Neurology

Question 1

Characterized by ipsilateral ptosis (droopy eyelid), miosis (constricted pupil), and anhidrosis (lack of sweating) of the face

Answer 1

Horner Syndrome

Question 2

Characterized by an initial resistance to passive movement, followed by a sudden release, referred to as the clasp-knife phenomenon

Answer 2

spasticity

• Abnormalities of tone include spasticity, rigidity, and hypotonia
• results from upper motor neuron dysfunction
• tends to occur in conjunction with disuse atrophy

Question 3

Seen with lesions of the basal ganglia, characterized by resistance to passive movement that is equal in the flexors and extensors regardless of the velocity of movement (lead pipe)

Answer 3

Rigidity

Question 4

abnormally diminished tone and is the most common abnormality of tone in neurologically compromised neonates

Answer 4

hypotonia

Question 5

At what age is clonus not a pathologic sign in a pediatric patient?

Answer 5

in patients <3 mos old, 5-10 beats of clonus may be observed, symmetrically

• sustained clonus is always pathologic

Question 6

Primitive reflex elicited by supporting the infant in a semierect position and then allowing his or her head to fall backwards onto the examiner’s hand

Answer 6

Moro reflex

• normal response consists of symmetric extension and abduction of the fingers and upper extremities, followed by flexion of the upper extremities and an audible cry.
• onset: 28-32 wks
• fully developed: 37 wks
• duration: 5-6 mos
• Absence: suggests significant dysfunction of the CNS
• asymmetry: fractured clavicle, brachial plexus injury or hemiparesis

Question 7

Primitive response elicited by placing a finger in the open palm of each hand; by 37wk of gestation, the reflex is strong enough that the examiner can lift the infant from the bed with gentle traction

Answer 7

grasp response

onset: 28 wks
fully developed: 32 wks
duration: 2-3 mos

Question 8

A primitive reflex produced by manually rotating the infant’s head to one side and observing for the characteristic fencing posture (extension of the arm on the side to which the face is rotated and flexion of the contralateral arm)

Answer 8

tonic neck reflex

• abnormal when infant gets stuck in this posture, implies CNS disorder
• onset: 35 wks
• fully developed: 1 mo
• duration: 6-7 mos

Question 9

Primitive reflex that occurs in slightly older infants, can be evoked by holding the infant’s trunk and then suddenly lowering the infant as if he or she were falling. The arms will spontaneously extend to break the infant’s fall, making this reflex a prerequisite to walking.

Answer 9

parachute reflex

• onset: 7-8 mos
• fully developed: 10-11 mos
• duration: remains throughout life

Question 10

When does the rooting reflex develop?

Answer 10

onset: 32 wks
fully developed: 36 wks
duration: less prominent after 1 mo

Question 11

What are the contraindications to performing a lumbar puncture?

Answer 11

suspected mass lesion of the brain/spinal cord
symptoms and signs of impending cerebral herniation in a child with probable meningitis; critical illness (on rare occasions); skin infection at the site of the lumbar puncture; and thrombocytopenia with a platelet count <20 ? 109/L.

Question 12

The most common form of migraine in both children and adults

Answer 12

migraine without aura

• recurrent (at least 5 attacks)
• lasts 4-72 hrs
• 2 of the ff: unilateral, pulsating, mod-sev pain, aggravated by or cause avoidance of physical activity
• during headache: nausea/vomiting or photophobia/phonophobia
• in children, headaches may be shorter and bilateral

Question 13

How can one differentiate migraine headache from secondary headache related to increased ICP?

Answer 13

headaches related to increase in ICP tend to be increasing in intensity, usu in early morning upon awakening, occuring almost daily, remits with upright posture

migraine headaches are episodic, not worsening, but when it presents upon awakening, getting up and doing upright activities will make headache and vomiting worse.

Question 14

What are the indications for neuroimaging in a child with headaches?

