Azhar Medicine Assiut

Pediatric Department

FLACCID
PARALYSIS
Group (7) Boys

Under Supervision
Prof. Dr. Hosny El-Masry
‫)‪Group 7 Boys (67-77‬‬
‫محمد جمال حسن جاد هللا‬ ‫‪67‬‬
‫محمد رافت محمد عشري‬ ‫‪68‬‬
‫محمد شرف الدين محمد‬ ‫‪69‬‬
‫محمد عبد الباسط محمود‬ ‫‪70‬‬
‫محمد عبد الفتاح محمد مرسي‬ ‫‪71‬‬
‫محمد عيد محمد علي‬ ‫‪72‬‬
‫محمد ماهر عبد الشكور‬ ‫‪73‬‬
‫محمد ماهر محمد عبد العال‬ ‫‪74‬‬
‫محمد مجاور مهدي‬ ‫‪75‬‬
‫محمد مجدي سيد‬ ‫‪76‬‬
‫محمد محمد ابو العال عبد العال‬ ‫‪77‬‬
DEFINITION
Rapid-onset weakness and
paralysis in one or more limbs
most commonly occurs in young
children.
It is a lower motor neuron lesion
(LMNL)
 Causes
1. Cerebral:
strokes e.g. acute hemiplegia
2. Spinal cord:
a. Transverse myelitis
b. Spinal cord trauma as in traffic road accidents, compression by abscess or
tumors
3. Anterior horn cells:
a. poliomyelitis: asymmetric ascending paralysis
b. motor neuron diseases: spinal muscular atrophy
4.Peripheral nerves
a. gullian barre syndrome ( the commonest cause): (symmetric ascending paralysis(
b. post diphtheritic paralysis : symmetric descending
 Causes......
5. Neuromuscular:
a. botulism: symmetric descending paralysis
b. tick-bite paralysis: symmetric ascending paralysis
c. insecticide: organophosphorus
d. myasthenia gravis: symmetric descending
6.Muscular:
a. familial periodic paralysis (hypokalemic, hyperkalemic)
b. inflammatory myopathy
7. Others:
a. rabies, tetanus, and porphyria
b. pseudoparalysis: Bone, joint diseases e.g.: osteomyelitis,
fractures, scurvy
Guillain Barre
syndrome
Definition
Auto immune, often acute postinfectious polyneuropathy
involving mainly motor but also sensory and sometimes
autonomic nerves , characterized by ascending weakness,
hypo or areflexia & distal paresthesia

Incidence
It is the most common cause of acute paralysis in children
Affects any age but most cases are seen in children above the
age of 3 years.
Etiology & Pathogenesis
•It follows GIT or URT infection within 1-4 wks. before onset of paralysis:
bacterial infections e.g. campylobacter jejuni ,H pylori ,mycoplasma
viral infections e.g CMV ,EBV
post vaccination

•Manifestations are due to post infectious demyelination:

immune responses to non self antigen (infectious agents & vaccines) misdirected
to host(self) nerve tissue due to molecular mimicry
Clinical picture
1- History :
preceding infection or vaccination as respiratory or GIT infection few
weeks before the onset of paralysis
2- Neurological examination
1. Motor : Acute ascending flaccid paralysis: Acute onset and usually regressive course
Criteria:
- Bilateral & symmetric usually (asymmetric in 9%)
- Proximal(contrary to other types of polyneuropathy)
- Associated hypotonia & hyporeflexia or areflexia (LMNL)
Progress: within hours or days
- Lower Limb (inability or refusal to walk)>> trunk >> upper limb.
- Bulbar palsy (in 50%) >> dysphonia, dysphagia & lost bulbar reflexes.
- Respiratory muscles leads to paradoxical breathing (seesaw abdominal
movements)→ respiratory failure.
- Miller-Fisher syndrome(polyneuritis cranialis) consists of Triad: acute external
ophthalmoplegia, ataxia, and areflexia
Clinical picture
2- Neurological examination
2. Sensory
- absent or mild in the form of distal parasthesia (glove & stock hypothesia)
- Tender calf
3. Autonomic (Dysautonomia)
- Labile blood pressure & heart rate
- Urinary incontinence or retention of urine in about 20%
4. Cranial nerves (7,10):
bilateral facial paralysis (Facial diplegia) and bulbar palsy.
SEESAW BREATHING
Prognosis
- The clinical course is usually benign, and spontaneous complete recovery begins within 2-3
wk.

