Description about immuno mediated disorders

1. Immune Mediated Disorders
Dr. Sunanda Bhowmik(PT)
Assistant Professor
MMIPR,MMDU

2. INTRODUCTION
• Guillain-Barre syndrome is an immune mediated disorder of
peripheral nervous system of acute or sub acute onset,
• It is most commonly characterized by combination of generalized
weakness paresthesias of limbs, and areflexias.
• The reported incidence of GBS is 1 to 2 per 100,000 populations
• Since the marked decline in poliomyelitis incidence, GBS has
become the most common cause of acute flaccid paralysis in
children.

3. • GBS, reported worldwide, involves both genders and all ages
• The incidence for children under 15 years being similar to that of
adults.
• it predominantly affects children aged 1- 5 years
• There is a slight male predominance with male to female ratio of
1.5:1
• The motor axonal form of GBS occurs in epidemics during
summer in northern China and predominantly affects children
probably by campylobacter jejuni infection

4. Approximately half to two thirds of patients are reported to have a
preceding illness, most commonly upper respiratory tract infection or
gastroenteritis, within 4 weeks period before the onset of the GBS
symptoms.

5. PATHOPHYSIOLOGY
• The GBS is believed to be an autoimmune disorder based
on T-cell activation with production of antibodies.
• Infectious agents such as Epstein-Barr virus,
cytomegalovirus, mycoplasma pneumoniae and
campylobacter jejuni, immunizations, surgery may trigger
formation of such antibodies.

6. • The primary pathologic process of this condition is axonal injury in
many cases
• Specific nerve antigens have been implicated in a number subtypes of
GBS(Miller-Fisher syndrome) in axolemmal membrane of nerves
name GQ1b, a glycolipid.
• The muscle weakness in GBS results from conduction block and
concomitant or primary axonal injury in the affected motor nerves.
• Pain and paresthesias are the clinical correlates of sensory nerve
involvement.

7. • Axonal GBS after campylobacter jejuni infection is usually
mediated by antibodies to the GM1 ganglioside.
• Antibody-mediated demyelination leads to mononuclear infiltrates
around the endoneurial vessels and vesicular myelin degeneration.
• This demyelination may be discrete or diffuse and may affect the
peripheral nerves at any point from their origin in the spinal cord to
the neuromuscular junction

8. CLINICAL FEATURES
• progressive muscle weakness, typically starts in the lower extremities and
ascends into the upper within days to weeks.
• Diminished deep tendon reflexes
• weakness is primarily proximal; fifty percent of affected children reach
the nadir of weakness by one week, 80% by 2 weeks, and over 90% by 3
weeks
• All children with uncomplicated GBS will reach their maximum level of
weakness by 4 weeks from onset
• Sensory disturbances are also quite common, as are neuropathic pain,
dysesthesia
• Back, buttock or leg pain due to PNS inflammation

9. The diagnosis of GBS should always be considered in small children
presenting with pain and decreased movement or refusal to bear
weight and when testable, position and vibratory sensations are
diminished.
The weakness, sensory loss, and pain, whether alone or together, often
present as a gait disturbance with an ataxic component
Some cases of pediatric GBS present as the Miller-Fisher variants of
ataxia, ophthalmoplegia and areflexia without peripheral weakness.

10. • Cranial nerve involvement with facial weakness, difficulty in
swallowing, and occasionally involvement of the cranial nerves
that control ocular movements.
• Presentations of autonomic involvement are disturbances of
sweating, heart rate and rhythm, blood pressure control, sphincter,
visceral and pupillary function

11. STAGES
GBS has three stages
• Progressive phase lasting over several days to several weeks,
• Plateau phase of similar duration,
• Recovery phase over weeks to months.
• The major complications occur in the first phase which does not
persist beyond 4 weeks.

12. THANK YOU

13. TRANSVERSE MYELITIS

15. INTRODUCTION
Transverse myelitis (TM) is a rare, acquired focal inflammatory
disorder often presenting with rapid onset weakness, sensory deficits,
and bowel/bladder dysfunction.
TM generally occurs around the spinal cord at any level, but most
commonly affects the thoracic region
The disorder transverses the spinal cord causing bilateral deficiencies
The duration of this disease may be as little as 3 to 6 months or may
become permanently debilitating.

