilepsy (particularly nonsurgical candidates) or as a first-line treatment for specific types of epilepsy (such as GLUT1 transporter deficiency) Surgical Interventions and vagus nerve stimulator for intractable epilepsy . Counseling: AEDs are teratogenic; therefore, contraception and thorough patient and family education is essential for females of child-bearing age. Tdap contraindicate within 7 days of encephalopathy (coma, seizure, ..) Complication: Status epilepticus (SE) are seizures that may be continuous, or frequent, without recovery between episodes. E refers to a seizure that lasts for greater than 30 minutes or multiple seizures without recovery between events. MEDICAL EMERGENCY (rectal diazepam, intranasal midazolam, buccal clonazepam) Febrile Seizures: above 38 c, occur before or after the seizure, high risk 6-60 month. Febrile Seizures occurring with fever outside of those age parameters warrants further evaluation Simple febrile seizures present as generalized seizures and last for less than 15 minutes. Complex febrile seizures can present with generalized or focal seizures, duration greater than 15 minutes, and/or with clustering of seizures Tests: LP, CBG, EEG, MRI Management: apap, motrin ( for comfort only, wont prevent seizure) protect air way, seizure>5 min, call 911 Education: no prevention, no long-term consequences for simple/complex febrile seizures. Simple seizure manage by pcp. Psychogenic Nonepileptic Seizures (PNES) Have dx epilepsy difficult to distinguish from epileptic seizures, even after direct observation characteristic: unilateral or bilateral, occur before withness, reactive pupils, situation specific, slow onset, no tongue bitting, abrupt recovery, no eeg changes Treatment for PNES is generally directed by psychology/psychiatry and often centers around cognitive behavioral therapy Benign Paroxysmal Vertigo BPV is a syndrome characterized by episodic vertigo. BPV is one of the most common causes of episodic vertigo in children before 4 years old. Family hx migraine. Child present with migraine, consider vestibular migraine Rapit onset, acute unsteadiness, nystagmus, vomit/nausea, lethargic, drowsy. Test: MRI No medication tx due to short duration, BPV resolve between 8-10 years Cerebral Palsy Cerebral palsy (CP) is a chronic nonprogressive motor disorder that is the result of damage to the areas in the brain that control motor function within first few years. Affect multiple systems: vision, lung, skin, cognition, … three major types of CP: (1) spastic, (2) athetoid (or dyskinetic), and (3) ataxic. History: prenatal/birth history, vision/hearing, menigitis, seizure, communication problems, difficult movement. Physical exam: neuro exam, Asymmetric or abnormal deep tendon reflexes and movement Ankle clonus, no fasciculations , affect visual, development, feeding Persistent primitive reflexes (e.g., tonic neck and Moro after 6 months of age) • Delayed reflexes (e.g., parachute reflex remains absent after 9 to 10 months of age; side-protective reflexes remain absent after 5 months of age) • Preferred handedness before 1 to 2 years of age • Abnormalities of head size such as macrocephaly or microcephaly Test: MRI, LP if sepsis suspected, Chromosomal and metabolic studies REFERRAL, family education Hypotonic Infant Hypotonia may also be seen in the child with seizures, abnormal reaction to painful stimuli, muscle wasting, abnormal reflexes, tongue fasciculation, and asymmetric movement of extremities hypotonia can result from ischemic or hemorrhagic brain insults, congenital brain malformations, genetic syndromes, metabolic disease, prematurity, or for idiopathic reasons MRI; sometimes muscle biopsies or various neurophysiologic studies are used. Injuries and Congenital Conditions Brachial Palsy brachial plexus in neonates can occur during a difficult vaginal delivery; such injury has also been reported following cesarean births Neonatal risk factors for plexus injuries include high birth weight, macrosomia, prolonged labor, breech delivery, maternal gestational diabetes, and instrumented delivery.Brachial plexus injuries can also occur in children restrained with a seatbelt during an automobile accident. Physical Examination Soon after birth, the infant is found to have asymmetric active range of motion of the arms, specifically lack of spontaneous upper extremity movement. Radiographs can be considered to the evaluate humerus and clavicular fractures, if suspected. CT or MRI can be used to help characterize injury patterns; MRI is currently preferred because it eliminates radiation exposure Treatment: Gentle range-of-motion, physical therapy , surgery Prognosis The majority of brachial plexus palsies spontaneously resolve over several weeks to several months. A quarter to a third of infants with brachial palsy will have residual neurologic deficits, so immediate intervention and assessment is vital. Bell palsy Bell Palsy is an acute unilateral paralysis or weakening of the facial nerve that may include individual or all branches of the facial nerve. 15-40 years, rapid onset. localized pain or tingling in one ear and then experience sagging of the facial features corresponding to the affected branches of the facial nerve on the affected. viral illness or symptomatology within the two weeks preceding onset. Physical Examination Unilateral motor changes in the forehead, cheek, and perioral area; face muscles Dribbling liquids from the weak sid Lacrimation, taste (half of patients); anterior two-thirds of tongue and salivation may be impaired • No limb weakness • Any skin lesions to suggest herpes on the affected side of the face (would indicate active viral infection of the nerve or its motor neurons) • Otoscopic examination to evaluate for symptoms of infection methylcellulose eye drops or ocular lubricant to the affected eye several times daily and patch the eye Tethered Cord a tethered cord, however, the caudal end is fixed by a ropelike filum terminale at or below the L2 