Answer 14

Abnormal neuro exam
Focal neurologic signs
Seizures or very brief auras (<6yr old or any child that cannot adequately describe their headache
Brief cough headache in a child or adolescent
Headache worst on first awakening or that awakens the child from sleep
Migrainous headache in the child with no family history of migraine or its equivalent

Question 15

What is the imaging of choice for a child with headache, if indicated?

Answer 15

an MRI is the imaging of choice as it provides the highest sensitivity of detecting posterior fossa lesions and does not expose the child to radiation

Question 16

Two groups of medications for acute migraine attacks

Answer 16

NSAIDs and triptans

• goal of the primary acute medication should be headache relief within 1hr with return to function in 10 out of 10 headaches
• use NSAIDs first, for only 2-3x/wk then add triptans (almotriptan) for mod-sev attacks

Question 17

Which antiemetic drugs are very helpful adjuncts in relieving acute migraine headaches because of their mechanism of action (dopamine antagonist) and can be added to the regimen if unresponsive to NSAIDs and triptans?

Answer 17

metoclopramide and prochlorperazine

Question 18

Preventive therapy for migraine may be done if headaches frequent and disabling. What is the most commonly used preventive therapy for headache and migraine?

Answer 18

amitriptyline

• antiepileptic drugs have also been used: topiramate, valproic acid, levetiracetam

Question 19

seizures that occur between the age of 6 and 60mo with a temperature of 38C or higher, that are not the result of CNS infection or any metabolic imbalance, and that occur in the absence of a history of prior afebrile seizures

Answer 19

febrile seizures

• patients with febrile seizures do not have any increase in incidence of abnormalities of behavior, scholastic performance, neurocognitive function, or attention

Question 20

Differentiate a simple febrile seizure from complex febrile seizure

Answer 20

simple febrile seizure: generalized, usually tonic-clonic, attack associated with fever, lasting for a maximum of 15min, and NOT recurrent within a 24-hour period
complex febrile seizure: more prolonged (>15min), is focal, and/or recurs within 24hr

Question 21

What are the major risk factors in recurrence of febrile seizures?

Answer 21

Age < 1 yr
Duration of fever < 24 hr
Fever 38-39C

Minor risk factors:
       Family history of febrile seizures
		Family history of epilepsy
		Complex febrile seizure
		Day care
		Male gender
		Lower serum sodium

Having no risk factors carries a recurrence risk of about 12%; 1 risk factor, 25-50%; 2 risk factors, 50-59%; 3 or more, 73-100%

Question 22

When is lumbar puncture indicated in a patient with febrile seizure?

Answer 22

Recommended in children below 18 mos because the clinical signs of meningitis may be subtle in this age group

• EEG would not predict the future recurrence of febrile seizures or epilepsy even if the result is abnormal - not routinely done
• blood studies not routinely recommended
• CT or MRI not recommended after first simple febrile seizure

Question 23

When are apneic episodes in neonates associated with seizures?

Answer 23

When the apnea is accompanied by tachycardia (not bradycardia)

Question 24

Prolonged expiratory apnea is responsible for the cyanotic episodes, which result from intrapulmonary shunting. An episode starts with a cry and marked pallor, progresses to apnea and cyanosis. Spells usually begin between 6 and 18mo of age. Syncope, tonic posturing, and even reflex anoxic seizures may follow significant episodes

Answer 24

pallid breath-holding spell

• Often pallid breath-holding spells are made worse by iron deficiency anemia

Question 25

One of the most common mimickers of generalized tonic clonic seizures and is usually triggered by dehydration, heat, standing for a long time without movement, hot showers, the sight of blood, pain, or sudden stress

Answer 25

Vasovagal (neurocardiogenic) syncope

Question 26

How can seizures be differentiated from jitteriness?

Answer 26

Jitteriness can be defined as rapid motor activities, such as a tremor or shake, that can be ended by flexion or holding the limb

Seizures do not end with tactile or motor suppression

Question 27

Starts between the ages of 2 and 12mo and consists of a triad of infantile spasms that usually occur in clusters (particularly in drowsiness or upon arousal), developmental regression, and a typical EEG picture called hypsarrhythmia

Answer 27

West Syndrome

• Many patients start with Ohtahara syndrome, develop West syndrome, and then progress to Lennox-Gastaut syndrome.

