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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
What are the investigations to order in spinal muscular dystrophy?
1- CK: normal to increased 2- EMG: fasciculation, fibrillation, denervation 3- genetic testing: SMN1 4- muscle biopsy: grouped atrophy
26
What is the risk associated with prenatal SMA? (SMA type 0)
Usually dead at birth or have a life expectancy of <6m.
27
Differentiate between SMA type 1 and type 2?
Type 1 usually occur in <3 month & are unable to sit. | Type 2 usually occur in 3-6 month & are able to sit.
28
In type 3 SMA, the child is able to ......... and unable to .......... :
Able to stand - sit | Unable to walk.
29
In which type of Spinal muscle atrophy is child able to walk assisted an un-assisted?
Assisted: type 3 | Un-assisted: type 4
30
How to treat SMA?
Genetic intrathecal injection of spinaraz | using adenovirus to deliver genetic information
31
What is the most common cause of acute flaccid paralysis in children
GBS
32
What is the etiology of GBS?
Immune mediated
33
What is a key word to look for in a case of GBS?
The patient developed GI (campylobacter) or respiratory (mycoplasma pneumoni) infection 10 days prior to symptoms
34
What is the most common form of GBS?
AIDP | “Acute inflammatory demylinating polyriduclopathy”
35
What is the classical presentation of GBS?
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
What is the miler fisher variant of GBS?
Ataxia - partial ophthalmoplegia - areflexia
37
What are the findings in physical examination of GBS?
- symmetrical weakness - diminished\absent reflex - gait abnormalities
38
How to treat GBS?
IVIG or plasma exchange
39
What is the organism responsible for developing infantile botulism?
Clostridium botulinum
40
What is the risk of infantile botulism?
Life threatning neuroparalytic syndrome
41
How does clostridium cause infantile botulism?
Blocks (pre-synaptic) chloinergic transmission at NMJ
42
Differentiate between the pattern of spread of weakness in infantile botulism vs GBS?
GBS: ascending Infantile: descending
43
What is the clinical presentation of infantile botulism
- constipation\urinary retention - hypotonia\weakness - cranial nerve dysfunction - poor feeding
44
What is the cranial nerve dysfunction in infantile botulism?
decreased 1- gag reflex 2- eye movement - pupillary contraction - ptosis.
45
Where do infants get the infection of clostridium botulinum?
Spores in: - canned food - honey - soil and dust
46
What is the mode of inheretence for Duchenne muscular dystrophy?
X-linked recessive
47
What is Duchenne muscular dystrophy?
Progressive myofiber degeneration adn replacement by fibrotic tissue
48
What is the age that commonly affects duchenne muscular dystrophy?
2-3 years of age.
49
What special test to preform in patients with DMD and what to expect as positive finding
Asking patient to stand from ground [Gower’s sign] (Using hands to push on legs); because it’s proximal muscle weakness
50
What movement are most difficult for DMD patients to preform?
Running, jumping, walking up the stairs
51
What is the investigation of choice for DMD?
- Serum CK (10-20x ULN) - DMD genetic testinh
52
What is the course of illness in DMD?
- by 13: wheelchair | - by 20s: death (respiratory\CVD)
53
What is the role of muscle biopsy in DMD?
Has no role, only showing dystrophy which is present in many other conditions
54
What is the clinical presentation of DMD?
1- psudohypertrophy of calf + short achiles 2- scoliosis + lordosis 3- hyporeflexia + hypotonia 4- waddling gait.
55
What is the sign seen in hypotonic patients with vertical suspension
Axillary slippage
56
How is the tone in UMNL?
Spastic + clonus
57
How is the deep tendon reflex, fasculations, wasting, planter response in UMNL
- hyperreflexia - no fasculations - late wasting - upgoing toes
58
How is the deep tendon reflex, fasculations, wasting, planter response in LMNL
- hyporeflexia - present in ant. Horn cell lesion (SMA) - early wasint - normal response
59
Name central causes of hypotonia:
1- Hypoxic ischemic encephalopathy HIE 2- structural brain abnormalities & genetic disorder 3- down syndrome 4- prader-willi syndrome
60
What is more common central and peripheral hypotonia
Central
61
Name peripheral causes of hypotonia:
- SMA - congenital myopathy - myasthenia - myotonic dystrophy
62
What is krabbe disease
Genetic disease affecting white matter
63
Name clinical features of central hypotonia:
- Seizure & abnormal consciousness - preserved power and reflex. - axial weakness - dysmorphism
64
Name clinical features of peripheral hypotonia:
- alert - absent reflex and movement - weakness against gravity - deformities of bone\joint + Fasiculations
65
What are the signs of hypotonia?
- scarf sign - frog leg - head lag - vertical: axillary slip - ventral: Inverted U shape - flat in prone position - poor crying, swallowing, coughing - abdominal breathing
66
What are the maternal infections that may be associated with hypotonia
TORCH - Toxoplasmosis - Other: coxakie, chickebpox, HIV - Rubella - CMV - Herpes simplex
67
Name maternal risk factors for hypotonia in pediatrics
- infection - polyhydamnios - diseases (SLE, MG, DM, epilepsy) - medics - breech
68
Name family history risk factors for hypotonia in pediatrics:
- advanced maternal\paternal age - consanguinity - early infant death - miscarriage - delayed developmental milestone

Pediatrics (41 decks)

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