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Approximately one of three people with diabetes is affected by distal symmetric sensorimotor polyneuropathy (DSPN) which is associated with marked impairment in quality of life due to partly excruciating neuropathic pain on the one hand and painless foot ulcers on the other hand. The prevalence of painful DSPN may reach up to one quarter of patients with diabetes, while DSPN may be asymptomatic in up to half of the patients affected. Regrettably, DSPN still remains underdiagnosed. Typical neuropathic symptoms include pain, paresthesias and numbness particularly in the feet and calves. The management of DSPN includes three cornerstones: (1) lifestyle modification, causal treatment aimed at near-normoglycemia and multifactorial cardiovascular risk intervention, (2) pathogenesis-derived treatment and (3) symptomatic treatment of neuropathic pain. Multimodal pain treatment should not only aim at pain relief, but also allow for improvement in quality of sleep, mobility, and general quality of life.

Vasculitic neuropathies result from inflammation of the vasa nervorum followed by ischemia and destruction of the peripheral nerve. The inflammation can be systemic or localized, i.e. non-systemic. Systemic vasculitis can be divided into primary and secondary forms. The latter is associated with, e.g. connective tissue diseases, infections, cancer or induced by certain drugs. Around two thirds of patients with systemic vasculitis develop vasculitic neuropathy presenting as characteristic painful, multifocal mononeuropathy of acute onset. The group of non-systemic neuropathies has grown in recent years with the addition of diabetic and non-diabetic lumbosacral radiculoplexus neuropathies, among others. Within the group of connective tissue diseases, other non-vasculitic neuropathies can occur as nerve-entrapment syndromes and sensory ataxic neuropathy. The aim of this article is to present a condensed overview of neuropathies associated with vasculitis and connective tissue diseases and to communicate characteristic clinical symptoms supporting rapid diagnostic and therapeutic procedures.

Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt vorliegt.

An average of 50-80% of patients treated in the intensive care unit is affected by disturbances of neuromuscular functions due to damage to the nerves and muscles, which has led to the terms critical illness polyneuropathy and myopathy. Both components occur in 30-50% of patients, while the others predominantly show a pure myopathy, while pure neuropathy is rare. Meanwhile, the descriptive term of the concept as intensive care unit-acquired weakness (ICUAW) is preferred. The most significant risk factors for the development of ICUAW are sepsis, multiorgan dysfunction and acute respiratory distress syndrome (ARDS). In at least one third of patients, persistent impairment by paralysis, sensory disturbances and balance problems persist when they leave the ICU. At approximately 10%, these leg-accentuated and highly everyday relevant disorders persist over the first year after ICU therapy. Pure myopathy rarely leads to residual disturbances, while the neuropathic component is responsible for long-term impairments.

In this article, a case of recurrent epithelial defects in neurotrophic keratopathy is described. Multiple abrasions of the corneal epithelium with a therapeutic contact lens, corneal stitches, and amniotic membrane transplantation in combination with artificial tears brought only short-term success. However, a botulinum toxin A induced protective ptosis could finally achieve permanent epithelial closure. As this case shows, protective ptosis can be a promising approach in spite of multiple previous ineffective therapeutic efforts.

The peripheral nervous system is subject to changes during the ageing process, e. g. deep tendon reflexes decrease, as does proprioception. Polyneuropathies, on the other hand, need to be distinguished from age-related changes as independent diseases with etiologies similar to those at younger ages. Etiologies includes metabolic disorders, primary inflammatory polyneuropathies, and systemic disorders. Neuropathies associated with diabetes, malignancy, and monoclonal gammopathies appear to be more common in older patients. Using a systematic approach, it is possible to establish a specific diagnosis in the majority of cases. Since polyneuropathies contribute to reduced mobility in the elderly, an assessment of functional skills is mandatory. Polyneuropathy therapy is primarily based on the treatment of underlying conditions and neuropathic pain management. Physiotherapy and rehabilitation target pain relief and maintaining activities of daily living.

