Critical illness myopathy is frequent: accompanying neuropathy protracts ICU discharge.

Objectives: Neuromuscular dysfunction in critically ill patients is attributed to either critical illness myopathy (CIM) or critical illness polyneuropathy (CIP) or a combination of both. However, it is unknown whether differential diagnosis has an impact on prognosis. This study investigates whether there is an association between the early differentiation of CIM versus CIP and clinical prognosis.

Critical illness polyneuropathy and myopathy in patients with acute respiratory distress syndrome.

Objective: Critical illness polyneuropathy/myopathy (CIP/CIM) is frequently described in critically ill patients who survive severe sepsis. Clinically relevant paresis is major symptom of CIP/CIM. We aimed at determining risk factors and diagnostic value of electrophysiologic testing for CIP/CIM in patients with acute respiratory distress syndrome (ARDS).

Osteochondroma of the cervical spine--a surprising finding in a liver transplanted patient with polyneuropathy and polyradiculitis: case report.

Background: Osteochondroma of the spine is a rare condition. We report a case of a patient with a cervical osteochondroma presenting with a polyneuropathy and polyradiculitis simultaneously.

Case Description: In a liver-transplant patient with progressive neurological deficits a polyneuropathy and a polyradiculitis were diagnosed.

Neuropathy under chemotherapy.

Neuropathy is a dose-limiting side effect for a number of effective chemotherapeutic agents. A better understanding of effective mechanisms will lead to novel treatment strategies that will protect neurons without decreasing therapeutic efficacy. The assessment of the efficacy and neurotoxicity of various chemotherapeutic agents is vital, for a determination of the maximum allowable dose.

[Variant of Klippel-Trenaunay syndrome. A case report].

Klippel-Trenaunay-Syndrome is a rare congenital vascular malformation with cutaneous naevi, varicose veins and limb hypertrophy. We report a patient with a variant of this syndrome presenting with extensive varicose veins and arteriovenous shunts within the left arm, bony hypotrophy of the left hand, mucocutaneous melanin spots in the face and thrombocytopenia. Imaging techniques play a major role in making a diagnosis in angiophakomatoses.

Unmyelinated fibers in human greater auricular and sural nerves: a comparative morphometric study.

Since normal structural details of human greater auricular nerve (GAN) have not as yet been studied with modern techniques, light and electron microscopic findings of seven presumably normal GANs, obtained from five patients during radical neck dissection, were compared with those of normal sural nerves (SNs). In GANs there was a tendency to higher densities per mm2 and a larger number of small-diameter fibers in myelinated fibers (MFs) and unmyelinated fibers (UFs) without obvious signs of de- or regeneration. UF histograms were unimodal in both groups, with mean UF diameters being somewhat smaller in GANS than in SNs.

Chronic multifocal neuropathy with persistent conduction block (Lewis-Sumner syndrome). A clinico-morphologic study of two further cases with review of the literature.

We present data of 2 patients suffering from chronic motor-sensory multifocal neuropathy with persistent conduction block. The first case concerns a 9-year follow-up of a female, aged 24 years at onset with persistent multiple conduction blocks and a tendency towards generalization of clinical deficits. Eight years after onset sural nerve biopsy revealed extreme interfascicular variations of de/remyelination, onion bulb formation, fiber loss, edema, and proliferation of basal lamina of endoneurial capillaries.

Sural nerve biopsy findings in leprosy: a qualitative and quantitative light and electron microscope study in 4 treated cases of the lepromatous spectrum.

Reports on biopsy findings in multifascicular nerves in lepromatous leprosy (LL) are rare and detailed morphometrical data are not available. In a case of early LL with normal electrodiagnostic findings in sural nerve, the present study revealed marked segmental de- and remyelination concomitant with the sequelae of considerable Wallerian degeneration of preferentially small myelinated fibers (MF) in spite of a normal number/nerve and density/mm2. Segmental de- and remyelination of several consecutive internodes in teased fibers suggests continuous bacterial spread via Schwann cells.

Schmidt-Lanterman clefts: a morphometric study in human sural nerve.

In the human sural nerve, large myelinated fibers contained 35 Schmidt-Lanterman (SL) clefts per mm, and small myelinated fibers contained only eight SL clefts per mm. The incidence of SL clefts is linearly related to myelin thickness. The SL clefts extended over 13 micron in large and over 9 micron in small fibers, the total extent of the SL region amounting to nearly 50% of internodal length in large and to 6% in small fibers.

Three dimensional analysis of Schwann cells associated with unmyelinated nerve fibres in human sural nerve.

