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Pediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Seizures in the neonatal period may be associated with what type of hypotonia

A

Central hypotonia

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2
Q

Acquired causes of central hypotonia

A
  • Electrolyte abnormalities
  • Hypoxic-ischemic injury
  • Infection: sepsis, meningitis
  • Intracranial hemorrhage
  • Trauma: cerebral, cervical
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3
Q

Congenital causes of central hypotonia

A
  • Cerebral malformation
  • Chromosomal disorder: Down syndrome, Prader-Willi syndrome
  • Metabolic disorder: urea cycle defect
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4
Q

Causes of peripheral hypotonia

A
  • Spinal cord: spinal muscular atrophy
  • Peripheral nerves: familial dysautonomia
  • Neuromuscular junction: botulism, neonatal myasthenia, magnesium toxicity
  • Muscle: congenital muscular dystrophy, congenital myotonic dystrophy, metabolic myopathy (Pompe’s disease, phosphofructokinase deficiency), structural myopathy (central core disease, nemaline rod myopathy)
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5
Q

Spinal muscular atrophy

A

Anterior horn cell degeneration that presents with hypotonia, weakness and tongue fasciculations

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6
Q

Classifications of spinal muscular atrophy

A
  • Type I: infantile form with onset less than 6 months, aka Werdnig-Hoffman disease
  • Type II: intermediate form with onset 6-12 months
  • Type III: juvenile form with onset after 3 years
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7
Q

Etiology of spinal muscular atrophy

A
  • Autosomal recessive
  • Mutation of survival motor neuron gene (SMN1) on chromosome 5
  • Pathology of the spin cord shows degeneration and loss of anterior horn motor neurons and infiltration of microglia and astrocytes
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8
Q

Clinical features of spinal muscular atrophy

A
  • Weak cry, tongue fasciculations, and difficulty sucking and swallowing
  • Bell shaped chest
  • Frog leg posture when in supine position, generalized hypotonia, weakness and areflexia
  • Normal extraocular movements and normal sensory exam
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9
Q

Infantile botulism

A

Bulbar weakness (impairment in CN IX-XII) and paralysis that develops infants during the first year of life secondary to ingestion of Clostridium botulinum spores and absorption of the toxin

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10
Q

Etiology of infantile botulism

A

Toxin prevents the presynaptic release of acetylcholine

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11
Q

Clinical features of infantile botulism

A
  • Onset of symptoms occurs 12-48 hours after ingestion of spores
  • Constipation is the classic first symptom of botulism
  • Neurologic symptoms follow, including weak cry and suck, loss of previously obtained motor milestones, ophthalmoplegia and hyporeflexia
  • Paralysis is symmetric and descending
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12
Q

Management of infantile botulism

A
  • Botulism immune globulin improves the clinical course

- Antibiotics are contraindicated and may worsen the clinical course

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13
Q

Congenital myotonic dystrophy

A
  • Autosomal dominant
  • Muscle disorder that presents in the newborn period with weakness and hypotonia
  • Myotonia is the inability to relax contracted muscles
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14
Q

Etiology of congenital myotonic dystrophy

A
  • Trinucleotide repead disorder with autosomal dominant inheritance and variable penetrance
  • Chromosome 19
  • Transmission to affected infant is throughout the affected mother in more than 90% of cases
  • the earlier the onset of the disease in the mother, the more likely she will have affected offspring
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15
Q

Clinical features of congenital myotonic dystrophy

A
  • Polyhydramnios
  • Decreased fetal movement
  • Feeding and respiratory problems
  • Physical examination is notable for facial diplegia (bilateral weakness), hypotonia, areflexia and arthrogryposis (multiple joint contractors)
  • Myotonia is not always present in the newborn but develops later, almost always be 5 years
  • In adulthood, typical myotonic features include myotonic facies (atrophy of masseter and temporal is muscles), ptosis, a stiff straight smile and inability to release the grip after hand shaking (myotonia)
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16
Q

Congenital causes of hydrocephalus

A
  • Chiari type II malformation
  • Dandy-Walker malformation
  • Congenital aqueductal stenosis
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17
Q