Question 28

usually last 1-2min and are often preceded by an aura, such as a rising abdominal feeling, d?j? vu or d?j? v?cu, a sense of fear, complex visual hallucinations, micropsia or macropsia (temporal lobe), generalized difficult-to-characterize sensations (frontal lobe), focal sensations (parietal lobe), or simple visual experiences (occipital lobe)

Answer 28

Complex partial seizures

• Subsequent manifestations consist of decreased responsiveness, staring, looking around seemingly purposelessly, and automatisms.

Question 29

Cafe-au-lait spots are the hallmark of which disease and is present in 100% of patients?

Answer 29

Neurofibromatosis

2 types: NF-1 and NF-2

• NF-1 is more common (also known as von Recklinghausen disease)
• Lisch nodules (hamartomas in iris), optic gliomas, neurofibromas (peripheral tumors)
• NF-2 is rare, manifests primarily as congenital bilateral acoustic neuromas (vestibular schwannomas), cafe-au-lait spots less common
• hearing loss, unsteadiness, and sometimes headache or facial weakness

Question 30

One of the most common complications of NF-1 (neurofibromatosis-1) affecting approximately 30% of children

Answer 30

learning disabilities

Question 31

What is the hallmark of tuberous sclerosis?

Answer 31

CNS involvement

• characteristic brain lesion is a cortical tuber
• Brain MRI is the best way of identifying cortical tubers
• most common neurologic manifestations of TSC: epilepsy, cognitive impairment, and autism spectrum disorders

Question 32

What are the classic dermatological, cardiac, ophthalmologic and pulmonary manifestations of tuberous sclerosis?

Answer 32

derma: hypomelanotic macules, facial angiofibromas, Shagreen patch
cardiac: cardiac rhabdomyoma
ophtha: retinal hamartomas and white depigmented patches
pulmonary: Lymphangioleiomyomatosis (LAM) - classical pulmonary lesion in TSC and only affects women after the age of 20yr

Question 33

Sporadic vascular disorder and consists of a constellation of symptoms and signs including a facial capillary malformation (port-wine stain), abnormal blood vessels of the brain (leptomeningeal angioma), and abnormal blood vessels of the eye leading to glaucoma

Answer 33

Sturge-Weber Syndrome

• incidence of epilepsy in patients with SWS is 75-90%, and seizures develop in most patients in the 1st yr of life
• MRI with contrast is the imaging modality of choice for demonstrating the leptomeningeal angioma in SWS

Question 34

A clinical syndrome that mimics brain tumors and is characterized by increased intracranial pressure (ICP; >200 mm H2O in infants and >250mm H2O in children), with a normal cerebrospinal fluid (CSF) cell count and protein content and normal ventricular size, anatomy, and position documented by MRI.

Answer 34

pseudotumor cerebri

• most frequent symptom is headache; vomiting is rarely as persistent and insidious as that associated with a posterior fossa tumor
• Most patients are alert and lack constitutional symptoms
• (+) MacEwen sign (percussion of the skull produces a resonant sound) due to separation of the cranial sutures
• Papilledema with an enlarged blind spot is the most consistent sign in a child beyond infancy.

Question 35

Inability to make smooth, accurate, and coordinated movements, usually due to a dysfunction of the cerebellum, its inputs or outputs, sensory pathways in the posterior columns of the spinal cord, or a combination of these

Answer 35

ataxia

Question 36

An autosomal recessive condition, is the most common of the degenerative ataxias and is heralded by ataxia beginning at about age 2yr and progressing to loss of ambulation by adolescence

Answer 36

ataxia-telangiectasia

Question 37

Movement disorder characterized as habitual, stereotypic movement, vocalization, or behavior that can be temporarily suppressed by the patient

Answer 37

Tic

Question 38

Differentiate dystonia from chorea

Answer 38

Chorea - Fluid, continuous; movements migrate rapidly from one part of the body to another

Dystonia - torsional, sustained muscle contraction, frequently causing twisting and repetitive movements or abnormal postures

Question 39

How is Tourette Syndrome diagnosed?

