ACUTE BACTERIAL MENINGITIS CONTRAINIDCATION IN DIAGNOSIS

MC Cause:  Evidence of ↑ ICP (bulging fontanel/CN 3 or CN 6

 Streptococcus pneumoniea nerve palsy/depressed consciousness)
 Neisseria meningitides  Severe cardiopulmonary compromised
 Infection of skin overlying lumbar puncture site
MAJOR RISK FACTOR:  RELATIVE Contraindication:
Lack of immunity to specific pathogens THROMBOCYTOPENIA
associated with young age Medications:
 IV Furosemide - ↓ brains welling by venodilation and
Additional risks: diuresis without ↑ ICP
 Recent colonization with pathogenic bacteria  Mannitol – shifts fluid from CNS to plasma thru
 Close contact osmolar gradient
 Poverty  For seizures:
 Male gender o Immediate: IV DIAZEPAM & Lorazepam
o After immediate: PHENYTOIN
RISK FACTOR FOR EACH ORGANISM
MENINGOCOCCAL H. L. SUBDURAL EFFUSION
INFLUENZA MONOCYTOGENES  Bulging fontanel
TYPE B  Diastasis of sutures
- Complement - Splenic - T. lymphocyte  Enlarging head circumference
system (C5-C8) Dysfunction defects  Emesis
Defect - Asplenia
 Seizures
- Properdin System
Defect  Fever
PNEUMOCOCCAL (Streptococcus) STAPHYLOCOCCAL  Highest mortality with PNEUMOCOCCAL
- Splenic Dysfuction - Lumbosacral dermal MENINGITIS
- Asplenia sinus and  Poor prognosis: ,6 mo old, high concentration of
- Leak across mucocutaneous barrier meningomyelocele bacteria in CSF, seizures >4 days
- Otitis media, Sinusitis, Otorrhea - CSF Shunt infection Chemoprophylaxis
Neisseria Rifampicin, Quadrivalent (A,
Neisseria Meningitides meningitides 10mg/kg q 12 hr for C, Y, W-135)
 A, B, C, Y, W-135 2 days Conjugated
 2 peaks of incidence: <5 years olf and 15-21 y/o (MCV-4)
Streptococcus Heptavalent
H. influenza Type B pneumoniae
H. influenzae Rifampicin, 20
 2 months to 2 years of age
Type B mg/kg/day for 4
days
Clinical Manifestations
INFANTS VIRAL MENINGOENCEPHALITIS
 Fever 50% MC cause: ENTEROVIRUSES
 Seizures 40% Enterovirus 68 – flaccid paralysis Parechovirus – aseptic
 Poor Feeding Arbovirus – arthropod, mosquitos, ticks
 Abdominal Distention HSV Type 1 – severe, SPORADIC HSV Type 2 - mild
 Respiratory Distress Varicella-zoster – cerebellar ataxia, can become latent
 Bulging Anterior Fontanelle 30% MUMPS – can damage CN8
 Altered Sensorium S/Sx: headache, hyperesthesia
OLDER CHILDREN DX: CSF – mild mononuclear predominance
 TRIAD: Fever, Headace, Nuchal Rigidity EEG – diffuse slow-wave w/o focal change
Neuroimaging – swelling of brain parenchyma
Treatment : Acyclovir for HSV; In general, it is supportive
 SEIZURES IN CHILDHOOD
EOSINOPHILIC MENINGITIS Seizure : sudden, involuntary, time-limited alteration in
 > 10 eosinophils/mm3 of CSF behaviour
 MC Cause: Helminthic parasites Epilepsy: episodic, paroxysmal condition in w/c an
S/Sx:
individual is predisposed to RECURRENT seizure
 Fever
 Peripheral eosinophilia Initial evaluation of the FIRST SEIZURES:
 Vomiting  adequacy of the airway, ventilation and cardiac
 Abdominal pain function
 Creeping skin eruptions  temperature, blood pressure, glucose measurement
Treatment: Supportive  Acute evaluation includes search for potentially life
threatening causes
Focal Seizures Motor OR Sensory symptoms
BRAIN ABSCESS Tonic Seizures ↑ tone or rigidity
Cause: Embolization due to Congenital Right-to-Left Shunt ATONIC Seizures Flaccidity or lack of movements during
(TOF); meningitis, otitis, etc convulsion
Frontal lobe- sinusitis or orbital cellulitis CLONIC RHYTHMIC muscle contraction and
Temporal lobe or cerebellum – chronic otitis media and relaxation
mastoiditis MYOCLONUS SHOCKLIKE
Abscess due to penetrating Injury – S. aureus
FEBRILE SEIZURES
Diagnosis:
 Occurs btw 6 and 60 months with temp of 38ºC
CT: parenchymal low density; RING-enhancing lesion  Not due to an infection, electrolyte imbalance, no hx
MRI: ↑ signal intensity of afebrile seizures
SIMPLE ATYPICAL
 Generalized tonic-  Focal seizures are
clonic present during post
 Few seconds to10 ictal
min  >15 minutes
 Followed by brief  Repeated
period of post ictal convulsions occur
drowsiness within same day

