Ghazala Hayat

Static Magnetic Field Therapy for Symptomatic Diabetic Neuropathy: A Randomized, Double-Blind, Placebo-Controlled Trial

OBJECTIVE To determine if constant wearing of multipolar, static magnetic (450G) shoe insoles can reduce neuropathic pain and quality of life (QOL) scores in symptomatic diabetic peripheral neuropathy (DPN). DESIGN Randomized, placebo-control, parallel study. SETTING Forty-eight centers in 27 states. PARTICIPANTS Three hundred seventy-five subjects with DPN stage II or III were randomly assigned to wear constantly magnetized insoles for 4 months; the placebo group wore similar, unmagnetized device. INTERVENTION Nerve conduction and/or quantified sensory testing were performed serially. MAIN OUTCOME MEASURES Daily visual analog scale scores for numbness or tingling and burning and QOL issues were tabulated over 4 months. Secondary measures included nerve conduction changes, role of placebo, and safety issues. Analysis of variance (ANOVA), analysis of covariance (ANCOVA), and chi-square analysis were performed. RESULTS There were statistically significant reductions during the third and fourth months in burning (mean change for magnet treatment, -12%; for sham, -3%; P<.05, ANCOVA), numbness and tingling (magnet, -10%; sham, +1%; P<.05, ANCOVA), and exercise-induced foot pain (magnet, -12%; sham, -4%; P<.05, ANCOVA). For a subset of patients with baseline severe pain, statistically significant reductions occurred from baseline through the fourth month in numbness and tingling (magnet, -32%; sham, -14%; P<.01, ANOVA) and foot pain (magnet, -41%; sham, -21%; P<.01, ANOVA). CONCLUSIONS Static magnetic fields can penetrate up to 20mm and appear to target the ectopic firing nociceptors in the epidermis and dermis. Analgesic benefits were achieved over time.

Neuromyotonia: autoimmune pathogenesis and response to immune modulating therapy

BACKGROUND Neuromyotonia (NMT) has been postulated to be an autoimmune channelopathy, probably by affecting voltage gated potassium channels (VGKC) leading to excitation and abnormal discharges [Sinha et al., Lancet 338 (1991) 75]. OBJECTIVE To report three patients with NMT who had other associated immune-mediated conditions, i.e., myasthenia gravis, thymoma and various types of peripheral neuropathies. One patient had peripheral neuropathy and involvement of pre- and post-synaptic neuromuscular junction. RESULTS All three patients had evidence of polyneuropathy and neuromyotonic discharges on electrodiagnostic studies. Elevated acetylcholine receptor antibodies were noted in all patients and malignant thymoma was found in two patients with metastasis. All three patients showed moderate to marked response to plasma exchange. CONCLUSIONS These findings strongly suggest a humoral autoimmune pathogenesis of NMT, probably by K(+) channel involvement, affecting acetylcholine quantal release and postsynaptic membrane. Clinicians should be aware of this association of immune-mediated conditions in NMT patients and marked improvement with plasma exchange.

Toxoplasmic polymyositis revisited: case report and review of literature

Toxoplasmosis can cause polymyositis either by reactivation or by recent infection. Inconsistent response to antiprotozoal therapy has been the strongest argument against toxoplasmic polymyositis as a separate entity. We report a biopsy-proven case of toxoplasmic polymyositis in a cardiac transplant patient presenting with a severe proximal weakness, myopathic, electromyographic changes and ten-fold increase of anti-Toxoplasma antibodies. An early antiprotozoal therapy and plasmapheresis led to recovery. A review of previously reported cases of toxoplasmic polymyositis suggests that an early antiprotozoal therapy is the most important variable affecting the outcome of this disease. We propose that toxoplasmic polymyositis has two phases: acute, responsive to antiprotozoal therapy, and chronic, manifested by altered immune response requiring steroids. We suggest that all patients presenting with polymyositis should have serological tests for toxoplasmosis as a part of their initial evaluation and an early trial of antiprotozoal therapy in case of positive findings.

Idiopathic Inflammatory Myopathies: Clinical Approach and Management

Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair. They are uniquely identified by their clinical presentation consisting of muscular and extramuscular manifestations. Laboratory investigations, including increased serum creatine kinase (CK) and myositis specific antibodies (MSA) may help in differentiating clinical phenotype and to confirm the diagnosis. However, muscle biopsy remains the gold standard for diagnosis. These disorders are potentially treatable with proper diagnosis and initiation of therapy. Goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity, and improve quality of life. This review aims to provide a basic diagnostic approach to patients with suspected IIM, summarize current therapeutic strategies, and provide an insight into future prospective therapies.

Vecuronium-associated axonal motor neuropathy: a variant of critical illness polyneuropathy?