Answer 39

(TS) is diagnosed by the presence of multiple motor tics and at least one vocal tic, persisting more or less uninterrupted for 1 year

• a history of discrete, stereotypic movements or vocalizations that can be inhibited by the patient and of disappearance of the movements during sleep supports the diagnosis of tic/TS.

Question 40

The parents of a 7-year-old boy bring him for evaluation because he has developed a habit of blinking his eyes. This habit has been noted for 3 weeks. Sometimes, he will blink incessantly and, at other times, hardly at all. His habit can be suppressed for up to a minute but not longer. He remains alert and acts normally during the blinking episodes. He does not blink when he is asleep. Other children do not notice his habit of blinking, but his father is very worried and requests that the doctor order an MRI scan to make certain that nothing is wrong with his son’s brain.
No other unusual behaviors are reported. However, in retrospect, the parents note that 6 months ago, the child developed a habit of clearing his throat that was attributed to allergy but persisted longer than expected. The child has no history of other significant medical problems. He had one episode of streptococcal pharyngitis 1 year ago that was treated with amoxicillin.
The child is a good student and gets along well with other children but is described as “a worrier.” His maternal aunt was treated for obsessive-compulsive disorder.
Physical examination reveals a high-strung, alert boy in good general health. There are no signs of infection or inflammation in the pharynx, and no adenopathy is noted. The neurological examination reveals no focal deficits. The child frequently and forcefully blinks his eyes and also periodically stretches his neck.

What is your diagnosis?

Answer 40

Tic disorder (Tourette Syndrome)

• Some patients with tics also have a learning disability (LD) that is suggested by a history of poor grades and further suggested by a learning evaluation.
• Tics do not normally result from a brain tumor, any other intracranial lesion, or a degenerative disorder and are usually readily differentiated on clinical grounds from other abnormal movements

Question 41

What is the most common acquired chorea of childhood, which occurs in 10% to 20% of patients with acute rheumatic fever, typically weeks to months after a group A β-hemolytic streptococcal infection, with peak incidence at age 8 to 9yr, and a female predominance of 2 : 1?

Answer 41

Sydenham chorea

• clinical hallmarks of SC are chorea, hypotonia, and emotional lability

Question 42

Very rapid (“lightning-fast”) involuntary movement, most often of the upper extremities, occuring mostly during sleep, cannot be voluntarily suppressed like tics.

Answer 42

Sleep myoclonus

-

Question 43

Severe rigidity combined with high fever, autonomic symptoms (tachycardia, diaphoresis), delirium, and dystonia

Answer 43

neuroleptic malignant syndrome

Question 44

Delayed onset involuntary movements that develop in the setting of chronic neuroleptic use, at least 3mo in duration

Answer 44

tardive dyskinesias

Question 45

In acute drug-induced dystonic movement disorder, what is the drug of choice to rapidly reverse the symptoms, especially the life-threatening ones presenting as laryngospasm and airway compromise?

Answer 45

diphenhydramine (1-2 mg/kg/dose)

Question 46

Severe rigidity combined with high fever, autonomic symptoms (tachycardia, diaphoresis), delirium, and dystonia which typically occurs a few days after starting or increasing the dose of a neuroleptic drug, or in the setting of withdrawal from a dopaminergic agent.

