Diagnosis and treatment in inflammatory neuropathies

Abstract

The inflammatory neuropathies are a large diverse group of immune-mediated neuropathies that are amenable to treatment and may be reversible. Their accurate diagnosis is essential for informing the patient of the likely course and prognosis of the disease, informing the treating physician of the appropriate therapy and informing the scientific community of the results of well-targeted, designed and performed clinical trials. With the advent of biological therapies able to manipulate the immune response more specifically, an understanding of the pathogenesis of these conditions is increasingly important. This review presents a broad overview of the pathogenesis, diagnosis and therapy of inflammatory neuropathies, concentrating on the most commonly encountered conditions.

CIDP: mimics and chameleons

“…Cases are classified as definite, probable or possible, depending upon the number of criteria fulfilled. In most, finding a raised CSF protein without CSF leucocytosis5 further supports the diagnosis. Clear evidence of macrophage-associated demyelination and remyelination, with or without a T-cell inflammatory endoneurial infiltrate in a sensory nerve biopsy, remains the gold standard supportive criterion (figure 1).…”

Cerebellar learning distinguishes inflammatory neuropathy with and without tremor

“…Neurology Inflammatory mediated neuropathies are common and potentially treatable. 1 Tremor occurs with immunoglobulin M paraproteinaemic neuropathy (IgMPN) [2][3][4] and less commonly in other inflammatory neuropathies. 5 It has been suggested that temporally distorted peripheral inputs reach a normally functioning central processor, such as the cerebellum, which is misled into producing a delayed second agonist burst and tremor.…”

Clinical Reasoning: A 34-year-old man with recurrent limb weakness

“…Furthermore, in some cases where conduction block is not found or the diagnosis remains in question, IVIg may be used as a diagnostic trial. 4 In contrast to CIDP, steroids do not have a role in MMN and may worsen the patients' weakness. 3 Plasmapheresis is not helpful either.…”

“…Other immunosuppressive therapies such as cyclophosphamide, cyclosporine, and azathioprine have been tried with variable response but there are no double-blind, randomized, placebo-controlled studies to support the use of these medications. 4 The prognosis of patients with MMN is relatively good in at least two-thirds of patients with a good response to IVIg. 5 Unfortunately, some patients do not respond to IVIg, and other immunosuppressive therapy should be tried, although evidence for effectiveness is lacking.…”