level. This can cause abnormal stretching and damage to nerve cells, fibers, and blood vessel Tethered cord often associated with a congenital spinal anomaly, such as spina bifida (90%), hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence. Test : mri, referral pediatric neurosurgeon Arnold-Chiari Malformation Arnold-Chiari malformations consist of two types of uncommon congenital spinal cord anomalies whose sequelae are usually not evident until late childhood or into adulthood Type I malformation involves the downward elongation, Type II malformation is present in 0.5 to 1 per 1000 of children with spina bifida myelomeningocele. Type I malformation can cause headache, neck pain, atrophy and decreased reflexes in the lower extremities, sensory losses, and scoliosis type II may include hydrocephaly, respiratory distress, syncope, poor feeding, vomiting, dysphagia, tongue paralysis, and cardiopulmonary failure. Test: MRI Due to the risk of brainstem herniation, an LP should never be attempted in a child with an Arnold-Chiari malformation Myelomeningocele the protrusion of both the spinal cord nerve roots (myelo) and the three layers of membranes (meninges) that cover the spinal cord and brain through this spinal defect. spina bifida cystica is often used interchangeably with myelomeningocele. When the vertebral arches fail to close but there is no subsequent herniation of cord or meninges, the term spina bifida occulta is used. take of certain drugs and toxins is associated with neural tube defects: folic acid antagonists (trimethoprim, carbamazepine, phenytoin, phenobarbital, and primidone), retinoic acid derivatives Clinical Findings: poor folic acid, Flaccid paralysis of lower extremities, Absence of deep tendon reflexes, Lack of response to touch and pain in lower extremities • Incontinence issues including constant urinary dribbling, encopresis Management: surgery within a week after birth, shunt Prognosis With aggressive early treatment, survival rates can be quite high, and children can be maintained with current interventions; deaths more commonly occur before 4 years of age. Prevention: folic acid, prenatal vitamin Central Nervous System Infections over 1 to 24 hours) or chronically (over 1 to 7 days or more). Bacteria, viruses, fungi, spirochetes, protozoa, and parasites can all cause CNS infection Clinical finding: Upper respiratory tract or gastrointestinal symptoms accompanied by fever, Increasing lethargy and irritability or behavioral change, Recent head injury or neurosurgical procedure, Immunocompromised host,History of travel, sick contact, insect bites, animal contacts Physical exam Systemic signs, cns sign, kernig or Brudzinski positive, bulging fontanelle, increase head circumference, papilledema Diagnostic: Blood culure, cbc, urinalysis, LP, CSF culture (gold standard, CT, MRI Send to ER Zika Virus mosquito-borne flavivirus to human, cause fetal malformation. NO treatment Reye Syndrome Encephalopathy process often associated with a viral infection primarily affecting those under 18 years of age. ue to the association between salicylates and the development of Reye syndrome, it is recommended that children under age 18 avoid salicylates. Severe vomiting progresses to irrational behavior; stupor and coma; apnea, fixed pupils, and decorticate posturing with increasing brain edema; and finally, death. Supportive treatment Myasthenia Gravis MG is an autoimmune disorder that produces an immune-mediated neuromuscular blockade or neuromuscular junction disorder. Mother can transfer MG to infant. Once the infant’s own receptors regenerate and reinsert into synaptic membranes, the symptoms resolve Symptoms Ptosis and some degree of extraocular muscle weakness, dysphagia, muscular weakness, fatigue Test: EMG, achR antibody test, CK, XRAY, CT scan, muscle biopsy, ecg Treatment 1 line anticholinesterase therapy (pyridostigmine), 2 line Corticosteroids or immunosuppressant Neurofibromatosis NF1 disorder, genetic mutation in the NF1 tumor suppressor gene. Symptom: skeletal deformties, pigmentary abnormalities, breast cancers, leukemia and lymphoma, pheochromocytoma, rhabdomyosarcoma, and GI tumors. Test: MRI, complete physical (neurologic system), baseline cardiology, eye exam Treatment: referral to multidisciplinary specialists Guillain-Barré Syndrome acute polyradiculoneuropathy that primarily affects peripheral nerves and is characterized by progressive weakness and diminished or absent reflexes History: fever, viral infection, weaknes Exam: pain in muscle palpation, irritability, respiratory insufficiency, dysphia, urinary incontinent, cardiac changes Test: CSF, CK, EMG Treanment : IVIG for 5 days is standard protocol, may need second dose, lasmapheresis and/or immunosuppressive drugs may be used in cases unresponsive to IVIG;. Chronic pain management Neurodegenerative Disorders Neurodegenerative disorders occur when the gray or white matter of the brain is affected. These insults can affect the basal ganglia, cerebellum, brainstem, spinal cord, peripheral and CNs, or cerebrum Diagnose: onset, genetic, symptoms Referral neurology pediatric Rett Syndrome Genetic neurodevelopmental disorder characterized by developmental arrest and regression and multisystem comorbidities. Symptoms: partial/loss language, bruxism, apraxia, gait abnormalities, and stereotypic hand movements, decrease head grow, respiratory dysregulation, gastrointestinal dysfunction, scoliosis, sleep disturbance, and less commonly prolonged QTc interval. Neurologic comorbidities may include seizures and spasticity (late development) Test: EEG Physical, occupational, and speech therapies and seizure management. Multiple Sclerosis MS is a chronic, relapsing disorder of the CNS that involves demyelination of the brain, spinal cord, and optic nerves, rare in children >10 Symptoms: Unilateral weakness, ataxia or other cerebellar symptoms more than 24 hr, headache, motor symptom, visual disturbance, vertigo, dysarthria. Test: MRI, LP