16. Reasons
• The diagnosis may incorporate the terms idiopathic, where no
specific bacterial, viral, or other obvious inflammatory cause can
be found.
• Secondary, where there is an antecedent or associated disease.
• Other commonly encountered descriptors include acute, acute
partial, acute complete, and longitudinally extensive

17. ETIOLOGY
There are multiple causes of transverse myelitis, but can they be
broadly divided into idiopathic, post infectious, systemic
inflammation, or multifocal central nervous system disease
The most common cause of TM is idiopathic
TM can affect men and women equally. Women tend to predominate
those associated with multiple sclerosis
TM can affect patients of all ages, but it has spiked occurrences
around the ages 10, 20, and over 40.

18. Classically, the majority of cases were characterized by perivascular
infiltration, demyelination
axonal injury by monocytes and lymphocytes at the lesion site.
Heterogeneity, along with both gray and white matter involvement,
gives evidence that this is not a pure demyelinating disorder.
TM may, in fact, be a mixed inflammatory disorder involving
neurons, axons, oligodendrocytes, and myelin.
Alternative histopathologic causes of TM have been reported to
include molecular mimicry and super antigen-mediated disease
associated with autoimmune causes

20. SYMPTOMS
• Weakness of the limbs, Weakness may affect only the lower limbs
or all four limbs with varying severity. It may be complete,
incomplete, or may present as one of the spinal cord syndromes
• sensory impairments, pain, and difficulties with the bowel and
bladder.
• Sensory complaints may include hypersensitivity, numbness,
tingling, coldness, burning, or as a circumferential constriction.
• Pain is a common symptom in one third to one half of patients
and may be central or localized, aching or radicular in character.

21. Physical Examination
• motor weakness,
• changes in sensation (pinprick, light touch,
vibration, position sense, or temperature),
• tone,
• muscle stretch reflexes,
• coordination,
• bowel and bladder functioning,
• cognitive dysfunction
• cranial nerve and visual abnormalities, (generally
not seen with idiopathic TM)

22. • Fever, tachycardia, and tachypnea may indicate
an infectious etiology.
• Scales: Gross muscle strength, measured with the
Oxford grading system,
• VAS,NPRS
• Ashworth grading scale
• FIM
• DTR

23. DIAGNOSIS
To diagnose transverse myelitis, a compressive cord lesion must be
excluded first.
Exclusion is usually performed by magnetic resonance imaging (MRI).
This is followed by a confirmation of inflammation either by a
gadolinium-enhanced MRI or lumbar puncture (LP)

24. • Diagnostic criteria include:
• Sensory, motor, or autonomic dysfunction originating from the
spinal cord
• T2 hyperintense signal changes on MRI
• No evidence of a compressive lesion
• Bilateral signs/symptoms.
• Clearly defined sensory level.
• Evidence of inflammatory process demonstrated by gadolinium
enhancement on MRI, cerebrospinal fluid (CSF) analysis showing
pleocytosis, or elevated immunoglobulin G (IgG) index.
• Progression to nadir between 4 hours and 21 days

25. PROGNOSIS
• Most patients with idiopathic transverse myelitis should at least
have a partial recovery.
• This recovery should begin within 1 to 3 months, If there is no
recovery within the first 3 to 6 months, then recovery is unlikely.
• Rapid onset with complete paraplegia and spinal shock is
associated with poorer prognosis.
• Recovery may take years, and some degree of persistent
debilitation may exist.

26. PROBLEM LIST
• Lower extremity (LE) numbness and tingling
• Weakness in bilateral anterior LE and left thigh
posteriorly down to toes,
• Pain in left lower back when sleeping in a supine
• patient fatigued very easily

27. SHORT TERM GOALS
• reduce pain
• frequent muscle spasms
• normalize muscle tone
• improve joint range of motion

28. LONG TERM GOALS
• Strengthen the muscle ROM
• To restore functional independence