A 42-year-old patient presented with acute allodynia and hyperalgesia in her distal limbs, most severe in the innervation area of the ulnar nerve. The patient developed critical illness myopathy/polyneuropathy after septic shock 5 months prior to her presentation. After exclusion of differential diagnosis, "late onset small fiber neuropathy" after critical illness was diagnosed. Recent studies showed small fiber lesions during critical illness and in follow-up exams, where additionally neuropathic pain were proved. Dysfunction of voltage-gated Sodium channels related to severe insulin resistance during critical illness might explain the pathophysiology, as seen in critical illness myopathy/polyneuropathy. Therefore we recommend cooling, pharmacotherapy with carbamazepin/oxcarbazepine, tricyclic antidepressive agents and peripheral nerve blocks to treat patients with "late onset small fiber neuropathy" after critical illness., (Georg Thieme Verlag KG Stuttgart · New York.)

Intensive care unit acquired weakness (ICUAW) is a frequent and severe complication of intensive care management. Within ICUAW critical illness polyneuropathy (CIP) and myopathy (CIM) can be differentiated. The major symptom of ICUAW is progressive quadriparesis, which makes weaning from the respirator more difficult, can appear early after admission to an ICU and can often be detected several months after discharge from the ICU. The pathophysiology of ICUAW is multifactorial and complex. Potential therapeutic approaches are the early and sufficient therapy of mulitorgan dysfunction, optimal control of glucose levels as well as early and intensive physiotherapy. This review article discusses the data on incidence, pathophysiology, diagnostic approaches and prognosis of ICUAW.

Background: Increasingly more patients reaching our hospitals as an emergency are chronically ill or are in advanced stages of infaust, e.g., malignant, diseases. On the other hand, the treatment options for malignant diseases are improving. In an emergency, a decision must be made between life-sustaining treatment (in the context of a potentially poor prognosis) versus palliation., Development of a Decision-Making Model: The current literature about this topic is heterogeneous. The aim of the present article is to present a method using four case reports to decide either for life-sustaining treatment or for palliation with the help of the following: (1) the prognosis of the chronic disease and (2) the association of the acute situation with the chronic disease., Conclusion: This method has an advisory role and cannot be taken as a guideline. Its usefulness can only be proven in practice.

Critical illness polyneuropathy (CIP) and critical illness myopathy (CIM) are frequent complications in critically ill patients and both are associated with sepsis, systemic inflammatory response syndrome (SIRS) and multiorgan failure. Major signs are muscle weakness and problems of weaning from the ventilator. Both CIP and CIM lead to elongated times of ventilation, elongated hospital stay, elongated times of rehabilitation and increased mortality. Electrophysiological measurements help to detect CIP and CIM early in the course of the disease. State of the art sepsis therapy is the major target to prevent the development of CIP and CIM. Although no specific therapy of CIP and CIM has been established in the past, the diagnosis generally improves the therapeutic management (weaning from the ventilator, early physiotherapy, etc.). This review provides an overview of clinical and diagnostic features of CIP and CIM and summarizes current pathophysiological and therapeutic concepts.

Human immunodeficiency virus (HIV)-associated polyneuropathy has become the most common neurological complication of HIV infection and is one of the main risk factors for development of a neuropathy worldwide. Therefore HIV should always be considered as an underlying cause in patients with neuropathy. Many types of peripheral neuropathies are seen in HIV infection depending on the stage of infection. The inflammatory demyelinating neuropathies both acute (Guillain-Barré syndrome, GBS) and chronic (chronic inflammatory demyelinating neuropathy, CIDP) occur mainly at the time of seroconversion or early in the course of the disease while syndromes associated with opportunistic infections like CMV (i.e. polyradiculoneuropathy) occur in the late phase of HIV infection and are related to the loss of immune function. Distal symmetrical polyneuropathy (DSP) is the most common neuropathy in HIV-infected patients. We review the clinical manifestations, epidemiology, clinical diagnostics, pathophysiology and management strategies for HIV-associated polyneuropathies.

