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Pediatrics > Tone Disorders > Flashcards

Tone Disorders Flashcards

1
Q

Is cerebral palsy a progressive disease?

A

No, but clinical picture change ass CNS matures

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2
Q

What are the factors that may increase the risk of developing cerebral palsy?

A
  • decrease gestational age

- decrease birth weight

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3
Q

Name possible causes for cerebral palsy other than prematurity and low birth weight

A 
1-preeclampsia 
2- intrauterine infection 
3- placental abruption 
4- multiples 
5- maternal alcohol consumption 
6- kernicturus
7- PKU
8- meningitis\encephalitis 
9- IUGR 
10- asphyxia 
11- trauma
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4
Q

For functional evaluations of CP, name the levels from 1 to 5

A 
Walk:
1-  w\out limitation
2- w\limitation 
3- w\hand held mobility device 
4- w\powered self mobility 
5- manual wheelchair
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5
Q

Name the subtypes of CP:

A

1- spastic diplegia - hemiplegia - quadraplegia
2- Dyskinetic subtype
3- Ataxic CP

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6
Q

What subtype of CP is associated with PVL on MRI?

A

Spastic diplegia

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7
Q

What limbs are affected usually in spastic diplegia

A

Lower limbs more, but even the upper limbs

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8
Q

What causes spastic hemiplagia in neonates?

A

Neonatal stroke

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9
Q

Which CP subtype predominantly affect preterm more than term infants?

A

Spastic diplegia

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10
Q

What is the most common subtype of CP?

A

Spastic quadraplegia

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11
Q

What is a common clinical feature of CP spastic quadraplegia subtype?

A

Psychomotor delay

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12
Q

In dyskinetic CP, what is structure of the brain are most commonly affected?

A

Thalamus, basal ganglia, cerebellum.

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13
Q

What is the cause for ataxic CP and how it usually presents?

A

Cerebellar hypoplasia.

Hypotonia and incordination

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14
Q

How does the periventricular leukomalacia usually develop in diplegic CP?

A

1- Starts as intraventricular hemorrhage seen in antenatal ultrasound
2- hyperintestity cystic leison in the posterior horns
3- then it’s replaced by CSF.

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15
Q

What are the non-motor clinical manifestations of CP?

A

1- pain\irretibility
2- poor feeding, GERD, constipation
3- swallowing dysfunction & neurogenic bladder
4- speech, cognitive and developmental delay
5- poor visual attention
6- epilepsy & disturbed sleep.
7- deformities, scoliosis, osteopenia, contracture.

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16
Q

What investigations to order to confirm CP?

A

No investigations, diagnosis is made clinically.

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17
Q

How to manage CP

A

Improve QOL, by treating dystonia, spasticity, orthopedic problems, medical comorbidities

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18
Q

Name diseases that affect the lower motor neurons and localize the site of lesion

A
  • spinal muscular atrophy: ant horn cells
  • guillen-barre syndrome: peripheral nerve
  • boutlism: NMJ
  • duchenne muscular dystrophy: muscle
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19
Q

Spinal muscular atrophy is autosomal recessive or dominant?

A

Recessive

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20
Q

How does spinal muscular atrophy usually develops?

A

Degeneration of anterior horn cells starting from intrauterine life or any other time

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21
Q

What is the mutation in spinal muscular atrophy?

A

(SMN1) in chromosome 5q13.2

[biallelic deletion or mutation]

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22
Q

In spinal muscular atrophy, which is more affected

  • the upper limb or lower limb.
  • The proximal part or the distal part?
A

symmetrical Lower limb the proximal part

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23
Q

Other than limb manifestations of spinal muscular atrophy, what else is affected?

A

1- bulbar muscles: weak cry, poor suck\ swallow, & tongue fascinations.
2- absent deep tendon reflex
3- Respiratory muscle weakness causing respiratory failure.

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24
Q

What will be the findings in examination of spinal muscle atrophy?

A

Paradoxical respiration (bell shaped chest)

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25
Q

What are the investigations to order in spinal muscular dystrophy?

A

1- CK: normal to increased
2- EMG: fasciculation, fibrillation, denervation
3- genetic testing: SMN1
4- muscle biopsy: grouped atrophy

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26
Q

What is the risk associated with prenatal SMA? (SMA type 0)

A

Usually dead at birth or have a life expectancy of <6m.

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27
Q

Differentiate between SMA type 1 and type 2?

A

Type 1 usually occur in <3 month & are unable to sit.

Type 2 usually occur in 3-6 month & are able to sit.

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28
Q

In type 3 SMA, the child is able to ……… and unable to ………. :

A

Able to stand - sit

Unable to walk.

29
Q

In which type of Spinal muscle atrophy is child able to walk assisted an un-assisted?

A

Assisted: type 3

Un-assisted: type 4

30
Q

How to treat SMA?

A

Genetic intrathecal injection of spinaraz

using adenovirus to deliver genetic information

31
Q

What is the most common cause of acute flaccid paralysis in children

A

GBS

32
Q

What is the etiology of GBS?

A

Immune mediated

33
Q

What is a key word to look for in a case of GBS?