Chiari type II malformation

A
  • Downward displacement of the cerebellum and medulla through the foramen magnum, blocking CSF flow
  • Often associated with lumbosacral myelomeningocele
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18
Q

Dandy-Walker malformation

A

Combination of an absent or hypo plastic cerebellar vermis and cystic enlargement of the fourth ventricle, which blocks the flow of CSF

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19
Q

Congenital aqueductal stenosis

A

Some cases of aqueductal stenosis are inherited as an X linked trait and these patients may have thumb abnormalities and other CNS anomalies such as spina bifida

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20
Q

Sunset sign

A

A tonic downward deviation of both eyes caused by pressure from the enlarged third ventricles on the upward gaze center in the midbrain

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21
Q

Highest and lowest incidence of spina bifida occur where

A

Highest incidence occurs in Ireland and lowest in Japan

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22
Q

Associated anomalies and complications of spina bifida

A
  • Hydrocephalus
  • Cervical hydrosyringomyelia (accumulation of fluid within the central spinal cord canal and with the cord itself)
  • Defect in neuronal migration
  • Orthopaedic problems
  • Genitourinary defects
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23
Q

Most common causes of coma in children younger than 5

A

Nonaccidental trauma and near-drowning

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24
Q

Most common causes of coma in older children

A

Drug overdose and accidental head injury

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25
Q

Flaccidity or no movement suggests

A

Severe spinal or brainstem injury

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26
Q

Decerebrate posturing (extension of arms and legs) suggests

A

Subcortical injury

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27
Q

Decorticate posturing (flexion of arms and extension of legs) suggests

A

Bilateral cortical injury

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28
Q

Asymmetric responses suggests

A

Hemispheric injury

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29
Q

Hypoventilation suggests

A

Opiate or sedative overdose

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30
Q

Hyperventilation suggests

A
  • Metabolic acidosis (Kussmaul respirations or rapid, deep breathing, may occur)
  • Neurogenic pulmonary edema
  • Midbrain injury
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31
Q

Cheyne-Stokes breathing (alternating apnea and hyper apneas) suggests

A

Bilateral cortical injury

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32
Q

Apneustic breathing (pausing at full inspiration) suggests

A

Pontine damage

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33
Q

Ataxic or atonal breathing (irregular respirations with no particular pattern) suggests

A

Medullary injury and impending brain death

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34
Q

Unilateral dilated nonreactive pupil suggests

A

Uncal herniation

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35
Q

Bilateral dilated nonreactive pupils suggest

A
  • Topical application of dilating agent
  • Postictal state
  • Irreversible brainstem injury
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36
Q

Bilateral constricted reactive pupils suggest

A
  • Opiate ingestion

- Pontine injury

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37
Q

Oculocephalic maneuver (doll’s eyes)

A
  • When turning the head of the unconscious patient, the eyes normally look straight ahead and the slowly drift back to midline position because the intact vestibular apparatus senses a change in position
  • INJURED BRAINSTEM: movement of the head does not evoke any eye movement; termed negative oculocephalic maneuver
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38
Q

Caloric irrigation

A
  • When the oculocephalic response is negative or cannot be performed because of possible cervical cord injury, this test should be performed
  • Involves angling the head at 30 degrees and irrigating each auditory canal with 10-30 mL of ice water
  • An intact (normal) cold caloric response is reflected by eye deviation to the irrigated side
  • Abnormal response suggests PONTINE INJURY
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39
Q

Abnormal corneal and gag reflexes suggest

A

Significant brainstem injury

40
Q

Causes of acute seizures during childhood

A
  • Head trauma: cerebral contusion, subdural hematoma
  • Brain tumor: astrocytoma, meningioma
  • Toxins: amphetamines, cocaine
  • Infections: meningitis, encephalitis, brain abscess, neurocysticercosis
  • Vascular: cerebral infarction, intracranial hemorrhage
  • Metabolic disturbances: hypocalcemia, hypoglycemia, hypomagnesemia, hyponatremia, hypernatremia, pyridoxine deficiency
  • Systemic diseases: hypertension, hypoxic-ischemic injury, inherited metabolic disorder, liver disease, renal failure, neurocutaneous disorders (tuberous sclerosis)
41
Q

