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Nervous System Anatomy, Assessment, and Conditions

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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
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