Neuromuscular blocking agents are routinely used as an adjunct therapy for critically ill patients. Unlike many neuromuscular blocking agents, vecuronium does not cause significant histamine release, which may lead to bronchoconstriction. Due to a short duration of action and limited accumulation, vecuronium has been widely used. Prolonged ventilatory dependence due to persistent neuromuscular blockade has been reported in patients treated with vecuronium. We report a case of an 8-year-old girl who had a primarily motor axonopathy presenting with weakness after extended vecuronium administration with prolonged course of recovery. This primarily motor neuropathy with axonal features may be a variant of critical illness polyneuropathy, a rarely reported condition in pediatric patients.

Solitary Intramedullary Neurosarcoidosis: Role of MRI in Early Detection

Background. Intramedullary neurosarcoidosis may be the first manifestation of the disease and may mimic a tumor clinically and radiographically. Two patients who presented with cervical intramedullary lesions on magnetic resonance imaging (MRI) scans were found to have neurosarcoidosis. Clinical presentation. Two patients with negative past medical history presented with progressive myelopathic features, and intramedullary cervical lesions were detected on MRI scan; the diagnosis was made on biopsy of the lesions. Early therapeutic intervention led to a favorable outcome. Conclusion. Intramedullary neurosarcoidosis, especially in the cervical cord, can be the initial presentation of the disease, mimicking a tumor. MRI scan, biopsy, and, in fewer cases, angiotensin‐converting enzyme levels can help with the diagnosis and may lead to a favorable outcome.

Amyotrophic lateral sclerosis: An emerging era of collaboratie gene discovery

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease (MND). It is currently incurable and treatment is largely limited to supportive care. Family history is associated with an increased risk of ALS, and many Mendelian causes have been discovered. However, most forms of the disease are not obviously familial. Recent advances in human genetics have enabled genome-wide analyses of single nucleotide polymorphisms (SNPs) that make it possible to study complex genetic contributions to human disease. Genome-wide SNP analyses require a large sample size and thus depend upon collaborative efforts to collect and manage the biological samples and corresponding data. Public availability of biological samples (such as DNA), phenotypic and genotypic data further enhances research endeavors. Here we discuss a large collaboration among academic investigators, government, and non-government organizations which has created a public repository of human DNA, immortalized cell lines, and clinical data to further gene discovery in ALS. This resource currently maintains samples and associated phenotypic data from 2332 MND subjects and 4692 controls. This resource should facilitate genetic discoveries which we anticipate will ultimately provide a better understanding of the biological mechanisms of neurodegeneration in ALS.

Cauda equina and conus medullaris syndrome in sarcoidosis.

Background:Neurosarcoidosis is a rare manifestation of sarcoidosis. Involvement of the nervous system in sarcoidosis can range from peripheral or cranial neuropathy to central nervous system disease. Cauda equina sarcoidosis is distinctly rare. Review Summary:The authors present a 58-year-old patient with systemic sarcoidosis who developed cauda equina and conus medullaris syndrome. Seventeen previous published cases of cauda equina sarcoidosis are reviewed. The history of systemic sarcoidosis, cerebrospinal fluid characteristics of lymphocytic pleocytosis with elevated protein, and evidence of acute denervation by needle electromyography are helpful in the diagnosis of this condition. Early diagnosis and treatment of cauda equina sarcoidosis usually provide a rapid recovery and yield a good prognosis. Conclusion:Although rare, sarcoidosis should be considered in the differential diagnosis of cauda equina syndrome, particularly in patients with unclear etiology.

Unexpected recovery in a newborn with severe hypomyelinating neuropathy

We report the case of a severely hypotonic and weak term newborn who required ventilatory support from the time of birth. Serial neurophysiologic studies were consistent with severe demyelinating polyneuropathy. The infants condition deteriorated over several weeks despite treatment with corticosteroids and intravenous immunoglobulin (IVIG) for presumed inflammatory demyelinating polyneuropathy. Histopathologic findings in a sural nerve biopsy, however, were similar to those previously reported in congenital hypomyelinating neuropathies. After 12 weeks of hospitalization and after discontinuation of corticosteroids, the patient began to recover and required no further ventilatory support. Remarkable improvement has continued for 18 months. This patient raises questions about the underlying mechanisms of hypomyelinating neuropathies in early infancy.

Magnetic resonance evidence of perineural metastasis.

Contiguous spread along perineural and endoneural spaces, that is, perineural tumor extension, in cutaneous squamous cell carcinoma is fairly common. Infrequently, these tumors spread and involve intracranial structures. One consequence of th1s complication is meningeal carcinomatosis which is underrecognized. Herein described is a patient with recurrent cutaneous squamous cell carcinoma with perineural invasion along the maxillary nerve that was subsequently shown by magnetic resonance imaging to the trigeminal root The patient Initially presented with a cavernous sinus syndrome but despite aggressive treatment, extensive meningeal carcinomatosis and cauda equina dysfunction developed. Awareness of perineural invasion and proper evaluation are crucial. Penneural spread intracranially worsens the prognosis and limits treatment options to palliation.