Types of generalized seizures

A
  • Tonic-clonic (most common) (has postictal state)
  • Tonic
  • Clonic
  • Myoclonic
  • Absence (no postictal state)
  • Atonic
42
Q

Alternative treatments for seizures

A
  • Vagal nerve stimulator: pacemaker sized device that sends an electrical impulse to the vagus nerve; common side effect is hoarseness
  • Ketogenic diet
43
Q

Simple febrile seizures last

A

Less than 15 minutes and is generalized

44
Q

Complex febrile seizures last

A

More than 15 minutes, has focal features or recurs within 24 hours

45
Q

Epidemiology of infantile spasms/ West syndrome

A

Age of onset is typically 3-8 months. Infantile spasms are rare in children older than 2 years.

46
Q

Etiology of infantile spasms/ West syndrome

A
  • Tuberous sclerosis is the most commonly identified cause
  • Other inherited and acquired causes include phenyketonuria, hypoxic-ischemic injury, intraventricular hemorrhage, meningitis and encephalitis
47
Q

Clinical features of infantile spasms/ West syndrome

A
  • Brief myoclonic jerks, lasting 1-2 seconds each, occurring in clusters of 5-10 seizures spread over 3-5 minutes
  • The jerks consist of sudden arm extension or head and trunk flexion (also known as jackknife seizures or salaam seizures)
48
Q

Diagnosis of infantile spasms/ West syndrome

A

EEG shows hypsarrhythmia pattern, a highly disorganized pattern of high amplitude spike and waves occurring in both cerebral hemispheres

49
Q

Management of infantile spasms/ West syndrome

A
  • ACTH intramuscular injections for a 4-6 week period are effective in more than 70% of affected patients
  • Valproate is 2nd line
  • Vigabatrin is the most effective drug for patients with infantile spasms associated with tuberous sclerosis
50
Q

Etiology of absence epilepsy of childhood

A

Autosomal dominant with age-dependent penetrance

51
Q

Clinical features of absence epilepsy of childhood

A
  • Absence seizures last 5-10 seconds
  • Occur frequently
  • Often accompanied by automatisms, such as eye blinking and incomprehensible utterances
  • Loss of posture, urinary incontinence and postictal state do not occur
52
Q

Benign rolandic epilepsy/ benign centrotemporal epilepsy

A

Involves partial seizures with secondary generalization

53
Q

Epidemiology of benign rolandic epilepsy/ benign centrotemporal epilepsy

A
  • Most common partial epilepsy during childhood
  • Commonly presents at 3-13 years
  • Peak incidence is at 6-7 years
  • Boys more likely to be affected
54
Q

Etiology of benign rolandic epilepsy/ benign centrotemporal epilepsy

A

Autosomal dominant with variable penetrance

55
Q

Clinical features of benign rolandic epilepsy/ benign centrotemporal epilepsy

A
  • Seizures occur in the early morning hours when patients are asleep with oral-buccal manifestations (moaning, grunting, pooling of saliva)
  • Seizures spread to face and arm then generalize into tonic-clonic seizures
56
Q

Diagnosis of benign rolandic epilepsy/ benign centrotemporal epilepsy

A

EEG shows biphasic spikes and sharp wave disturbance in the mid-temporal and central regions

57
Q

Management of benign rolandic epilepsy/ benign centrotemporal epilepsy

A

Valproate (1st line) or carbamazepine

58
Q

Primary intracranial causes of headaches

A
  • Primary dysfunction of neurons or muscles
  • Migraine
  • Cluster
  • Tension
59
Q

Secondary intracranial causes of headaches

A
  • Increased intracranial pressure or meningeal irritation
  • Irritative: meningitis, subarachnoid hemorrhage
  • Increased ICP: brain tumor, hydrocephalus, subdural hematoma
60
Q

Local causes of headaches

A
  • Sinusitis, perioral abscess, toothache, chronic titis media, or refractive errors
  • Ears (otitis media)
  • Eyes (refractive error, glaucoma)
  • Nose (sinusitis)
  • Mouth (toothache, abscess)
  • Temporomandibular joint dysfunction
61
Q

