In a series of 44 consecutive patients with Charcot-Marie-Tooth disease (CMT), we found restless legs syndrome (RLS) in 10 of 27 CMT type 2 (CMT2) patients (37%) and in none of 17 CMT type 1 patients (p = 0.004). In the CMT2 patients, RLS was associated with positive sensory symptoms (10/10 versus 10/17; p = 0.026). This finding supports the view that a disorder of sensory input plays a role in the pathogenesis of RLS. Symptomatic treatment may benefit these patients.
Low-grade non-Hodgkin B-cell lymphoma was found during the evaluation of 3 aged patients with predominantly sensory neuropathy of mild to moderate severity. Presenting manifestations were sensory ataxia and right ulnar mono-neuropathy in a 75-year-old man, and painful dysesthesias of the legs in two 78-year-old women. A neurophysiological study showed mainly axonopathic alterations. M-protein was present in all cases (Ig-K in two, triclonal gammopathy IgG(K)/IgM(K)/IgM-A, in one). The male patient had IgM antisulfatide antibody in high titer, whereas the other 2 patients had cryoglobulinemia (type II and type III, respectively). Our report emphasizes the occurrence of mild polyneuropathy as presenting manifestation of low-grade non-Hodgkin lymphoma, different from the clinicopathological entity of neurolymphomatosis, in which severe nerve damage occurs in association with manifest lymphoma, related to nerve infiltration by lymphomatous cells. Alternative pathogenetic mechanisms, such as antibody-mediated nerve damage, or vasa nervorum changes caused by cryoglobulin, may be implicated in our cases. Non-Hodgkin lymphoma should be considered in the diagnostic evaluation of polyneuropathy of unknown cause, especially in patients with paraproteinemia and/or cryoglobulinemia.
A 35-year-old man affected with pulmonary sarcoidosis had a 12-year history of fatigue and pain in the limbs, with normal neurological examination, except for diffusely absent deep tendon reflexes. Muscle biopsy samples showed multiple noncaseating granulomas, most prominent around the intramuscular nerves, with predominance of CD4+ cells. Intramuscular nerve bundles surrounded by granulomas were immunolabelled with laminin alpha1, alpha2, beta1 and gamma1 chain, and collagen IV. Sural nerve biopsy samples were normal. This patient showed a unique histopathological pattern of sarcoid neuromyopathy characterized by distribution of granulomas or infiltrating cells around intramuscular nerve fibers. The clinical picture, restricted to nonspecific symptoms of fatigue and myalgia, and loss of deep tendon reflexes, correlated well with the selective localization of sarcoid lesions in contiguity with the intramuscular nerves. To our knowledge, this peculiar clinico-pathological correlation has not been reported previously.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.