Answer 46

neuroleptic malignant syndrome (NMS)

• occurs within a month of medication initiation or dose increase, in contrast to acute dystonic rxns which take place within days
• drug of choice: dantrolene

Question 47

Autosomal recessive inborn error of copper transport characterized by cirrhosis of the liver and degenerative changes in the CNS, particularly the basal ganglia

Answer 47

Wilson Disease

• copper metabolism
• liver cirrhosis
• CNS (basal ganglia): initial sign is often progressive dystonia

Question 48

Drug of choice for children with generalized dystonia

Answer 48

anticholinergic agent trihexyphenidyl (Artane)

Question 49

Apneic and focal motor seizures that start around the fifth day of life

Answer 49

benign idiopathic neonatal seizures (fifth day fits)

Question 50

Childhood seizure disorder that lasts for only a few seconds, and are accompanied by flutter or upward rolling of the eyes but typically not by automatisms of the complex partial seizure type (absence seizures can have simple automatisms like lip-smacking or picking at clothing and the head can minimally fall forward). Usually starts at 5-8yr of age and are often, owing to their brevity, overlooked by parents for many months even though they can occur up to hundreds of times per day.

Answer 50

Absence seizure

• do not have a postictal period and are characterized by immediate resumption of what the patient was doing before the seizure
• Hyperventilation for 3-5min can precipitate the seizures and the accompanying 3Hz spike–and–slow wave discharges

Question 51

Aphasia+Seizure disorder
- characterized by loss of language skills in a previously normal child, common in boys; the aphasia may be primarily receptive or expressive, and auditory agnosia may be so severe that the child is oblivious to everyday sounds. Hearing is normal, but behavioral problems, including irritability and poor attention span, are particularly common. Seizure may be focal or generalized tonic-clonic, atypical absence, partial complex, and, occasionally, myoclonic

Answer 51

Landau-Kleffner syndrome

• Anticonvulsant of choice: valproic acid

Question 52

Seizure lasting for more than 30 min or recurrent seizure lasting for 30 min from which the patient does not regain consciousness

Answer 52

Status epilepticus

• although popular definition is recurrent or continuous seizures lasting for at least 30 min, it is recommended to start treatment using status epilepticus protocol when seizures persist for more than 5 minutes

Question 53

What are the priorities in the management of status epilepticus?

Answer 53

• airway: lateral decubitus position, suction secretions, intubate when indicated (sedate to avoid vasovagal stimulation)
• oxygenate and ventilate
• check NVS
• secure IV access, D50.9 NaCl (except for neonates)
• drug therapy
  0-5 min: diazepam (0.3 mg/kg IV), lorazepam IV or rectal diazepam/ buccal midazolam
  5-20 min: Pb 20 mg/kg IV, rate 1-2 mg/kg/min, max 800 mg
  20-30 min: additional 10 mg/kg Pb if max dose not yet reached

Question 54

A 15-year-old adolescent male experiences sudden-onset severe weakness of the right leg while playing soccer and collapses to the ground. His right arm is also weak, and his speech is slurred. He is immediately transported to the emergency room.
The patient has no prior history of any medical problems. He has never had a migraine headache or a seizure, and he does not report a headache now. He takes no medication.
He presents as a frightened, healthy-appearing teenager. His blood pressure is 145/87. He does not have a fever. The neck is supple, and no carotid or cranial bruits are noted. The cardiac rhythm and heart sounds are normal. The patient’s
P.271

speech is slurred but intelligible, and he follows directions well. The visual fields are full to confrontation. The pupils are equal and reactive, and the extraocular movements are intact. Facial, tongue, and palatal movements are symmetrical. Motor examination reveals decreased tone on the right side, a positive pronator drift test, slow fine finger movements, 4/5 strength in the upper extremity, and 3/5 strength in the lower extremity. Motor examination of the left upper and left lower extremity is normal. The deep tendon reflexes are diminished on the right side, and the right toe does not move in response to plantar stimulation.
A CT scan of the head reveals no evidence of bleeding or any other intracranial abnormality.

What is your diagnosis?

Answer 54

Acute ischemic stroke

• disorders of the cerebral arteries and has emerged as the leading cause of childhood AIS, present in >50% of children
• 3 types of etiology: arteriopathic, cardiac, hematologic

Question 55

What are the 3 basic guidelines in the treatment of acute ischemic stroke?