Systemic causes of headaches

A
  • Anemia
  • Depression
  • Hypoglycemia
  • Hypertension
  • Psychogenic
  • Carbon monoxide poisoning
62
Q

Migraine headaches

A
  • Prolonged, often more than an hour
  • Unilateral
  • Associated with nausea, vomiting, visual changes
  • Caused by changes in cerebral blood flow
63
Q

Epidemiology of migraine headaches

A
  • Most common cause of headaches in children and adolescents
  • Age of onset is younger than 5 years in 20%
  • Before puberty, incidence is higher in males
64
Q

Etiology of migraine headaches

A
  • Autosomal dominant
  • Changes in cerebral blood flow are secondary to release of 5-HT, substance P, and vasoactive intestinal peptide from changes in neuronal activity
65
Q

Most common form of migraine in children

A

Migraine without aura

66
Q

Migraine with aura

A

Onset of headaches is preceded by transient visual changes or unilateral paresthesias or weakness

67
Q

Migraine equivalent

A

In young children, the headaches itself may be absent, but there is a prolonged, but transient, alteration of behavior that manifests as cyclic vomiting, abdominal pain or paroxysmal vertigo

68
Q

Ophthalmoplegic migraine

A

Unilateral ptosis or cranial nerve III palsy accompanies this headache

69
Q

Basilar artery migraine

A

Vertigo, tinnitus, ataxia or dysarthria may precede the onset of this headache

70
Q

Clinical features of migraines

A
  • Prolonged, throbbing unilateral headache starts in the supraorbital area and radiates to the occiput
  • Nausea and vomiting
  • Visual disturbances include blurred vision, scotomata, and jagged streaks of light that take on the outline of old forts
  • Photophobia or phonophobia
  • OTC analgesics often ineffective
  • Improved with sleep
  • Neuro exam is normal
71
Q

Management of migraines

A
  • Abortive: sumatriptan, selective 5-HT agonist available in injectable, intranasal and oral forms
  • Prophylactic: propranolol
72
Q

Tension headaches

A

Bifrontal or diffuse, dull, aching headaches that are often associated with muscle contraction

73
Q

Epidemiology of tension headaches

A

Unusual during childhood and extremely rare in children younger than 7

74
Q

Clinical features of tension headaches

A
  • Dull, aching and rarely throbbing
  • Increases in intensity during the day
  • Usually bifrontal pain but may be diffuse
  • Isometric contraction of the temporalis, masseter, or trapezius often companies this headache
  • NO vomiting, visual changes or paresthesias occur
75
Q

Cluster headaches

A
  • Extremely rare in childhood
  • Unilateral frontal or facial pain, accompanied by conjunctival erythema, lacrimation and nasal congestion
  • Last less than 30 min but may recur several times in a day and then not again for weeks or months
  • Abortive: oxygen or sumatriptan
  • Prophylactic: calcium channel bluchers or valproate
76
Q

Acute cerebellar ataxia of childhood

A

Unsteady gait secondary to presumed autoimmune or postinfectious cause

77
Q

Epidemiology of acute cerebellar ataxia of childhood

A
  • Most common cause of ataxia in children
  • Age of onset between 18 months and 7 years
  • Rarely occurs in older than 10 years
78
Q

Etiology of acute cerebellar ataxia of childhood

A
  • Common preceding infections: varicella, influenza, EBV, mycoplsama
  • Follows viral illness by 2-3 weeks
  • Postulated cause is immune complex deposition in the cerebellum
79
Q

Clinical features of acute cerebellar ataxia of childhood

A
  • Truncal ataxia with deterioration of gait
  • Slurred speech and nystagmus
  • Fever is absent
80
Q

Guillain-Barre syndrome/ acute inflammatory demyelinating polyneuropathy

A

Demyelinating polyneuritis characterized by ascending weakness, areflexia and normal sensation

81
Q

Pathophysiology of Guillain-Barre syndrome/ acute inflammatory demyelinating polyneuropathy

A
  • Principal sites of demyelination are the ventral spinal roots and peripheral myelinated nerves
  • Injury is triggered by a cell mediated immune response to an infectious agent that cross reacts to antigens on the Schwann cell membrane
82
Q

Clinical features of Guillain-Barre syndrome/ acute inflammatory demyelinating polyneuropathy

