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Pediatrics 2 > Neurology > Flashcards

Neurology Flashcards

1
Q

What characterizes a static condition?

A

seen in first few months and do not change over time

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2
Q

What characterizes a progressive condition?

A

degenerative disease or neoplasm that worsens over time

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3
Q

What characterizes an intermittent or brief disease?

A

Epileptic or migraine-like symptoms. You either have it or you don’t.

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4
Q

What characterizes a saltatory condition?

A

bursts of symptoms followed by partial recovery, some periods are worse than others

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5
Q

Ophthalmoplegia and Limb Weakness

A

Myasthenia gravis, Botulism, Myotonic dystrophy, Miller Fisher variant of GB sydnrome

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6
Q

Facial and Bulbar Weakness

A

All of the same conditions as ophthalmoplegia and limb weakness plus polio and facioscapulohumeral dystrophy

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7
Q

Spinal muscular atrophy syndrome

A

AR, Chr 5, progressive weakness of LMN due to degeneration of anterior horn cells, most are present at infancy

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8
Q

What disorder in adults is spinal muscular atrophy syndrome similar to?

A

ALS– Flaccid weakness (symmetric), decreased spontaneous movement, hypotonia, decreased DTR, fasciculation, progress to decreased facial expressions with increased drooling. death due to respiratory dys or infection.

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9
Q

What is important to remember about patients with spinal muscular atrophy syndrome?

A

They have normal cognitive, social, and language development

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10
Q

What is acute transverse myelitis? Where is it usually located?

A

It is acute inflammation of gray and white matter of one or more spinal cord segments, usually thoracic

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11
Q

What is the cause of acute transverse myelitis?

A

Unknown, but related to multiple sclerosis, infections, autoimmune or post-infectious inflammation, vasculitis, and certain drugs

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12
Q

What are the symptoms of acute transverse myelitis?

A

Onset of hours to weeks of bilateral motor, sensory, and sphincter deficits below the level of the lesion. Usually comorbid with back pain, sudden paresthesias, weakness.

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13
Q

How is a diagnosis of acute transverse myelitis made?

A

MRI and CSF analysis. MRI will reveal lighter areas of localized inflammation on the peripheral surfaces

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14
Q

What is Guillain Barre Syndrome also known as? What is it described as? What causes it?

A

Acute idiopathic polyneuritis; inflammatory peripheral neuropathy; caused by post infectious (respiratory or GI) campylobacter jejuni (maj), cytomegalovirus, EBV, and mycoplasma pneumoniae

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15
Q

What are the hallmarks of GB syndrome?

A

Rapid loss of motor strength (ascending), symmetric weakness, many have sensory symptoms with few sensory findings, loss of DTRs EARLY ON

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16
Q

What other symptom might someone with GB syndrome have?

A

Autonomic nerve dysfunction

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17
Q

How is a diagnosis of Guillain Barre Syndrome made?

A

Elevated CSF protein with no increased WBC

EMG

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18
Q

What is the mortality of someone with Guillain Barre Syndrome?

A

1-2% if treated properly, 20% end up on ventilator

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19
Q

What is the treatment for Guillain Barre Syndrome?

A

IV Immunoglobulins, plasmaphoresis

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20
Q

What is myasthenia gravis?

A

Chronic disease of rapid fatigability of striated muscle. It is usually immune mediated and creates a NM blockade. The post-synaptic muscle membrane (motor end plate) is less responsive due to circulating Ach nicotinic receptor binding antibodies.

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21
Q

What age groups can you see myasthenia gravis?

A

begins in teenage years

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22
Q

What are hallmark symptoms of myasthenia gravis?

A

Ptosis, diplopia, ophthalmoplegia, weakness of face/extremities, dysphagia. Symptoms worsen throughout the day or with exertion. No primary complaint of muscle pain or sleepiness

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23
Q

How do you diagnose myasthenia gravis?

A

CT scan for thymoma, serology for autoantibodies, NCS/EMG (repetitive), tensilon (AchE) challenge test

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24
Q

How do you treat myasthenia gravis?

A

AChE inhibitors, immunosuppressors

25
Q

What is the most common hereditary NM disease?

A

Duchenne + Becker Muscular dystrophy

26
Q

What is the sign for Duchenne Becker Muscular Dystrophy? What kind of heredity is it?

A

Gower’s Sign, sex-linked recessive

27
Q

What are some hallmarks of DMD?

A

Mild intellectual impairment, hypertrophy of calves, proliferation of connective tissue

28
Q

How do you diagnose DMD?