 Duration of antibiotic is 4-6 weeks

Surgery is indicated when:
 Abscess >2.5 cm
 gas is present
 multiloculated
 located in posterior fossa

TRANSVERSE MYELITIS
 both motor and sensory deficit
 Spinal cord inflammation by MRI
 CSF Pleocytosis (>10 cells)
 ↑ IgG index
 No standard treatment
 Initial approach can be Methylprednisone
 GENERALIZED SEIZURES

ABSENCE SEIZURES (TYPICAL)

 > 5 y/o
 SUDDEN Cessation of motor activity or speech with
BLANK FACIAL EXPRESSION and FLCIKERING OF
EYELIDS
 HEAD may fall forward
 Provoked by Hyperventilation
 < 30 seconds

JUVENILE ABSENCE SEIZURES

 Similar to typical but occurs at later age

GENERALIZED SEIZURES
GENERALIZED EPILEPSY WITH FEBRILE SEIZURE PLUS
(GEFS+) TONIC – starts with loss of consciousness and with sudden
- Autosomal Dominant cry, upward rolling of eyes
- onset in early childhood; remission in mid-childhood CLONIC – slowing of rhythmic contractions
- multiple febrile with subsequent afebrile
FIRST AID MEASURES
THE FOLLOWING ARE NOT ROUTINELY RECOMMENDED  Position px on his/her side
for FIRST FEBRILE SEIZURE  Clear mouth f it is open
 EEG  Extend the head
 Blood Studies  Mouth should not be forced open with a foreign object
 Neuroimaging or with fingers
BENIGN GENERALIZED SEIZURES
FOCAL EPILEPSIES
 JUVENILE MYOCLONIC EPILEPSY (JANZ
 FOCAL SEIZURES WITHOUT IMPAIRMENT OF
SYNDROME)
CONSCIOUSNESS
o MC generalized epilepsy in young adults
 FOCAL SEIZURES WITH IMPAIRMENT – last 1-2
o Starts in early adolescent with
min preceded by aura or automatism
 Myoclonic jerks in morning causing
BENIGN EPILEPSY WITH px to drop things
FOCAL SEIZURES  Generalized tonic clonic upon
awakening
 BENIGN CHILDHOOD EPILEPSY WITH  Sleep deprivation/alcohol can act
CENTROTEMPORAL SPIKES as precipitant
o 3-10 y/o; OUTGROWN IN ADOLESCENCE  PHOTOPAROXYSMAL EPILEPSY
o Wakes up at night due to buccal and throat o Precipitated by strobe lights or flipping thru
tingling and tonic-clonic contractions of 1 channels
side of face with drooling
o DOC: CARBAMAZEPINE SEVERE GENERALIZED SEIZURES
 NOCTURNAL AUTOSOMAL DOMINANT FRONTAL
 EARLY MYOCLONIC INFANTILE
LOBE EPILEPSY
ENCEPHALOPATHY
SEVERE EPILEPSY WITH o starts during 1st 2 months of life
FOCAL SEIZURES o BURST SUPPRESSION Pattern
o St inborn errors of metabolism
 MESIAL TEMPORAL SCLEROSIS  OHTAHARA SYNDROME
o Atrophy and gliosis of hippocampus o dt syntaxin binding protein1 mutation
o MC Cause of surgically remediable partial  WEST SYNDROME
epilepsy in adults o 2 to 12 mo old
 RASMUSSEN ENCEPHALITIS o TRIAD:
o Unilateral intractable partial seizure  Infantile epileptic spasm
 Developmental regression
 HYPSARRHYTHMIA
  LENNOX-GASTAUT SYNDROME
MIMICS OF SEIZURE
o Btw ages 2 and 10 years
o TRIAD BENI33GN PAROXYSMAL VERTIGO
 Developmental delay  Fall or refuse to walk or sit
 Multiple seizure types  Child appears frightened and pale with N/V
 Atypical absences NIGHT TERRORS