Answer 55

Antithrombotic strategies: anticoagulation
Neuroprotective strategies: control of blood glucose, temperature, and seizures and aggressive maintenance of cerebral perfusion pressure
Secondary stroke prevention: antiplatelet therapy

Question 56

What is the leading cause of term-born cerebral palsy (congenital hemiplegia)?

Answer 56

Perinatal stroke

Question 57

A group of permanent disorders of movement and posture causing activity limitation, that are attributed to nonprogressive disturbances in the in the developing fetal or infant brain

Answer 57

cerebral palsy

Question 58

What are the major lesions that contribute to CP in very premature infants weighing <1000 g?

Answer 58

intracerebral hemorrhage and periventricular leukomalacia (PVL)

Question 59

What is the first clinical indication of spastic diplegia in a child?

Answer 59

The 1st clinical indication of spastic diplegia is often noted when an affected infant begins to crawl. The child uses the arms in a normal reciprocal fashion but tends to drag the legs behind more as a rudder (commando crawl) rather than using the normal four-limbed crawling movement

• spastic legs with brisk reflexes, ankle clonus, and a bilateral Babinski sign

Question 60

What is most common neuropathologic finding in children with spastic diplegia?

Answer 60

PVL

• visualized on MRI in more than 70% of cases

Question 61

The most severe form of CP because of marked motor impairment of all extremities and the high association with mental retardation and seizures

Answer 61

spastic quadriplegia

Question 62

Which disease causes prominent dystonia that can resemble CP but with normal MRI?

Answer 62

dihydroxyphenylalanine (DOPA)-responsive dystonia (Segawa disease)

• diurnal variation in their signs with worsening dystonia in the legs during the day
• These patients can be tested for a response to small doses of l-dopa and/or cerebrospinal fluid can be sent for neurotransmitter analysis

Question 63

Which type of CP is associated with kernicterus?

Answer 63

athetoid CP

Question 64

What is the diagnostic study of choice essential in the diagnosis of spinal cord tumors, especially intramedullary spinal cord tumors?

Answer 64

MRI with and without gadolinium enhancement of the spinal cord

Question 65

Cystic distension of the spinal cord caused by obstruction of the flow of spinal fluid from within the spinal cord to its point of absorption.
- classic presentation is the central cord syndrome. In this situation the patient develops numbness beginning in the shoulder in a capelike distribution followed by the development of atrophy and weakness in the upper extremities.

Answer 65

syringomyelia

Question 66

What is the most common cause of bacterial meningitis in children 1mo to 12yr of age?

Answer 66

Neisseria meningitidis

Question 67

Give the normal values of CSF findings

pressure:
leukocytes:
protein:
glucose:

Answer 67

pressure: 50-80 mm H2O
leukocytes: 50 mg/dL (or 75% of serum glucose)

Question 68

What are the clinical signs of meningeal irritation?

Answer 68

Meningeal irritation is manifested as nuchal rigidity, back pain, Kernig sign (flexion of the hip 90 degrees with subsequent pain with extension of the leg), and Brudzinski sign (involuntary flexion of the knees and hips after passive flexion of the neck while supine)

Question 69

What are the signs of increased ICP?

Answer 69

Increased ICP is suggested by headache, emesis, bulging fontanel or diastasis (widening) of the sutures, oculomotor (anisocoria, ptosis) or abducens nerve paralysis, hypertension with bradycardia, apnea or hyperventilation, decorticate or decerebrate posturing, stupor, coma, or signs of herniation.

Question 70

What are the contraindications for an immediate LP?

Answer 70

1) evidence of increased ICP (other than a bulging fontanel), such as 3rd or 6th cranial nerve palsy with a depressed level of consciousness, or hypertension and bradycardia with respiratory abnormalities
2) severe cardiopulmonary compromise requiring prompt resuscitative measures for shock or in patients in whom positioning for the LP would further compromise cardiopulmonary function; and
3) infection of the skin overlying the site of the LP.

Thrombocytopenia is a relative contraindication for LP. If an LP is delayed, empirical antibiotic therapy should be initiated.