In a cross section the profile of one or several unmyelinated axons is embraced by profiles of one or several Schwann cells, all surrounded by a basal lamina. Reconstructions demonstrate that this complex structure is the result of overlap and regrouping of contiguous Schwann cells and only to a lesser extent to branching of Schwann cells. More than half the 36 Schwann cells reconstructed did not branch within the 200 micrometer analysed, and one fourth had two branches.

Electrophysiology and nerve biopsy in men exposed to lead.

ABSTRACT Twenty lead-exposed men were selected on the basis of a maximum level of lead in the blood of 70-140 μg/100 ml within the past year. There was no clinical evidence of neuropathy attributable to lead and haemoglobin levels were normal. In individuals, maximum motor and sensory conduction and the amplitude of the evoked potentials were normal or borderline in the median, peroneal and sural nerves, except in the distal portion of the deep peroneal nerve.

Histology and ultrastructure of alterations in neuropathy.

Histologic findings are described in nerves from men exposed to lead, from patients with discrete clinical signs of peripheral neuropathy, and from controls. Every nerve from control subjects showed an abnormality (paranodal remyelination, segmental remyelination, or regeneration) in teased fibers. The only histologic alteration in eight lead-exposed males without signs or symptoms of neuropathy was a slightly increased incidence of paranodal remyelination.

Sensory action potentials and biopsy of the sural nerve in neuropathy.

In 167 consecutive patients with various types of neuropathy, the amplitude of the sensory potential and the maximum conduction velocity along the sural nerve were compared with conduction in other sensory nerves, and were related to structural changes revealed by nerve biopsy. Electrophysiological findings in the sural nerve were similar to those in the superficial peroneal and the median nerve, though the distal segment of the median nerve was normal in 20 per cent of the patients when it was abnormal in the sural nerve. Quantitation of histological findings was a more sensitive method than the electrophysiological study in that two-thirds of 33 patients with normal electrophysiology in the sural nerve showed mild loss of fibres or signs of remyelination in teased fibres.

Polyneuropathy. Facts and fancies.

Some conclusions are drawn from findings in 167 consecutive patients with the ordinary "garden variety" of polyneuropathy; the aetiology was unknown in 15%. Histological findings in sural nerves were related to clinical and electrophysiological abnormalities. In some patients with discrete clinical abnormalities, sensory and motor conduction and amplitudes of evoked sensory and muscle action potentials were normal, whereas the nerve biopsy showed slight but definite abnormalities.

Nerve biopsy and conduction studies in diabetic neuropathy.

Morphological findings in sural nerves were related to nerve conduction in 12 patients with diabetic neuropathy, five with mainly sensory involvement, four with severe, symmetrical sensory-motor polyneuropathy, and three with multiple mononeuropathy. All had loss of large and small myelinated and of unmyelinated fibres, even early in the disease; segmental remyelination was the most prominent myelin alteration in teased fibres, segmental demyelination was found in only a few fibres. Axonal degeneration and Schwann cell damage seem to proceed independently of each other.

Peroneal muscular atrophy (PMA) and related disorders. II. Histological findings in sural nerves.

Biopsy of the sural nerves distinguished two groups of patients with peroneal muscular atrophy (Charcot-Marie-Tooth): a hypertrophic type and a neuronal type. In patients with the hypertrophic type (10 nerves), 30-100 per cent of teased fibres of the sural nerve had demyelinated segments, numerous onion-bulb formations and often an increase in endoneurial space. Large and small fibres, with a diameter of more than 7 micron and less than 5 micron were diminished in number.

Unmyelinated fibres and Schwann cells of sural nerve in neuropathy.

Electron micrographs of 45 sural nerves from patients with acquired (22) or heredodegenerative neuropathy (23) were analysed with respect to the number of unmyelinated nerve fibres, 37 nerves with respect to the number of Schwann cell sub-units and of structures connected with Schwann cells. Findings were compared with those in 6 nerves from control subjects and referred to the total number rather than to the number per mm2 to eliminate error due to increase in the transverse endoneurial area, present in more than half the diseased nerves. Ninety-one per cent of the diseased nerves showed one or several abnormalities in unmyelinated fibres of their Schwann cells.

Electrophysical study of peroneal palsy.

The diagnostic yield of different electrophysiological criteria was examined to establish whether a peroneal palsy was due to compression of the nerve in the region of the capitulum fibulae. Slowing of sensory conduction along the segment of the nerve across the capitulum fibulae localized the lesion in 64% of 47 consecutive patients with a history indicating or suggesting compression of the nerve in the vicinity of the capitulum fibulae and there were no false positive findings in 18 patients whose peroneal palsy was not due to compression at the capitulum fibulae. In 20% of the patients with slowing along the segment across the capitulum, conduction velocity was normal when measured from the superior retinaculum to the popliteal fossa.