Multifocal motor neuropathy

Muley, Suraj; Parry, Gareth

doi:10.1016/j.jocn.2012.02.011

Cited by 32 publications

(28 citation statements)

References 80 publications

(106 reference statements)

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“…To address this uncertainty, we reviewed numerous articles, reviews and book chapters for published definitions, the most salient of which are here cited 12,114,[125][126][127][128][129][130][131][132][133][134][135][136][137] , and identified key elements of a complete and unambiguous definition of multifocal neuropathy. These elements are as follows: involvement of two or more individual, named peripheral nerves and not unnamed nerve twigs; limitation to the sensorimotor somatic and not the autonomic PNS; involvement defined clinically by distribution of weakness or sensory loss; no overlap or contiguity between affected nerves; applicability of the definition to any peripheral or cranial nerve; and allowance for nerves to be affected either sequentially or simultaneously.…”

Section: Multifocal Neuropathy: Definitionmentioning

confidence: 99%

The nonsystemic vasculitic neuropathies

2017

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The vasculitides are a clinically diverse group of diseases with the histopathological signature of blood vessel-centred inflammation that results in vascular damage and ischaemic injury to the affected tissues 1 . Vasculitis can be caused by drugs, infections and cancers, but diagnostic workup reveals no trigger in most patients. Autoimmune mechanisms are active in all vasculitides, except those caused by direct infection of vessel walls. Most vasculitides are systemic and involve multiple organs and tissues. Those affecting small-to medium-sized vessels often manifest with a vasculitic neuropathy. For example, neuropathies occur in 60-70% of patients with polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis and cryoglobulinaemic vasculitis, and 40-50% of those with microscopic polyangiitis and rheumatoid vasculitis 2 .Vasculitic neuropathy can also occur without systemic vasculitis. This single-organ vasculitis of the PNS has commonly been referred to as nonsystemic vasculitic neuropathy (NSVN), but other forms of clinically isolated PNS vasculitis are now recognized and can be considered variants of NSVN 3 . Although NSVN is classically an acute, relapsing, multifocal neuropathy, it can present as a slowly progressive neuropathy without distinct asymmetries. As such, it should be considered as a possible cause of any progressive axonal neur opathy. NSVN can be diagnosed only with a nerve biopsy, with or without a concomitant muscle or skin biopsy; as nerve biopsies are seldom performed, the condition is under-recognized. The disorder must be distinguished from many other causes of multifocal neuropathy, the definition of which is itself not standardized and requires clarification.A Peripheral Nerve Society guideline group reviewed the literature on vasculitic neuropathy and published consensus recommendations on the classification, diagnosis and immunosuppressive treatment of NSVN in 2010 (REF. 4) Abstract | Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and -arguably -neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard defi...

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Section: Multifocal Neuropathy: Definitionmentioning

confidence: 99%

The nonsystemic vasculitic neuropathies

2017

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“…Despite the progressive nature of the weakness, none of the studied patients lost their job or their ability to comply with their professional duties as a result of the disease, attesting to a relatively modest effect of the disease on function as compared to some other inflammatory neuropathies. The number of years without IVIg therapy and the degree of axonal loss are identified as important determinants of disability and permanent weakness in MMN patients 1,5,40. Life expectancy in MMN is normal but conversely spontaneous remission has not been reported 1,5,40.…”

Section: Natural Historymentioning

confidence: 99%

“…The number of years without IVIg therapy and the degree of axonal loss are identified as important determinants of disability and permanent weakness in MMN patients 1,5,40. Life expectancy in MMN is normal but conversely spontaneous remission has not been reported 1,5,40. Life-threatening respiratory weakness associated with hypoventilation and respiratory insufficiency due to phrenic nerve involvement with diaphragmatic paresis has been described but is thought to be a rare manifestation of MMN 13,15…”

Section: Natural Historymentioning

confidence: 99%

Multifocal motor neuropathy: a review of pathogenesis, diagnosis, and treatment

Lawson¹,

Arnold²

2014

NDT

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Multifocal motor neuropathy (MMN) is an uncommon, purely motor neuropathy associated with asymmetric deficits with predilection for upper limb involvement. Even in the early descriptions of MMN, the associations of anti-GM1 antibodies and robust response to immunomodulatory treatment were recognized. These features highlight the likelihood of an underlying autoimmune etiology of MMN. The clinical presentation of MMN can closely mimic several neurological conditions including those with more malignant prognoses such as motor neuron disease. Therefore early and rapid recognition of MMN is critical. Serological evidence of anti GM-1 antibodies and electrodiagnostic findings of conduction block are helpful diagnostic clues for MMN. Importantly, these diagnostic features are not universally present, and patients lacking these characteristic findings can demonstrate similar robust response to immunodulatory treatment. In the current review, recent research in the areas of diagnosis, pathogenesis, and treatment of MMN and needs for the future are discussed. The characteristic findings of MMN and treatment implications are reviewed and contrasted with other mimicking disorders.

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“…19.1 ). The antibodies in MMN target gangliosides of the peripheral nerve (anti-GM1) similar to the acute demyelinating polyneuropathy AMAN/AMSAN, nevertheless without preceding infection with C. jejuni (Cats et al 2010a ; Joint Task Force of the EFNS and the PNS 2010a , b , c ; Muley and Parry 2012 ;Vlam et al 2013 …”

Section: Resultsmentioning

confidence: 99%