A

Muscle biopsy, absence of dystrophin (an Ig protein associated with muscle fiber pm), EMG, serum CPK levels-markedly elevated

29
Q

What is the primary treatment for DMD?

A

symptomatic with glucocorticoids, gene therapy is new, also combined with creatine and aminoglycosides

30
Q

What is the scapulohumeral muscular dystrophy?

A

AKA Emery-Dreifuss MD, it is a group of myopathies that affect the muscles of the hip and shoulder

31
Q

When is the onset of Emery-Dreifuss MD?

A

Mid to late childhood and early adolescence, usually wheelchair bound by age 30

32
Q

What is Limb-Girdle muscular dystrophy?

A

X-linked recessive long arm Xq28. It includes other mutations that cause myotubular myopathy

33
Q

What are two other myotubular myopathies that are associated with the Xq28 chromosome?

A

Adrenoleukocystrophy and Block-Sulzberger (pigment changes)

34
Q

What is the age of onset for Limb-Girdle MD?

A

5-15 years, cardiomyopathy is severe

35
Q

What are some symptoms of pediatric brain tumors?

A

Headache, nausea and vomiting, loss of balance, speech problems, sleepiness, personality changes, seizures, increased head circumference in infants

36
Q

What are the three types of gliomas we discussed?

A

Astrocytoma, brain stem glioma, and ependymomas

37
Q

What percentage of brain tumors in children are meduloblastomas?

A

15%

38
Q

What chromosome is Type 1 Neurofibromatosis (NF) carried on? What is its classification?

A

AD on 17; peripheral

39
Q

What chromosome is Type 2 Neurofibromatosis carried on? What is its classification? What are its characteristics?

A

AD on 22; central; incurable disease-multiple intracranial and spinal tumors.

40
Q

What are the physical characteristics of NF?

A

Cafe-au-lait spots, numerous macules greater than .5 cm present at birth. Neurofibromas form in late adolescence along nerves. There is also axillary and groin freckling and a greater incidence of CNS tumors.

41
Q

Is there any cognitive impairment with NF?

A

There is a range of cognitive impairment

42
Q

How do we treat NF?

A

Surgical excision of tumors

43
Q

Which fontanelle closes first? second? third?

A

posterior; sphenoid; anterior

44
Q

What characterizes an upper motor neuron lesion?

A

Spasticity, negative fasciculations, and increased tone (CNS)

45
Q

What characterizes a lower motor neuron lesion?

A

Flaccidity, positive fasciculations, decreased tone (PNS)

46
Q

Moro reflex

A

sudden head extension then extension and flexion of limbs, subsides at 0-6 months, originates in the brainstem

47
Q

Rooting reflex

A

tactile stimulus near the mouth then pursuing stimulus, subsides at 0-6 months, originates in the brainstem

48
Q

Trunk incurvation reflex

A

Stroke skin near vertebrae and display curvature of the spine opposite, subsides at 0-6 months, originates in the spinal cord

49
Q

Cerebral Palsy classification

A

70% of cases are caused prenataly, usually idiopathic. 4-9% may be due to asphyxia at delivery. NOT HEREDITARY.

50
Q

How do you treat cerebral palsy?

A

OT/PT/ST, anti-spasmodics such as dantrolene, some benzos. Botulism toxin

51
Q

What is the most frequent permanently disabling birth defect?

A

Spina bifida

52
Q

In what condition are Arnold-Chiari malformations present? And what are they?

A

Spina bifida, cerebellar herniation through foramen magnum

53
Q

What is the clinical presentation of spina bifida?

A

occipital HA that radiates up, becomes worse with valsalva

54
Q

What is the treatment for spina bifida?

A

Pain treatment or surgical decompression

55
Q

What other medical condition is spina bifida occasionally a/w?

A

hydrocephalus and 4th ventricle obstruction

56
Q

What is the most severe type of spina bifida? What are its characteristics?

A

Meningiomyelocele; SC or nerve roots are exposed on the back, area covered with sores and CSF leaks, leg paralysis and incontinence, Chiari malformations

57
Q

What is the rarest form of spina bifida? What are its characteristics?

A

Only meninges are exposed in a cyst-like sac, underlying sc is intact, no HC or neurologic deficits

58
Q

What is Dandy-Walker syndrome?

A

Enlargement of the 4th ventricle, 90% have hydrocephalus, usually a posterior fossa cyst, 50% have normal IQ

59
Q

How do you treat hydrocephalus?

A

Loop diuretic, acetazolamide, surgery with a shunt and removal of the obstruction

Pediatrics 2 (3 decks)

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