- Most patients (75%) regain full muscular strength, although some are left with residual
weakness(15%)

- Improvement usually follows a gradient opposite the direction of involvement: bulbar

function
recovering first, and lower extremity weakness resolving last

- The mortality rate is 5%, and death is caused by:

. autonomic dysfunction (hyper-hypotension,tachy-bradycardia, and sudden death)
. Aspiration pneumonia, respiratory failure
. complications of mechanical ventilation
. cardiovascular collapse
. pulmonary embolism
Investigation
Diagnosis is mainly clinical
1- CSF:
(2 wks. after the onset of paralysis; because it is often normal in the first days of the illness)
Cyto-albuminous dissociation :
-Increased protein but with normal cell count
- Negative bacterial culture

2- MRI of the spinal cord

- Thickening of the cauda equina and intrathecal nerve roots in >90% of patients
- Rule out other spinal disorders
3- NCS: Motor nerve conduction velocity are greatly reduced(diagnostic)

4- EMG: Electromyography shows evidence of acute denervation of muscle(reduced amplitude).

5- Serum creatine kinase (CK) level may be mildly elevated or normal

Treatment
Patients in early stages of this acute disease should be admitted to the
hospital(ICU) for observation because the ascending paralysis can rapidly
involve respiratory muscles during the next 24 hr.
1. Supportive:
a. Respiratory effort monitoring (spirometry) and support ; Mechanical ventilation
for patients
presenting with respiratory and bulbar paralysis (lifesaving)
b. Cardiac monitoring
c. Nasogastric feeding
d. Care of bladder (catheterization & neostigmine).
e. Physiotherapy

2- Specific
A. IVIG in all patients for 5 successive days . The best choice
B. Plasmapheresis
C. Steroids aren't effective
D.D.
- other causes of acute flaccid paralysis and other causes of polyneuropathy....

• This polyneuropathy can be difficult to distinguish from an acute spinal cord

syndrome

• Preservation of bowel and bladder function, loss of arm reflexes(areflexia),

absence of a sensory level, and lack of spinal tenderness  Guillain-Barré
syndrome.
Poliomyelitis
Incidence
ln Egypt, it is almost eradicated with the compulsory vaccination

Causes
RNA enterovirus
Acute viral infection of the AHCs
Transmission : feco-oral or droplet infection
Predisposing factor :
- Steroids - tonsillectomy
- Extremes of age - IM injection
- trauma to the muscle
Clinical types
1-Subclinical infection
2-Abortive poliomyelitis (minor illness)
3-Non-paralytic poliomyelitis
4-Paralytic poliomyelitis: acute asymmetric ascending paralysis:
Asymmetric patchy LMNL (hypotonia, hyporeflexia, wasting)
- in some patient, bulbar, respiratory paralysis may occur
5-Encephalitic poliomyelitis
Diagnosis
viral isolation from stool or throat
D.D.
Other causes of acute flaccid paralysis

Management
Prevention: polio vaccine
Treatment: Supportive
- Acute stage: Isolation and ttt of bulbar and respiratory affection
- Analgesics(avoid injections)
- Physiotherapy, orthopedic consultation
Postdiphtheric
paralysis
This form of toxic polyneuritis is uncommon nowadays because of the routine
vaccination.
The exotoxin of corynebacterium diphtheria has an affinity for the nerves

History of preceding sore throat and swollen neck (pharyngeal diphtheria) or

stridor (laryngeal diphtheria), 1 - 2 week before the onset of paralysis can be
obtained.

A. Paralysis is usually descending. Ocular and bulbar paralysis (3,7,10 Cr n affection )is
the initial
presentation which can be followed by peripheral paralysis of limbs
after one or 2 weeks. Paralysis is characteristically purely motor and
transient.
B. The course is benign and complete recovery occurs over few weeks.
• The initial bulbar involvement, absence of sensory involvement can differentiate the
condition from Guillain Barre syndrome.

Treatment
• Antidiphtheritic serum I.M
• Antibiotics & cardiac support
Botulism
 Botulism
It is a toxic neuromuscular blockade caused by Clostridium
botulinum (anaerobic gram positive bacteria).
According to the type of
infection, 3 forms are present:
(1) infant botulism :occurs in infants. Germination of spores in gastrointestinal
tract follows exposure to soil, house dust, honey or corn syrup;
(2) food-home botulism : occurs with ingestion of improperly home-
preserved foods containing the toxins.
Outbreaks usually occur from canned or undercooked food as fish and meat
(3) Wound botulism : occurs due to wound contamination with the Clostridium
botulinum organisms.
clinical pictiure
A. Paralysis is acute, symmetric and descending. It starts in bulbar nerves then
descends over a period of few hours or few days to involve the trunk and limbs.
Respiratory paralysis is common and usually necessitates prolonged mechanical
ventilation.
Paralysis is purely motor with no sensory involvement.