Introduction: in 2008, we described the documentation system of the ‘Klinik am Steigerwald’. Now, we report on the results gained with this system, especially on the therapeutic success and its sustainability., Patients and Methods: we evaluated 1,972 in-patients treated between 1999 and 2007. 74% of these patients were followed for up to 24 months. The therapeutic success achieved at the time of discharge from hospital was comparable in patients with follow-up and in patients lost to follow-up. Therefore, no relevant bias has to be assumed., Results: at discharge from hospital complaints had improved markedly or somewhat in 62–77% of the patients. 2 years after discharge from hospital between 62 and 85% of the patients said that their complaints had improved as compared to the time before admission. This is also true for progressive diseases and if medication had been reduced. The proportion of days with inability to work decreased from 21.6% before admission to 16.0% at 0–6 months after discharge to 14.0% at 6–12 months after discharge to 11.9% at 18–24 months after discharge from hospital. Special focus is laid on Morbus Crohn /colitis ulcerosa and polyneuropathy which are core areas of the ‘Klinik am Steigerwald’., Conclusion: documentation of the therapeutic success and its sustainability is essential for an evidence based medicine. Assessment of therapeutic success by complaints of patients is according to the idea of quality of life. This holds for modern as well as for traditional medical procedures like e.g., traditional Chinese medicine.

The group of autoimmune neuropathies includes the Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuritis, multifocal motor neuropathy, neuropathies associated with monoclonal gammopathies, and vasculitic neuropathies. This educational review first addresses diagnostic pathways that facilitate more rational diagnostic decisions. Many therapies are effective for treating immune neuropathies. Unfortunately, none of the available therapies are specific. In the acute phase, glucocorticosteroids, plasmapheresis, and intravenous immunoglobulins play key roles. The list of long-term therapies includes azathioprine, cyclosporine, cyclophosphamide, and immunoglobulins. The therapeutic mechanisms involved are not clear for most of these compounds. Modern immunotherapy has to consider medical aspects, available therapeutic evidence, and long-term economic burden.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated disease of the peripheral nervous system with an estimated prevalence of 1-2/100,000. The clinical presentation is heterogeneous, but the most common form causes symmetrical progressive or relapsing weakness affecting proximal and distal muscles. CIDP is among the most treatable peripheral nerve disorders and corticosteroids, plasmapheresis and intravenous immunoglobulin have been shown to be effective in short-term prospective, randomized controlled trials. Data however indicate that approximately one-third of patients do not respond to these treatment modalities, nor do they provide equivalent evidence for a durable clinical response. There is a lack of good quality controlled trials of any other immunosuppressive agent, but cyclophosphamide and cyclosporin may be of value in patients with poor response to first-line modalities. Alternatively, the use of combination therapy may increase the efficacy in unresponsive patients. This review highlights the current status of CIDP treatment trials and discusses the significance of any therapeutic option in terms of efficacy, tolerability and cost-effects.

There are a number of common aspects between rheumatology and neurology. First, many systemic rheumatic diseases also affect the central and peripheral nervous system as well as muscle causing characteristic symptom complexes. Second, there are similarities between neurological and rheumatological autoimmune diseases in terms of underlying pathomechanisms. Here the most important neurological aspects of rheumatological disorders including their diagnosis and therapy are highlighted.