Question 71

The most common sequela of bacterial meningitis and, usually, is already present at the time of initial presentation

Answer 71

sensorineural hearing loss

• The most common neurologic sequelae include hearing loss, mental retardation, recurrent seizures, delay in acquisition of language, visual impairment, and behavioral problems

Question 72

What is the most common etiology of viral meningoencephalitis?

Answer 72

enteroviruses

Question 73

What is the usual treatment regimen for brain abscess?

Answer 73

MCV (metronidazole + 3rd gen ceph (cefta/ceftri) + vancomycin)

• if etiology is cyanotic CHD: ampi-sul or (3rd gen ceph + metro)

Question 74

When is surgical excision of a brain abscess indicated?

Answer 74

when the abscess is >2.5cm in diameter, gas is present in the abscess, the lesion is multiloculated, the lesion is located in the posterior fossa, or a fungus is identified

Question 75

progressive hydrocephalus with a myelomeningocele

Answer 75

type II Chiari malformation

Question 76

Cystic expansion of the 4th ventricle in the posterior fossa and midline cerebellar hypoplasia, which results from a developmental failure of the roof of the 4th ventricle during embryogenesis. Infants present with a rapid increase in head size and a prominent occiput. Transillumination of the skull may be positive. Most children have evidence of long-tract signs, cerebellar ataxia, and delayed motor and cognitive milestones, probably due to the associated structural anomalies.

Answer 76

Dandy Walker malformation

Question 77

Patient presents with gradual onset of weakness beginning in the lower extremities and progressively involving the trunk, the upper limbs, and finally the bulbar muscles. This was preceded by 10 days of nonspecific viral infection. Proximal and distal muscles are noted to be symmetrically involved. Patient was noted to be irritable. Weakness was noted to progress to inability or refusal to walk and later to flaccid tetraplegia.

What is your diagnosis?

Answer 77

Guillain-Barre Syndrome

• pattern of weakness described is called Landry ascending paralysis
• in cases with an abrupt onset, tenderness on palpation and pain in muscles is common in the initial stages
• affected children are irritable; paresthesias can also occur
• implicated preceding infections: Campylobacter, Helicobacter, Mycoplasma
• Dx: CSF studies (high protein, low WBC, normal glucose)
• Tx: IVIG

Question 78

In patients with GBS what are the impending signs of respiratory failure?

Answer 78

dysphagia and facial weakness

-3 clinical features are predictive of poor outcome with sequelae: cranial nerve involvement, intubation, and maximum disability at the time of presentation

Question 79

In the diagnosis of GBS, CSF studies are essential. What is the classic CSF picture in GBS?

Answer 79

CSF protein is elevated to more than twice the upper limit of normal, glucose level is normal, and there is no pleocytosis. >10 WBCs/mm3 are found.

• The dissociation between high CSF protein and a lack of cellular response in a patient with an acute or subacute polyneuropathy is diagnostic of Guillain-Barre syndrome.
• Tx: IVIG

Question 80

chronic disease characterized by rapid fatigability of striated muscle

Answer 80

Myasthenia gravis

Question 81

What are the signs and symptoms of cerebral herniation? Describe how this can be prevented.

Answer 81

pupillary dilatation, systemic hypertension, bradycardia, extensor posturing
hyperventilation with an Fio2 of 1.0,
intubating doses of either thiopental or pentobarbital and
either mannitol (0.25-1.0g/kg, IV) or hypertonic saline (3% solution, 5-10mL/kg IV)

Question 82

What are the first line maneuvers in managing increased ICP?

Answer 82

ICP should be maintained <20mmHg
elevate head of the bed
ensure midline positioning of the head
controlled mechanical ventilation
sedation and analgesia (i.e., benzodiazepines and narcotics)
mannitol or hypertonic saline

Question 83

What is the visual acuity of term infants and when does it approach adult levels (20/20)?

Answer 83

Visual acuity in term infants approximates 20/150 and reaches the adult level of 20/20 by about 6mo of age