B. Affected patients are usually alert, afebrile with dry mucous membranes of mouth,
tongue and pharynx. Lacrimation is also decreased.
The course of illness is usually prolonged over several weeks.
Prolonged mechanical ventilation for months is not unusual(common).
 INESTIGATIONS

• CSF and nerve conduction velocity are normal (important differentiating points
from atypical descending Guillain Barre syndrome).
• The most important diagnostic investigation is the electromyography which
demonstrates the characteristic brief, small, abundant motor-unit action potentials
(BSAP).
• Also The diagnosis is established by demonstrating the presence of C botulinum
organisms or toxin in stool.
 TREATMENT

• Antitoxin administration
• Supportive: Mechanical ventilation, parenteral nutrition
• Elimination: induced vomiting, high enema
Transverse
myelitis
It is the second most common cause of acute symmetric paralysis.
It is a segmental dysfunction of the spinal cord without an evidence of spinal cord
compression.
The cause : is probably either a direct viral infection or an autoimmune disease.

A. Paralysis is acute, symmetric and usually involving only the lower limbs (paraplegia).
In rare situations where the lesion is above the 5th cervical segment, involvement of upper
limbs also occurs (quadriplegia).
Initially, paralysis is flaccid(shock stage) but gradual change to spasticity occurs over
few weeks (2-6 wks).

B. Sensory loss (with sensory level on the trunk) and autonomic disturbance (urinary
retention and stool incontinence) are usually present.
C. The course is usually prolonged over several months.
• Complete recovery occurs in only 60% of cases.

D. Myelography is important for differentiation from acute spinal cord compression. It is

normal in transverse myelitis.

• CT, MRI is more sensitive

• Treatment:
-high dose steroids
Acute spinal cord
compression
Causes:
➤Trauma to the back
➤spinal epidural abscess
➤ vascular anomalies of the cord may produce a picture which can not be clinically
differentiated from
transverse myelitis.
➤Occasionally, spinal cord tumors may also present with acute paralysis.
The hallmarks of spinal cord disease are:
• 1. sensory level
• 2. motor level
• 3. disturbance of bowel and bladder function
• 4. local spinal pain or tenderness

• Myelography is essential to demonstrate the obstruction.

• CT scan of the spine or MRI are more sensitive and can show the nature of
obstruction.

• Management: Trauma and tumors necessitate immediate neurosurgical

management to preserve vital function
MOVEMENT DISORDERS
In acute cerbellar ataxia: hypotonia is usually present and may be
prominent but the truncal ataxia with unsteady gait is the most
characteristic feature.

In rheumatic chorea: hypotonia is present and may be marked

simulating acute paralysis but abnormal involuntary choreic
movements is the dominating finding.
SPINAL MUSCLE ATROPHY
DEFINITION
Hereditary disease characterized by progressive hypotonia and muscular weakness
due to degeneration of the anterior horn cells

Incidence: the most common cause of floppy infant

Etiology

• • Transmission: autosomal recessive (gene in chromosome N 5)

• • There is progressive degeneration of the anterior horn cells due to apoptosis.

 TYPES OF SMA

SMA type 0 (fatal in the perinatal period)

SMA type 2 (slowly progressive with delayed onset)

SMA type 3 (the mildest as patients are ambulatory

 CLINICAL PICTURE OF SMA TYPE 1
(WERDING HOFFMANN DISEASE)
a. Intrauterine: diminished fetal movements are often noticed during pregnancy

b. At birth

a. Arthrogryposis (positional deformities of the limbs)

b. Floppy infant.
c. Later on

1. Motor system

- State: muscle wasting and fasciculations (worm like movement: best seen in the tongue:
characteristic clinical sign).

- Power and tone: severe weakness and severe hypotonia

2. Reflexes: absent tendon reflexes

3. Cranial nerves: bulbar palsy →Weak cough and weak cry

4. Normal mentality and normal eye movement.

5. Late respiratory paralysis →death in late infancy from respiratory failure

 Investigations

1. Electromyography
2. Genetic Study

Treatment
(supportive only)

• 1. Pulmonary complications (treatment of chest infection and pulmonary

ventilation)
• 2. Nasogastric feeding may be needed
• 3. Physiotherapy
• 4. New emerging therapies are now evolving based on the genetic testing
(Gene therapy; Nusinersen)
QUESTIONS
 WRITTEN QUESTIONS

1- Enumerate causes of acute flaccid paralysis.