Up to 40% of patients with malignant thymoma suffer from paraneoplastic symptoms (90% myasthenia, 10% other symptoms). A 55-year-old patient developed ascending symmetrical sensorimotor tetraparesis. A malignant thymoma without metastases was diagnosed 6 months later. Despite thymectomy followed by radiation and high-dose corticosteroid therapy, the polyneuropathy progressed. Six months after onset, the patient was bound to a wheelchair. Immunosuppressive therapy with cyclophosphamide was initiated, leading to marked remission. After ten cycles, the patient was able to walk independently with walking aids. After the sixth and tenth cycle, respectively, attempts to discontinue immunosuppression led to relapse. In several diagnostic workups, however, there was no tumour relapse. After 13 cycles, cyclophosphamide was replaced by immunoglobulins (0.4 g/kg per day i.v. for 5 days/month) due to progressive renal failure. The patient died just before the second course of this treatment. In conclusion, in the differential diagnosis of rapidly progressive polyneuropathy, a malignant thymoma should be considered, even in the absence of myasthenia. Immunosuppression with cyclophosphamide resulted in amelioration of symptoms in this patient.

Polyneuropathies are common disorders of the peripheral nervous system. Early diagnosis and therapy enables to stop the progression of the polyneuropathy and to ameliorate polyneuropathic symptoms in most cases. Clinical examination is sufficient to diagnose polyneuropathy. However, to reveal the etiology of a polyneuropathy additional diagnostic procedures are necessary. The general practitioner should recognize the signs and symptoms of a polyneuropathy and start necessary investigations. If the etiology of the polyneuropathy is revealed specific therapy can be started. Furthermore, polyneuropathic symptoms can be ameliorated independently of the underlying cause.

Vasculitic neuropathies are immune mediated diseases of the peripheral nervous system, in which inflammation of the blood vessels causes damage to the nerves. We distinguish neuropathies associated with primary and secondary systemic vasculitis, with rheumatic diseases, with malignant disorders, drug-induced vasculitis and the non-systemic vasculitic neuropathies (NSVN). The typical clinical picture consists in an asymmetric or multifocal, painful sensorimotor neuropathy with an acute, subacute or chronic course and acute relapses. Neurophysiology reveals an active, asymmetric, axonal sensorimotor neuropathy. The disorders usually respond to immunosuppressive treatment. A diagnosis of definite vasculitis can be made with evidence of vasculitis in a biopsy specimen. The absence of positive morphological evidence, however, does not exclude the diagnosis. There is no single laboratory test that can prove or exclude vasculitis, in NSVN even an elaborate panel of blood tests can show normal findings. Systemic vasculitis has an incidence of 4/100,000 per year and, untreated, has a poor prognosis, which is greatly improved by the use of immunosuppressive treatment. The prognosis of NSVN is generally better, although many patients need long term immunosuppression. Current treatment recommendations for vasculitic neuropathies are presented.

We report on a 60 year old patient with peripheral neuropathy, neurogenic muscular atrophy, skin ulcers, arthritis and weakness. Detection of cryoglobulins in association with typical clinical symptoms, exclusion of hepatitis C and any other disease led to a rare diagnosis: essential cryoglobulinemic vasculitis. The case demonstrates not only the difficult diagnostic process but also the problems of an adequate and effective therapy. Since the usual immunosuppressive treatments such as methotrexate, high dose corticosteroid and intermittent intravenous pulse cyclophosphamide therapy (Austin's scheme) failed, we performed plasmapheresis (cascade filtration), which brought about an immediate and long-term remission. Besides discussing various types of plasmapheresis procedures and potential pathophysiological mechanisms, we point out that this therapy could find an early use in severe essential cryoglobulinemic vasculitis because of its excellent risk/benefit ratio.

