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Axonal Multifocal Motor Neuropathy

without conduction block

 Read about axonal EMG findings in CIDP

ALS  &  CIDP LOOK SIMILAR

Rinsho Shinkeigaku. 2001 Dec;41(12):1210-3.
New trends in neuropathy practice: clinical approach to CIDP.
Baba M.

Department of Neurological Sciences, Hirosaki University School of Medicine.


Our recent study showed that the overall prevalence of CIDP was estimated as 2.2 per 100,000 population in Aomori Prefecture, in Northan Honshu of Japan. In our series of more than 80 cases with CIDP, a chronic acquired inflammatory demyelinating polyneuropathy, nearly 30% showed clear laterality of weakness, and electrophysiologic laterality or multifocality was apparent in almost all cases.

 Nearly 90% of patients were able to walk without walking aids or other assistance.

60% showed distal hand & foot weakness.

In 12 patients with ages under 15, high arched feet were seen  in 5 cases.

Two thirds complained numbness in the extremities during progressive phase.

 Four cases initially showed severe sensory ataxia associated with motor conduction block.

 It should be, thus, reminded that clinical spectrum of CIDP is enormously wide:

Chronic acquired demyelinating multiple mononeuropathy showing asymmetric involvement (Lewis-Summer syndrome) should be put on one side of the clinical presentation of CIDP.

Multifocal motor neuropathy (MMN) is, on the other hand, an unique syndrome mimicking amyotrophic lateral sclerosis (ALS). There may be, however, true association syndrome of CIDP and ALS presenting both peripheral nerve demyelination and pyramidal sign with progressive bulbar involvement.

 Recently, several atypical varieties of CIDP showing only one-limb involvement, upper limb weakness rather than lower limb power loss, or proximal weakness, etc ... have been reported in the literature.

 To realize such clinical variations of chronic acquired demyelinating neuropathy is important for early diagnosis and commencement of treatment of CIDP. Clinical guideline for suspicion of CIDP could be useful for general physicians and neurologists unfamiliar to peripheral neuropathies.

 

Results: GM1 antibodies titers were normal in all nine cases. Six patients were treated with either prednisone or IV immunoglobulin and three showed convincing improvement.

Conclusions: These findings suggest an immune-mediated motor neuropathy with axonal electrophysiologic features that appears to be distinct from both multifocal motor neuropathy and established motor neuron disorders.

 

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Stories links

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  • Story 7
  • Salutatory conduction
  • Nerve physiology
  • Nerve fiber speeds
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    Story continued


    Neuropathies are a significant cause of morbidity worldwide, mainly from diabetes mellitus, HIV infection and leprosy. Many are treatable with immunosuppression or intravenous immunoglobulin. Tight glycaemic control slows progression of diabetic neuropathy. Even when the underlying disorder is untreatable, making a specific diagnosis and appropriate management to avoid complications and neuropathic pain can be rewarding.

  • AUTOIMMUNE EPIDEMIC
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    continued

    The simplest treatment for any neuropathy or CIDP is a gluten free diet, 30-40 % diseasefor reversal. See our celiac section of cidpusa website. We provide cures for ailing humanity.

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    celiac neuropathy