 CASE
1- About 12 days after a mild upper respiratory infection, a 12-year-old boy complains of weakness
in his lower extremities. Over several days, the weakness progresses to include his trunk. On
physical examination, he has the weakness described and no lower extremity deep tendon reflexes,
muscle atrophy, or pain. Spinal fluid studies are notable for elevated protein only.
What is the most likely diagnosis in this case?
What are the investigations required for this case?
What is the best line of treatment?
 KAY ANSWER
• Diagnosis: Guillain Barre syndrome
• Investigation
- MRI: Thickening of the cauda equnia and intrathecal nerve roots
- CSF: cytoalbuminous dissociation
- NCS: ↓conduction velocity is diagnostic
• Best line of treatment
- The best choice: IVIG
- mechanical ventilation if bulbar or respiratory affection
MCQS
1-A 16-year-old female presents with leg weakness after recovering from an upper respiratory illness. On
examination her vital signs are normal. She is unable to stand alone. Motor strength is 5/5 in the arms and 2/5 in the
legs. Deep tendon reflexes are absent in the legs.

(a)- What finding is classically associated with this illness?

(A) hydrocephalus

(B) elevated serum C-reactive protein

(C) myoglobulinuria

(D) elevated cerebrospinal fluid protein

(E) presence of Clostridium species on stool culture

(b)- Testing demonstrates marked slowing of nerve conduction velocity. What would the most appropriate
intervention be at this time?

(A) administration of intravenous immune globulin

(B) discharge and reassurance about the overall benign nature of this disease

(C) administration of intravenous fresh frozen plasma

(D) supplemental oxygen via nasal cannula

(E) administration of intravenous interferon-beta

2- The feeding of honey to infants less than 6 months of age has been associated with which of
the following?
(A) anaphylaxis
(B) hypernatremia

(C) botulism
(D) jaundice
(E) listeriosis

3- A child who presents with acute flaccid symmetrical descending paralysis is likely
having:
a. Poliomyelitis.
b. GBS

c. Post-diphtheritic paralysis
d. Transverse myelitis
4- A previously healthy 3-month-old infant develops generalized weakness with
difficulty in sucking, swallowing, and crying, and labored breathing. No fever is present.
Which study will most likely provide the diagnosis?
• (A) stool culture
• (B) blood culture
• (C) head CT-scan
• (D) nerve conduction velocity testing
• (E) cerebrospinal fluid analysis
5- which of the following is characteristic of GBS?
• a. paralysis is descending
• b.Degeneration of the anterior horn cells
• c. Loss of motor function within weeks.
• d. The CSF examination 2 weeks after paralysis shows increased proteins.
6- Manifestations of post-diphtheritic palatal and pharyngeal paralysis include all of the
following except:
• a) Difficult swallowing
• b) Exaggerated jaw reflex
• c) Absent palatal and pharyngeal reflexes
• d) usally occurs on the 3rd week of the disease
• e) Nasal tone of speech and nasal regurge of fluids
7- In Guillain-Barre syndrome all are true except:
• a) Pattern of paralysis is usually ascending
• b) Paralysis is flaccid
• c) Absent deep tendon reflexe
• d) Sensory loss of glove and stocking distribution
• e) CSF shows increased neutrophils and normal protein level
8- The following investigations can help confirm the diagnosis of Guillain barre
syndrome except:
• a) CT brain
• b) CSF analysis
• c) Nerve biopsy
• d) MRI of the cauda equina region
• e) Nerve conduction study

9- Werdnig-Hoffmann is characterized by all of the following except:

• a. X-linked recessive disorder.
• b. Respiratory paralysis.
• c. Tongue fasciculations.
• d. Normal mentality.
10- An infant with severe hypotonia due to Werdnig-Hoffman disease has:
• a) Normal deep tendon reflexes
• b) Seizures
• c) Fasciculations of the tongue
• d) Recurrent fevers
• e) Atrophy of the optic nerve
11- One of the following is not a feature of Warding-Hoffman disease:
• a) Progressive hypotonia
• b) Muscle wasting
• c) Mental retardation
• d) Chocking and dysphagia
12- Predisposing factors for paralysis in poliomyelitis include:
• a) Intramuscular injection
• b) Steroid therapy
• c) Tonsillectomy
• d) Trauma to the muscle.
• e) All of the above
13- Guillain-Barre syndrome (Post infectious polyneuropathy) is manifested by:
• a) Acute onset and usually regressive course
• b) Flaccid paralysis in an ascending manner
• c) Bilateral symmetrical distribution
• d) Hypotonia and hyporeflexia
• e) All of the above
 KAY ANSWER
1.(a)→d
1.(b)→a
2→c
3→c
4→a
5→d
6→b
7→e
8→a
9→a
10→c
11→c
12→e 13→e
THANK OU