Cidp Presentation

8/11/2019 Cidp Presentation

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A 62 yr old patient from Akmeemana presenting with

limb weakness


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HistoryP/C

Progressive weakness of LL for 6 wks &Progressive weakness of UL for 2 wks

H/P/CWell 5 months backDeveloped upper abdominal pain

Episodic & lasted for 3 months Ass. with nausea, vomiting, & constipationTreatment taken but poor responseGradually subsided

Developed LL & UL weaknessGradual onset

Ascending type & symmetricalCalf thigh hand

Ass. with paraesthesiae ( pins & needles ), unsteady gait


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H/P/C ctd.No urinary incontinence / retention

No double visionSpeech normalNo bulbar symptomsNo headache or fits

No feverNo SOB, coughNo palpitations or fainting attacksNo diarrhoea or constipation


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P/M/H

Nothing significantNo similar illness

F/HNo significant illnesses

Drug history Antacid medication

S/HFarmer

UnmarriedOcc. AlcoholicNo exposure to pesticides or other toxinsDiet- adequate


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Examination

GeneralNot dyspnoeic

AfebrileNot pale, not cyanosed & anictericNo clubbingNo lymphadenopathyNo skin rashes or ulcers

No fasciculations

CVS/ Res / Abd NAD


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CNSHigher functions normalCranial nerves- B/L mild facial weaknessMotor

Wasting of calf & thigh muscles & small muscles of handsNo fasciculationsTone reduced in LL & distal ULPower grade 1 in LL & grade 2 distally in ULReflexes B/L BJ & TJ reduced , others absentPlantar down

SensoryTouch, JPS & vibration absentPain & temp. present

Fundus NADSingle breath count- 24


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Summary

A 62 yr. old farmer presents with gradual onsetof ascending type of progressive limb weaknessof 6wks. duration, preceded by a significantabdominal pain. On examination he hassymmetrical motor weakness of both upper &lower limbs, hyporeflexia & areflexia, -veBabinski response & mild B/L facial weaknesswithout bulbar or respiratory muscleinvolvement. Sensory system examinationrevealed impaired touch, JPS & vibrationsensation with intact pain & temp. andautonomic functions.


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DDCIDPGBSParaneoplasticPorphyria

Toxins Pb / As


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InvestigationsFBC

WBC- 7.3x10 /mm3Hb%- 13.5 g/dlPlt.- 375,000

BP - normalFBS - 5.2 mmol/lESR - 1 st hr 12mmLFT - normalRFT - normalSE - Na- 142 , K- 4.3 mmol/lS.Ca - 9.1 mg/dlS. protien- Total-72g/l Alb-43 g/lCXR NADUSS - NADUrinary PBG - Negative


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CSFProtein- 184mg/dlCells - polymorphs-0 lymphocytes-0Sugar 5 mmol/l


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NCSDL NCV Amp CMAP

AreaCMAPDura.

L/ulnar 6.2 32.9 P 2.5D 0.6

P 12.4D 3.5

P 8.7D 9.5

R/ulnar 5.7 38 P 2.5D 0.5

P 10.0D 2.7

P 7.4D 9.4

R/med. 10.6 33.9 P 1.1

D 2.8

P 4.8

D 4.3

P 8.1

D 8.7


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Analysis of NCS

Distal latency prolongation >130%

L/ULN DL 6.2/3.3=1.88=188%

R/ULN- DL 5.7/3.3=1.73=173%DL is prolonged


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Analysis of NCS

Conduction velocity slowing < 75%

L/ULN-CV 32.9/49=0.67 = 67%R/ULN-CV 38/49 = 0.78 = 78%

R/MN

CV 33.9/49 = 0.69 = 69%

CV is slow


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Conduction block & temporal dispersion isfound in all 3 nerves.

CB = proximal/distal CMAP area ratio 1.15

F response ( prolonged late response) not measured


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Chronic inflammatorydemyelinating polyneuropathy

CIDP


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Outline

General Clinical Manifestations Diagnosis

Treatment and prognosis


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CIDP

Acquired, immune-mediated polyradiculoneuropathy

Heterogeneous disorder with a wide range of clinical

expression ranging from subacute to a progressive orrelapsing-remitting course

Diagnosis is based on

clinical symptoms and signs,electrodiagnostic studies, CSF examination, and other laboratory tests


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CIDP: Incidence

In one study, prevalence was estimated to range from 1-7.7 per 100,000

These are likely underestimates, since the criteria toselect cases were strict

CIDP accounted for 13% of patients seen in oneneuromuscular center

Incidence increases with increasing ageChildren are rarely affected


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CIDP: Pathophysiology

In one study, CIDP occurred within a few weeksafter an infectious event in 16% of the patients

Because of the insidious onset, documenting

precipitating illnesses or events is very difficultBoth respiratory and gastrointestinal infectionshave been cited, but no causative organism hasbeen identifiedWith Guillain-Barre syndome, the most commonpreceding infection is Campylobacter


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CIDP: Clinical Manifestations

Slow progressive course in approximately 2/3 of cases

Relapsing course with partial or complete recoverybetween recurrences is seen in approximately 1/3 of cases

Periods of worsening and improvement usually lastweeks or monthsYoung patients tend to have a higher frequency of

relapsing course


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CIDP: Regional Variants

The classical phenotype that suggests CIDP is thepresence of proximal and distal weakness, with large fibersensory loss and areflexia

Few patients present with classic symtpoms

Subclassifications based on the clinical phenotype in orderto aid in diagnosis and treatment

Currently these subclasses are not thought to be distinctdiseases


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CIDP: Associated Conditions

Most frequently CIDP is an idiopathic illness

Has been known to occur with several conditions

The associated condition is included in the maindiagnosis to separate those cases from the idiopathicvariety

