http://neuromuscular.wustl.edu/antibody/pnimdem.html#variant
| Acute onset β-Tubulin antibodies Childhood CNS features Diabetes IgM vs GM2 & GalNAc-GD1a Motor M-protein: IgM; IgG or IgA Multifocal Upper limb Perineuritis POEMS Sensory Subacute Typical |
- Multifocal CIDP9
- Nosology
- Multifocal CIDP
- Lewis-Sumner1
- MADSAM
- Also see: Upper limb CIDP
- Age range: 28 to 58 years
- Weakness
- Asymmetric
- Distal > Proximal
- Arms > Legs (78%)
- Proximal syndrome: Occasional
- Phrenic nerve: Diphragm weakness; Respiratory insufficiency
- Suprascapular nerve: Infraspinatus ± Supraspinatus weakness
- Other scattered distal & proximal muscles
- Sensory loss: Distal; Pansensory; Rarely severe or disabling
- Tendon reflexes: Focal loss
- Course: Slowly progressive, or Relapsing-Remitting
- Laboratory
- Electrophysiology
- Multifocal conduction block
- Nerve conduction velocities: Variably slow
- Distal latencies: Variably prolonged
- CSF protein: High but usually < 100 mg/dL
- IgM anti-GM1 antibodies: Never
- MRI: Swollen nerves in brachial plexus with high T2 signal
- Nerve pathology
- Electrophysiology
- Treatment: Prednisone; HIG
- Nosology
- Multifocal upper limb CIDP2,6
- Male:Female = 1.9:1
- Onset
- Mean = 43 to 54 years; Range 9 to 75 years
- Rarely childhood
- Acute or Progressive
- Distal
- Clinical
- Weakness
- Onset: May be motor, sensory, or both; With reduced pain
- Single or multiple upper extremity nerves
- Distal > Proximal
- Asymmetric > Symmetric
- Monomelic or Bilateral
- Sensory
- Paresthesias or numbness early in most
- Pain: 22%; Often localized to peripheral nerve territory
- Sensory loss: Usually mild, distal; Often asymmetric
- Tendon reflexes: Often reduced in involved limb(s)
- Course
- Progressive: To legs in 25%
- Some stabilize or spontaneously resolve
- Cranial nerves: 17%; Optic; III; VII
- Rule out: Brachial neuritis
- Weakness
- Laboratory
- Anti-GM1 ganglioside antibodies: Uncommon
- CSF protein
- High in 40% to 72%
- Mean 64 mg/dL; Range 21 to 128
- Nerve conduction studies
- Conduction block (100%): Proximal or Distal segments
- Slowing 35%;
- CMAP amplitude: Reduced (56%)
- Abnormal SNAPs: Some patients; Up to 83%
- Leg involvement: 34%
- MRI
- Usually normal
- May have brachial plexus lesions
- Treatment
- Response to Prednisone or HIG in some (> 50%)
- Often less recovery than with generalized CIDP
- Motor predominant CIDP 36
- Clinical
- Onset
- Age: 8 to 24 years
- Weakness: Progressive over 1 to 6 months
- Weakness
- Distal > Proximal
- Symmetric
- Arm predominant or Arms & Legs
- May be exacerbated by heat (Uhthoff-like phenomenon)
- Sensory exam: Normal
- Tendon reflexes: Diffusely absent
- Onset
- Laboratory
- Nerve conduction studies
- Motor: Distal & F-wave latency long; Conduction block; NCV slowed (17 to 33 M/s
- Sensory: Normal or reduced SNAP amplitudes
- CSF: Protein high
- GM1 antibodies: Not present
- Nerve conduction studies
- Differential diagnosis: Multifocal motor neuropathy
- Clinical
- Sensory CIDP
- Clinical
- Sensory
- Loss: Distal predominant; Pansensory or Small fiber
- Pain
- Motor: Normal or minimal distal weakness
- Tendon reflexes: Normal or Reduced
- Sensory
- Electrophysiology (NCV): Motor & Sensory demyelination
- Motor
- Conduction block
- NCV: Slow
- Distal latency: Long
- Sensory: Slow NCV
- Motor
- Treatment: Poor response to prednisone or HIG
- Clinical
- Childhood CIDP
- Prevalence: ~ 1 in 300,000; Male > Female
- Preceding infections or vaccinations: 54%
- Onset: Childhood to Teens; Rarely infancy
- Course: Several patterns
- Monophasic: 25%
- Peak disability < 3 months
- Off medications in < 1 year
- Chronic
- Persistent weakness & disability
- Require continued immunosuppression
- Acute onset: 25%
- Relapsing: Often treatment related
- Monophasic: 25%
- Clinical patterns
- Overall