A

The patient developed GI (campylobacter) or respiratory (mycoplasma pneumoni) infection 10 days prior to symptoms

34
Q

What is the most common form of GBS?

A

AIDP

“Acute inflammatory demylinating polyriduclopathy”

35
Q

What is the classical presentation of GBS?

A

Tingling sensation in toes, and ascending up to the arms in the course of hours to days.

involve muscles of respiration in severe cases.

36
Q

What is the miler fisher variant of GBS?

A

Ataxia - partial ophthalmoplegia - areflexia

37
Q

What are the findings in physical examination of GBS?

A
  • symmetrical weakness
  • diminished\absent reflex
  • gait abnormalities
38
Q

How to treat GBS?

A

IVIG or plasma exchange

39
Q

What is the organism responsible for developing infantile botulism?

A

Clostridium botulinum

40
Q

What is the risk of infantile botulism?

A

Life threatning neuroparalytic syndrome

41
Q

How does clostridium cause infantile botulism?

A

Blocks (pre-synaptic) chloinergic transmission at NMJ

42
Q

Differentiate between the pattern of spread of weakness in infantile botulism vs GBS?

A

GBS: ascending
Infantile: descending

43
Q

What is the clinical presentation of infantile botulism

A
  • constipation\urinary retention
  • hypotonia\weakness
  • cranial nerve dysfunction
  • poor feeding
44
Q

What is the cranial nerve dysfunction in infantile botulism?

A

decreased
1- gag reflex
2- eye movement - pupillary contraction - ptosis.

45
Q

Where do infants get the infection of clostridium botulinum?

A

Spores in:

  • canned food
  • honey
  • soil and dust
46
Q

What is the mode of inheretence for Duchenne muscular dystrophy?

A

X-linked recessive

47
Q

What is Duchenne muscular dystrophy?

A

Progressive myofiber degeneration adn replacement by fibrotic tissue

48
Q

What is the age that commonly affects duchenne muscular dystrophy?

A

2-3 years of age.

49
Q

What special test to preform in patients with DMD and what to expect as positive finding

A

Asking patient to stand from ground

[Gower’s sign]

(Using hands to push on legs); because it’s proximal muscle weakness

50
Q

What movement are most difficult for DMD patients to preform?

A

Running, jumping, walking up the stairs

51
Q

What is the investigation of choice for DMD?

A
  • Serum CK
    (10-20x ULN)
  • DMD genetic testinh
52
Q

What is the course of illness in DMD?

A
  • by 13: wheelchair

- by 20s: death (respiratory\CVD)

53
Q

What is the role of muscle biopsy in DMD?

A

Has no role, only showing dystrophy which is present in many other conditions

54
Q

What is the clinical presentation of DMD?

A

1- psudohypertrophy of calf + short achiles
2- scoliosis + lordosis
3- hyporeflexia + hypotonia
4- waddling gait.

55
Q

What is the sign seen in hypotonic patients with vertical suspension

A

Axillary slippage

56
Q

How is the tone in UMNL?

A

Spastic + clonus

57
Q

How is the deep tendon reflex, fasculations, wasting, planter response in UMNL

A
  • hyperreflexia
  • no fasculations
  • late wasting
  • upgoing toes
58
Q

How is the deep tendon reflex, fasculations, wasting, planter response in LMNL

A
  • hyporeflexia
  • present in ant. Horn cell lesion (SMA)
  • early wasint
  • normal response
59
Q

Name central causes of hypotonia:

A

1- Hypoxic ischemic encephalopathy HIE
2- structural brain abnormalities & genetic disorder
3- down syndrome
4- prader-willi syndrome

60
Q

What is more common central and peripheral hypotonia

A

Central

61
Q

Name peripheral causes of hypotonia:

A
  • SMA
  • congenital myopathy
  • myasthenia
  • myotonic dystrophy
62
Q

What is krabbe disease

A

Genetic disease affecting white matter

63
Q

Name clinical features of central hypotonia:

A
  • Seizure & abnormal consciousness
  • preserved power and reflex.
  • axial weakness
  • dysmorphism
64
Q

Name clinical features of peripheral hypotonia:

A
  • alert
  • absent reflex and movement
  • weakness against gravity
  • deformities of bone\joint + Fasiculations
65
Q

What are the signs of hypotonia?

A
  • scarf sign
  • frog leg
  • head lag
  • vertical: axillary slip
  • ventral: Inverted U shape
  • flat in prone position
  • poor crying, swallowing, coughing
  • abdominal breathing
66
Q

What are the maternal infections that may be associated with hypotonia

A

TORCH

  • Toxoplasmosis
  • Other: coxakie, chickebpox, HIV
  • Rubella
  • CMV
  • Herpes simplex
67
Q

Name maternal risk factors for hypotonia in pediatrics

A
  • infection
  • polyhydamnios
  • diseases (SLE, MG, DM, epilepsy)
  • medics
  • breech
68
Q

Name family history risk factors for hypotonia in pediatrics:

A
  • advanced maternal\paternal age
  • consanguinity
  • early infant death - miscarriage
  • delayed developmental milestone

Pediatrics (41 decks)

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