o Some start with Ohtahara then develop West  Common in boys
and progress to Lennox  Child screams and appears frightened, dilated pupils,
o Seizures are difficult to control tachycardia and hyperventilation
 MYOCLONIC ASTATIC EPILEPSY  Happens between midnight and 2:00 am
o Milder than Lennox BREATH HOLDING SPELLS
 PYRIDOXINE-DEPENDENT EPILEPSY  CYANOTIC SPELLS
o With GIT symptoms o Provoked by upsetting or scolding an infant
o ↑ plasma, urine and CSF a-aminoadipic o Brief shrill cry followed by forced expiration
semialdehyde and ↑ plasma and CSF and apnea
pipecolic acid o Tx: parent counselling
o TX: Pyridoxine 100 mg DAILY  PALLID SPELLS
o initiated by painful experience
TREATMENT OF SEIZURES: o child stops breathing; loss of consciousness
 Most serious adverse anticonvulsant drug reactions SYNCOPE
occur during initial 2-3 months of therapy, monthly  SIMPLE SYNCOPE
blood screening for the 1st 3 months is recommended o Due to decreased blood flow resulting from
pain, fear, excitement prolonged standing
NEONATAL SEIZURES particularly in a warm environment
o Tx: oral b adrenergic
 FIFTH-DAY FITS  COUGH SYNCOPE
o 5th day of life
o Normal appearing neonate PROLONGED QT SYNDROME
o Present for <24 hours  Sudden loss of consciousness during exercise or
o Good prognosis emotional and stressful experience
 Tx: B adrenergic antagonist
 PYRIDOXINE DEFICIENCY
o LIFELONG PAROXYSMAL KINESIGENIC CHOEOATHETOSIS
o Inherited as autosomal recessive  sudden unilateral or bilateral choreoathetosis
 Tx: Phenytoin
STATUS EPILEPTICUS Shuddering attacks
- continuous convulsion lasting longer than 30 minutes  Onset at 4-6 months of age
 Sudden flexion of head and trunk and shuddering or
shivering movements
BENIGN Paroxysmal Torticollis
 Recurrent attacks of head tilt with pallor, agitation and
vomiting
NARCOLEPSY & CATAPLEXY
Narcolepsy – begins before adolescence; attacks of
irrepressible daytime sleep with transient loss of muscle tone
Tx: Modafinil
Pointers: Cataplexy – sudden loss of muscle tone and fall to the floor
 Hypertension not treated until status is controlled due to laughter, stress or frightening experience
 1st line drugs
o BZD MASTURBATION
o DIAZEPAM  Girls between 2 mos and 3 years
 2nd line drugs  Repetitive stereotyped episodes of tonic posturing
o Phenytoin with copulatory movements
o Thiopental PSEUDOSEIZURES
 btw 10-18 y/o; lack of cyanosis, normal reaction of
pupils
 INFREQUENT TENSION HEADACHE
HEADACHE

PAIN-SENSITIVE Structures in the Head

FREQUENT TENSION HEADACHE

CHRONIC TENSION HEADACHE

HEADACHE Suggesting RAISED ICP
 early morning headache
 vomiting in morning
 pain disturbing sleep
 headache worse with cough or valsalva

ACUTE HEADACHE – less than 48 hours duration; sudden

onset in a previously well child

MIGRAINE WITHOUT AURA

CONGENITAL MALFORMATIONS

SPINA BIFIDA OCCULTA

- MIDLINE defect of vertebral bodies without protrusion
of spinal cord or meninges