- Weakness: Proximal + Distal; Usually symmetric
- Sensory loss: Pansensory; Distal; Symmetric
- Tendon reflexes: Reduced or Absent
- Other: Rare clinical CNS features
- Disease Associations
- Comparison with adult CIDP: Less with M-proteins & Diabetes
- CNS changes: Rarely reported19
- Other systems: Renal; Hearing28
- Prognosis
- Better for remission with relatively rapid onset
- Worse: Axonal loss
- Laboratory
- Pathology
- Similar to adult CIDP
- Chronic cases may have prominent demyelinating features
- MRI: Gadolinium enhancement of roots very common; Occasional CNS changes
- Nerve conduction: Similar to adult CIDP
- Pathology
- Treatment
- Corticosteroids: Response in > 90%
- IVIg: Response in > 80%
- Methotrexate: 2nd line treatment
- CIDP + IgM M-protein vs β-tubulin
- Weakness: Slowly progressive; Asymmetric
- Hyporeflexia
- Poor response to prednisone
- Antigenic epitope: β-tubulin amino acids 301-314
- CIDP associated with IgG or IgA M-protein
- Similar clinical syndrome to typical CIDP
- Weakness: Slowly progressive; Symmetric
- Partial response to immunotherapy
- CIDP + Diabetes mellitus8
- Patient characteristics: Most similar to CIDP alone
- Differences from CIDP
- Age: Older (67 years)
- Symptoms: More gait imbalance
- Electrodiagnostic: More axonal loss
- Treatment: Less improvement
- CIDP: Acute onset32
- Onset: Progressive over days to weeks
- Clinical
- Weakness
- Symmetric
- Proximal & Distal
- Uncommon: Face, Tongue, Eye movements, Respiratory failure
- Less severe than GBS
- Sensory loss: Distal
- Tendon reflexes: Reduced
- Course
- Slower progression to nadir than GBS
- Partial, but not complete improvement in strength over months
- Treatment related exacerbations
- Common
- Time of first exacerbation: Median 18 days; Range 10 to 54 days
- Especially > 8 weeks after onset
- May occur ≥ 3 times
- Weakness
- Laboratory
- Electrodiagnostic
- Prominent demyelinating features early in disease course
- More slowing of motor nerve conduction velocity than GBS
- Conduction block (30%)
- EMG denervation (75%)
- CSF protein: High
- Antibodies: IgG anti-glycolipid uncommon
- Electrodiagnostic
- CIDP: Subacute onset23
- Male > Female: 2 to 1
- Onset
- Age: Childhood or Adult
- Progressive over 4 to 8 weeks
- Antecedent infection (38%)
- Clinical
- Weakness (80%)
- Symmetric (90%)
- Proximal & Distal
- Sensory loss (80%): Distal
- Tendon reflexes: Reduced
- Course
- Improvement with corticosteroid treatment
- Few relapses
- Weakness (80%)
- Laboratory
- Electrodiagnostic: Demyelination
- NCV: Slow
- Terminal latency: Prolonged (50%)
- Conduction block: 50%
- Temporal dispersion: 50%
- Sensory: Absent SNAPs
- CSF protein: High
- Nerve biopsy
- Demyelination in some: May require teased fibers to document
- Inflammation: Epineurial; Some patients
- Electrodiagnostic: Demyelination
- CIDP + CNS features19
- Frequency: Rare patients with CNS features
- Onset
- CNS features
- Ocular
- Papilledema: Associated with high CSF protein
- Visual loss: Optic atrophy
- Myelopathy
- Tendon reflexes may be brisk
- r/o Spinal cord compression from enlarged nerve roots
- Ataxia
- Focal CNS myelin loss: 1 patient
- CNS: Focal mass-like lesion
- Weakness: Mild; Distal
- Sensory loss: Mild vibration
- Tendon reflexes: Normal
- NCV: Slow; Long distal latency
- Multiple sclerosis + CIDP27
- MS duration at onset: 4 to 22 years
- Clinical
- Weakness: Distal > Proximal;
- Sensory loss: Distal; Pan-modal
- Tendon reflexes: Reduced or Absent
- CSF protein: > 120 mg/dL
- Nerve conduction studies: Demyelinating neuropathy
- Treatment: IVIg; Corticosteroids
- Ocular
- Rule out: Late onset dysmyelination (MLD; Krabbe)
- Treatment: Corticosteroids
- Perineuritis
- Usually axonal neuropathy: Occcasional demyelinating neuropathy reported
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