Transcript Treatment

Corticospinal (upper) and neuromuscular
(lower) loss of motor function
Upper:
This include Corticospinal tract &
its neuron up to the anterior horn
cell.
 Loss of voluntary control but
no total loss of movement.
 Posture: arm flexed ,leg
extended.
Tone ↑.
Reflexes ↑.
Babiniski: present.
Atrophy: possible.
Fasciculation: absent.
Lower:
This include AHC , motor root ,
peripheral motor nerve ,
neuromuscular junction &
muscles.
 Total loss of movement.
 Flaccid posture.
 Tone ↓.
 Reflexes ↓.
 Babinski: absent.
 Atrophy: possible.
 Fasciculation: possible.
Stroke in childhood:
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Obstruction of the blood flow may occur in arteries or
veins & may be caused by local thrombosis or
remobilization from distant site.
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Cerebral embolization occur within seconds &
associated with focal seizure , headache &
hemorrhagic infarction & the common sources are the
heart and the carotid artery.
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T I A : it precede the cerebral thrombosis that
produced its full deficits in hrs.
Etiology:
1. Arteriopathy: as
transient
cerebral
arteriopathy
(TCA),
postvaricella
angiopathy, trauma.
2. Cardiac Disease:
 Congenital: (cyanotic ≫ acyanotic)
 Acquired: as catheterization/procedure, arrhythmia, endocarditis …
3. Hematologic
Abnormalities:
as hemoglobinopathies,
thrombocytosis, disorders of coagulation.
polycythemia,
4. Other including metabolic/genetic etiologies:
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Acute systemic illness (e.g., dehydration, sepsis, DKA)
Chronic systemic illness (e.g., systemic lupus erythematosus, leukemia)
Illicit drugs and toxins (e.g., cocaine)
Neurofibromatosis type 1
Homocystinuria
Treatment :
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There is no treatment to repair the neurologic injury
after the stroke, so prevention of future stroke must
be the focus in children if the etiology can be
identified.
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Treatment of strokes in evolution, recurrent TIA, or
ongoing cerebral embolization:
 anticoagulants (heparin, warfarin).
 platelet antiaggregants (aspirin and dipyridamole).
 thrombolysis is not recommended for children
 treat underlying cause.
Disease of the lower motor unit produces
progressive loss of strength with hypotonia and no
abnormality of posture.
Etiology:
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5.
Anterior Horn Cell: as Spinal muscular atrophy( SMA).
Peripheral Nerve: as Guillain-Barré syndrome (GBS).
Neuromuscular Junction: as Myasthenia gravis (MG)
Muscle: as Duchenne (DMD) & dermatomyositis.
Metabolic, Endocrine, and Mineral: as GSDII, thyroid
excess or deficiency, potassium excess or deficiency
(periodic paralysis)
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Etiology:
Progressive degeneration of anterior horn cells is the key manifestation of SMA.
A genetic disease that may begin in intrauterine life or any time thereafter.
3 types
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Type 1: 25% “Werdnig-hoffmann disease (WHD)” severe infantile form.
Type 2: 50% “Kugelberg-Welander syndrome” late infantile slowly progressive form.
Type 3: 25% “Juvenile SMA” chronic form.
Autosomal recessive.
WHD: start as progressive proximal weakness , ↓spontaneous movement ,
floppiness , atrophy of muscles , loss of head control , drooling , ↓ facial expression
,loss of reflex , eyes remain bright open, tongue fasciculation (sleep), normal
mentality , language & sensation
Cause of death: respiratory infection , respiratory failure.
Diagnosis: CPKN/↑, EMGfasiculation, muscle biopsy specimens show
grouped atrophy. The diagnosis is established by DNA probe for SMA.
Treatment: Symptomatic therapy.
Prognosis:
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SMA1: most infants die withen the 1st 2 years.
SMA2: may survive to adulthood.
SMA3: slow progression & normal life expectancy.
 GBS is a postinfectious autoimmune polyneuropathy involving mainly motor
but sometimes also sensory and autonomic nerves.
 This syndrome affects people of all ages.
 The paralysis usually occur about 10 days after respiratory or GIT infecftion
( especially Mycoplasma pneumoniae or Campylobacter jejuni, but also
Helicobacter pylori)
 Symptoms:
 Numbness & parasthesia in the hand and feet then heaveness then weakness
followed by inability to walk in a symmetrical fashion beginning in the legs
and ascending to involve the arms, trunk, throat and the face either in rapid
progression over hrs , days or wks.
 Chronic Guillain-Barré syndrome that recur intermittently or do not improve
for a period of months or years.
 Sign:
Areflexia, hypotonia, minor sensory loss, meningial irritation, bulber &
respiratory insufficiency, normal bladder & bowel function, autonomic
dysfunctions.
Diagnosis:
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CSF: is often normal in the first days of the
illness, then shows elevated protein levels
without significant pleocytosis.
CK: mildly elevated or normal.
NCV: slow.
EMG: denervation.
MRI with gadolinium: may reveal
enhancement of the spinal nerve roots in
>90% of patients.
Treatment :
 Patients in early stages of this acute disease should be
admitted to the hospital for observation because the
ascending paralysis can rapidly involve respiratory
muscles during the next 24 hr.
 Therapy is symptomatic and rehabilitative.
 Rapidly progressive ascending paralysis is treated with
intravenous immunoglobulin (IVIG)
 Plasma exchange and immunosuppressive drugs are
alternative.
Prognosis:
 The clinical course is usually benign,
spontaneous recovery begins within 2–3 wk.
and
 Most patients regain full muscular strength,
although some are left with residual weakness.
 The clinical features which predict poor outcome
with sequelae:
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Cranial nerve involvement.
Intubation.
Maximum disability at the time of presentation.
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Etiology :
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X-linked recessive.
The disease results from absence of a large protein called
dystrophin.
Clinical Manifestations :
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Start at about 2 to 3 years of age.
boys develop an awkward gait and an inability to run properly.
Some have an antecedent history of mild slowness in attaining
motor milestones, such as walking and climbing stairs.
Examination shows:
 calf hypertrophy.
 mild to moderate proximal leg weakness.
 waddling gait and inability to arise from the ground easily. The
child typically arises from a lying position on the floor by using
his arms to "climb up" his legs and body (Gower sign).
 Weakness progress to involve the arm and the child become
confined to wheelchair at 12 yrs.
 Death due to pneumonia or CHF.
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Laboratory and Diagnostic Studies :
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CK level is always markedly elevated.
Blood PCR for the dystrophin gene mutation.
Muscle biopsy specimen shows muscle fiber
degeneration and regeneration.
Cardiac assessment by echocardiography, ECG,
and CXR is essential and should be repeated
periodically.
Treatment :
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Supportive care includes physical therapy,
bracing, proper wheelchairs, and prevention of
scoliosis.
Treatment of cardiac dysfunction & respiratory
infection.
Steroid therapy can delay motor disability.