MIGRAINE WITH AURA

MENINGOCOELE
- Meninges herniate through a defect in the posterior
vertebral arches or the anterior sacrum
 MYELOMENINGOCOELE
HEMATOMA
- Most severe form of dysraphism
- Aperta or open form involving vertebral column and EPIDURAL HEMATOMA SUBDURAL HEMATOMA
spinal cord  Middle Meningeal  Bridging veins
- Maternal periconceptional use of FOLIC ACID Artery
supplementation reduces incidence of NTDs
- MC in lumbosacral region Clinical Manifestations
Severe TBI – GCS 3-8
Moderate TBI – GCS 9-13
Mild TBI – GCS 14-15

HERNIATION SYNDROME &

INTRACRANIAL PRESSURE

HYDROCEPHALUS
PHYSIOLOGY: estimated 5th month AOG BRAIN DEATH
- Formed primarily in the ventricular system: lateral;  Irreversible coma with a known cause
third and fourth ventricles  Absence of brainstem reflexes
- Total volume of CSF: 50 mL in an infant and 150 mL  Apnea
in adult TUMORS
S/SX in INFANT
 Accelerated rate of head enlargement
 Anterior fontanel is wide open and bulging; scalp
veins are dilated
 Forehead is broad
 Eyes deviate downward because of impingement of
dilated suprapineal recess
TX: Acetazolamide and Furosemide

CRANIOCEREBRAL INJURIES

Etiology
 Motor vehicle crashes TUMORS
 Falls
 Assaults
 Abusive head trauma

Primary Head Injury

- Impact produces irreversible tissue disruption at time
of impact
Secondary Head Injury
- Ultimate damage seen in the injured brain evolves
over hours or days and underlying mechanisms
involved (edema, apoptosis and secondary axotomy)
are therapeutic targets
SPINAL MUSCULAR ATROPHY CSF: ALBUMINO-CYTOLOGICAL DISSOCIATION
 Lack of SNA1 gene DX: EMG & NCV
 Autosomal Recessive TX: Plasma Exchange: 200-250 mL/kg
 No Treatment IVIG: 0.4g/kg/day, transfused for 2 hours per day for 5
 DIAGNOSIS: EMG-NCV and Muscle Biopsy days

AUTOIMMUNE MYESTHENIA GRAVIS

 Chronic autoimmune disease of neuromuscular
blockade
 Rapid fatigability of striated muscle
 Patient fails to replenish acetylcholine
DX: Rapid Nerve Stimulation (RNS)
Anti-AchR antibodies
TX: Cholinesterase-inhibiting drugs (Neostigmine
methylsulfate

NEUFIBROMATOSIS 1
MUSCULAR DYSTROPHY
 >6 café-au-lait macules
 Axillary or inguinal freckling
 >2 iris Lisch nodules
 >2 neurofibromas
 Osseous lesion
 Optic gliomas
NEUROFIBROMATOSIS 2
 Vestibular schwannoma
DUCHENNE BECKER  Family history
 early childhood  After 8 y/o  Meningioma
 early inability to walk  Ability to walk
 (++) retained beyond 16 TUBEROUS SCLEROSIS
pseudohypertrophy y/o  Autosomal dominant
of muscles  (+)  Hallmark is CNS involvement
 More MR, cardiac pseudohypertrophy  Retinal lesions: hamartomas (mulberry lesions) or
involvement of muscles white depigmented patches
 Less MR and cardiac  Characteristic brain lesion is cortical tuber
invovement

GUILLIAN BARRE SYNDROME

 In 80% of cases; an acute infection is reported 2 to 6
weeks before neurologic symptoms
 Camplylobacter: most common associated
bacterium
 ↑ antiganglioside antibodies & activated T-cells

STURGE WEBER
 Port-wine stain
 Leptomeningeal angioma (abnormal blood vessels of
brain)
 Abnormal blood vessels of eye leading to glaucoma
DX:
VON-HIPPELLINDAU DISEASE
 AD mutation affecting tumor suppressor gene, VHL
 Cerebellar hemangioblastoma and retinal angiomas
PHACE SYNDROME