Toxic neuropathies generally result in length dependent axonal neuropathy with the exception of diphtheria and a few toxic neuropathies. In spite of occurrence of diphtheria in India there is paucity of published reports on diphtheritic neuropathy. Arsenic neuropathy commonly occurs in Bengal and Bangladesh because of ground water contamination whereas in Punjab it is due to contamination of op...

Inflammatory neuropathies are acquired disorders of peripheral nerves and occasionally of the central nervous system that can affect individuals at any age. The course can be monophasic, relapsing, or progressive. Inflammatory neuropathies are classified as acute or chronic. The acute form reaches a nadir by 4 weeks and the chronic form over 8 weeks or greater. The most common example of an acu...

Protein misfolding and aggregation have been linked to several human diseases, including Alzheimer's disease, Parkinson's disease, and systemic amyloidosis, by mechanisms that are not yet completely understood. The hallmark of most of these diseases is the formation of highly ordered and beta-sheet-rich aggregates referred to as amyloid fibrils. Fibril formation by WT transthyretin (TTR) or TTR...

During amyloid fibril formation, amyloidogenic polypeptides misfold and self assemble into soluble pre-fibrillar aggregates, i.e., protofibrils, which elongate and mature into insoluble fibrillar aggregates. An emerging class of chaperones, chaperone-like amyloid binding proteins (CLABPs), has been shown to interfere with aggregation of particular misfolded amyloid peptides or proteins. We have...

Background In familial aTTR amyloid polyneuropathy (FAP) cardiac involvement is of major prognostic value. Two approaches using radionuclide imaging proved relevant in the assessment of aTTR-related cardiac amyloidosis: detection of amyloid deposits with disphosphonates (DPD) and of sympathetic denervation with MIBG. The study aimed to compare the respective value of both approaches in patients...

Although amyloidogenic transthyretin (ATTR) mutations are common in several populations, such as black Americans, the small number of diagnosed patients homozygous for TTR amyloid and the short follow up in most studies has until now prevented an analysis of their phenotype. In Sweden, nine homozygous patients from eight families carrying the ATTR mutation Val30Met, which gives rise to fatal ne...

We discuss two of the most common of the acquired inflammatory neuropathies: Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, as well as their variants. We review their clinical presentation, electrophysiologic findings, and management, highlighting knowledge gained from the recent literature. Unfortunately, although treatments exist for both Guillain-Barre syndrom...

Neuropathic Disorders The peripheral nervous system (PNS) comprises the cranial nerves, spinal nerve roots, dorsal root ganglia, the peripheral nerve trunks (motor and sensory nerves), the terminal branchings of motor nerves as they innervate skeletal muscle, and the peripheral autonomic system [1]. Disorders of the parent cell bodies located in the spinal cord (and/or brainstem) are discussed ...