A
  • Ascending, symmetric paralysis
  • No sensory loss
  • Cranial nerve involvement
  • MILLER-FISHER SYNDROME: variant of Guillan-Barre syndrome characterized by ophthalmoplegia, ataxia and areflexia
83
Q

Albuminocytologic dissociation

A
  • Finding in Guillan-Barre syndrome

- Increase in CSF protein in absence of increased cell count

84
Q

Management of Guillain-Barre syndrome/ acute inflammatory demyelinating polyneuropathy

A
  • IVIG

- Plasmapheresis - removes patients plasma along with anti-myelin antibodies

85
Q

Syndenham chorea

A

Self limited autoimmune disorder associated with rheumatic fever that presents with chorea and emotional lability

86
Q

Epidemiology of syndenham chorea

A
  • Occurs in 25% of patients with rheumatic fever

- Most commonly between 5-13 years

87
Q

Pathophysiology of syndenham chorea

A

Secondary to antibodies that cross react with membrane antigens on both group A beta hemolytic streptococcus and basal ganglia cells

88
Q

Clinical features of syndenham chorea

A
  • Immunologic response usually follows the streptococcal pharyngitis by 2-7 months
  • Children appear restless
  • Speech affected
  • Unable to sustain protrusion of tongue (chameleon tongue)
  • Wrist is held flexed and hyperextended at the metacarpal joints (choleric hand)
  • On gripping examiners fingers, patients are unable to maintain the grip (milkmaid’s grip)
  • Emotional lability
  • Gait and cognition are not affected
89
Q

Neuroimaging of syndenham chorea

A
  • MRI may show increased signal intensity in the caudate and putamen on T2 sequence
  • Single photon emission computed tomography (SPECT) may demonstrate increased perfusion to thalamus and striatum
90
Q

Management of syndenham chorea

A
  • Haloperidol
  • Valproate
  • Phenobarbital
91
Q

Tourette syndrome

A
  • Chronic lifelong movement disorder that presents with motor and phonic tics before 18 years
  • Tics are brief, stereotypical behaviors that are initiated by an unconscious urge that can be temporarily suppressed
92
Q

Clinical features of Tourette syndrome

A
  • Motor tics can be simple (e.g. eye blinking, head or shoulder shaking) or complex (e.g. bouncing, jumping, kicking)
  • Phonic tics can be simple (e.g. coughing, groaning, barking) or complex (e.g. echolalia)
  • Tics must be present greater than 1 year
  • Absence of any signs of a neurodegenerative disorder
  • Associated findings include learning disabilities, ADHD, and OCD
93
Q

Management of Tourette syndrome

A
  • Pimozide is the drug of choice because it is effective with minimal extrapyramidal side effects
  • Clonidine is less effective, major side effect is sedation
  • Haloperidol, but tardive dyskinesia limits use
  • Hypnotherapy
94
Q

Duchenne and Becker MDs

A
  • Progressive, X linked myopathies characterized by myofiber degneration
  • DMD is more severe than BMD
  • Onset of symptoms is between 2-5 years
95
Q

Pathophysiology of DMD and BMD

A
  • Dystrophin is a high molecular weight cytoskeletal protein that associates with actin and other structural membrane elements
  • Absence of dystrophin causes weakness and eventually rupture of the plasma membrane, leading to injury and degeneration of muscle fibers
96
Q

Pathology of DMD and BMD

A
  • Degeneration and regeneration of muscle fibers
  • Infiltration of lymphocytes into the injured area and replacement of damaged muscle fibers with fibroblasts and lipid deposits
97
Q

Clinical features of DMD and BMD

A
  • Slow, progressive weakness affecting the legs first
  • In DMD, children lose the ability to walk by 10 years but in BMD by 20 or more years
  • Pseudohypertrophy of calves is present because of the excess accumulation of lipids, which replace the degenerating muscle fibers
  • Gower’s sign - due to weakness of pelvic muscles, patients arise from the floor in a characteristic manner by extending each leg and then climbing up each thigh until they reach an upright position
  • Cardiac involvement
  • Mild cognitive impairment in DMD

Pediatrics (11 decks)

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