Example: CIDP with HIV infection


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MGUSEvidence suggests that CIDP with IgM MGUS hasspecific clinical and electrophysiologic characteristics

Usually predominance of distal weakness withsensory symptoms greater than motor

Multiple sclerosisReports describe CNS white matter changes in

patients with CIDPWhether a true association exists between CIDP and

multiple sclerosis remains unclear


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Systemic lupus erythematosus

Chronic active hepatitis (B or C)CIDP associated with hepatitis should be differentiatedfrom cryoglobulinemic vasculitisThe latter causes either symmetric distal sensorimotorpolyneuropathy or mononeuropathy multiplex but on

pathologic examination shows wallerian degenerationand not the segmental demyelination seen in CIDP


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Inflammatory bowel diseaseCrohn disease and other inflammatory bowel conditions,although no direct correlation between the two afflictionsis knownThe mechanism an autoimmune abnormality?


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Diabetes mellitusIncreasing evidence supports the suggestion that somepatients with diabetes who have severe neuropathy orunusually progressive neuropathy may have CIDP

superimposed on their diabetic disorderDiabetes may predispose patients to CIDP

Pregnancy

Known to worsen CIDPWorsening usually occurs in the third trimester or in

the postpartum period


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CIDP: Diagnosis

Because of the clinical heterogeneity and the lack of adiagnostic test, various diagnostic criteria have beenproposed

In one series of patients all of whom had proximal anddistal weakness and in whom 95% of patients hadimprovement with treatment, only 30% had the classictriad of slow nerve conduction velocity, elevated CSFprotein and demyelination on nerve biopsy


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American Association of Neurology: Criteria

Developed criteria for the identification of patients with CIDP forresearch studies

Pathologic criteria

Electrophysiologic criteria:Require 3 demyelinating range abnormalities in motor nerves1. Slow conduction velocity,2. Prolonged distal motor latencies ,3. F wave latencies or4. Conduction block in 2 nerves


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CIDP: EMG

Critical test to determine whether the disorder is truly a peripheralneuropathy and whether the neuropathy is demyelinating

Findings of a demyelinating neuropathyMultifocal conduction block or temporal dispersion of compound

muscle action potentialProlonged distal latenciesConduction velocity slowing to less than 75% of normal

Absent or prolonged F wave latencies

As the disease progresses, patients tend to developsecondary axonal degeneration


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CIDP: Peripheral nerve biopsy

IndicationsPatients in whom the diagnosis is not completely clearCases where other causes of neuropathy (eg,hereditary, vasculitic) cannot be excludedCases where profound axonal involvement is observedon EMGSome experts recommend biopsy for most patients priorto initiating immunosuppressive therapy


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CIDP: Histologic Findings

Interstitial and perivascular infiltration of theendoneurium with inflammatory T cells andmacrophages with local edema

Evidence exists of segmental demyelination andremyelination with occasional onion bulbformation, particularly in relapsing cases

Some evidence of axonal damage also isobserved, with loss of myelinated nerve fibers

The inflammatory infiltrate with neutrophilinfiltration is observed in only a minority of patients


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CIDP: Histology

Note the decreaseddensity of nerve fibers(arrows)

Demyelinated fibers (D) Fibers undergoing active

macrophagemediateddemyelination (M)


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Other tests

Hepatitis screen, ESR, antinuclear antibody, biochemistry profile, serum and urine immunoelectrophoresis

are necessary to exclude important associatedsystemic disorders


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CIDP: Therapy

1. Prednisolone,2. IVIg and3. Plasmapheresisall been demonstrated to be effective in controlled clinical trials

In one study, response was seen to at least 1 of these 3 main

therapies in 66% of patients

Only 1/3 of patients have a sustained remission after initial treatmentand most require ongoing treatment

Early treatment is advisable to prevent axonal loss and motor neuronloss which leads to functional decline

May be irreversible


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CIDP: Therapy

Response is measured by improvement or stabilization ofpreviously documented progressive weakness, sensoryloss, or ataxia

In responsive patients, treatment is continued until maximalimprovement or stabilization is achieved, at which point itcan be tapered or discontinued

If there is further deterioration or a relapse, the therapy canbe re-instituted

Patients with chronic progressive disease requiremaintenance therapy, although tapering the treatment canbe re-attempted periodically to determine continued need


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CIDP: Therapy

PrednisoloneFirst line therapy

Agents used for refractory patients

CyclosporinCyclophosphamide

AzathioprineMycophenolate


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CIDP: Plasmapheresis

Several controlled studies confirmed benefit Proposed mechanism

Removal of antibodies and complement componentsthat are responsible for immune-mediated damage ofperipheral nerves

Has been shown to have similar efficacy as IVIg intreatment of CIDP


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CIDP: Plasmapheresis

Treatment regimensNot standardized due to a lack of controlled studiesCommon regimen

3 plasma exchanges per week for first 2 weeks Additional treatment is determined by clinical

response


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CIDP: Therapy

Neuropathic pain Antiepileptics

Carbamazepine

GabapentinTricyclic antidepressants Amitriptyline


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CIDP: Prognosis

The outcome of CIDP is difficult to predict owing to thevariety of clinical patterns and evolution

If left untreated, it can become disabling, with loss of abilityto ambulate, work, or function independently


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Thank you


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References

Acquired demyelinating neuropathy. Brain. 119. 257 270. Ad Hoc Subcommittee of the American Academy of

Neurology, AIDS Task Force (1991). Research criteria fordiagnosis of chronic inflammatory demyelinatingpolyradiculoneuropathy (CIDP). Neurology . 41. 617 618.

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