Only some patients with HIV-infection receive an adequate pain therapy. In later stages of HIV-infection up to 50% 6 of patients perform extraordinary doctor visits because of pain. Principally primary and secondary neuromanifestations of HIV-infection have to be differentiated. Rare forms of HIV-associated polyneuropathies represent mononeuropathy or mononeuritis multiple acute and chronic inflammatory demyelinating polyneuropathy and polyneuropathy caused by opportunistic infections. HIV-associated distal-symmetric polyneuropathy represents the most common form during HIV-infection with a prevalence up to 50%. Typical clinical symptoms and signs are pain, hyp- and dysaesthesia, diminuted deep tendon reflexes, motor deficits and autonomic disturbances. Always neurological examination and neurophysiologic investigation on the sural and peronaeal nerve are necessary for monitoring progression of polyneuropathy and as basics before starting antiretroviral therapy with neurotoxic substances. According to momentary opinion, HIV-associated distal-symmetric polyneuropathy represents no indication for antiretroviral therapy. Symptomatic therapy includes antiepileptic medication as gabapentine, antidepressive drugs as amitiptyline and additionally retarded opiates. Depressive disorders ma y accentuate pain problems a n d need psychotherapeutic and thymoleptic therapy. Special problems occur when neurotoxic substances evoke or deteriorate polyneuropathy. In these cases an individual therapeutic proceeding about continuation or discontinuation of neurotoxic medication is necessary. Symptoms of myopathy during HIV-infection are muscle pain, elevation of CK and typical changes of motor units detected by electromyography. In most cases biopsy is necessary for diagnosis of specific forms of HN-associated myopathy. HIV-associated polymyositis is treated by non-steroid analgetics, corticoids, immunoglobulines and plasmapheresis, myopathy induced by neurotoxic medication analogous to polyneuropathy.

Polyneuropathy developing in critically ill patients ("critical illness polyneuropathy"--CIP) is diagnosed with increasing frequency in intensive care units. CIP is an axonal polyneuropathy leading to difficulties in weaning from artificial ventilation and symmetrical flaccid tetraparesis. Pathogenetically CIP is considered to be part of the multiple organ dysfunction syndrome (MODS) in the course of sepsis or systemic inflammatory response syndrome (SIRS). The incidence of CIP in septic patients with MODS ranges from 50% to 70%. Electroneurography (ENG) and electromyography (EMG) are essential diagnostic procedures. The mortality in patients with CIP is higher than in patients without CIP. Depending on the severity of CIP, recovery of neurological function in survivors is usually relatively good.

High-dose intravenous immunoglobulins (ivIg) are an effective therapy in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In both diseases, data regarding ivIg long-term treatment are sparse. Therapy with ivIg was performed in 18 patients with CIDP or MMN. Sixteen patients responded to ivIg therapy; they were treated for more than 6 months. Two of them had not previously shown any positive response to other immunosuppressive treatments. Response to ivIg therapy could be observed even after a long disease duration (maximum of 19.5 years). All 16 therapy responders now have no or only mild clinical symptoms. Treatment could eventually be completely stopped in 6 patients; they have now been in complete remission without therapy for a maximum of 63 months. Ten patients still receive regular ivIg infusions in different dosages. No severe side effects were observed in the whole group. IvIg therapy is an effective, safe and easily applicable treatment regimen even in the long-term course of CIDP and MMN.

Pathophysiology and pathoanatomy of uremic neuropathy are not yet well understood. A single hemodialysis positively increases nerve conduction velocities of uremic patients, thus demonstrating a functional A alpha-fiber improvement by detoxification. This study tested whether non-invasive Vibrameter and Thermotest studies show a similarly positive effect for A beta-, A delta- and C-fibers and whether the psychophysical techniques might substitute for nerve conduction studies. 20 uremic patients depending on chronic intermittent hemodialysis were examined shortly before and after a hemodialysis. Using a scaled 128 Hz tuning fork, a Vibrameter and a "Marstock"-Thermotest, vibratory and warm and cold thresholds were assessed at both internal malleoli according to the method of limits. In addition, thermal thresholds were determined at the volar aspect of the non-shunted wrist. In nine patients the Vibrameter showed elevated thresholds before and after dialysis as did six patients with the tuning fork. Warm or cold thresholds were abnormal in four patients before treatment and in eight patients after dialysis. This was due to some patients reporting elevated thresholds after dialysis although they had had normal thresholds before the treatment. Still, the overall sum of abnormal thermal or vibratory thresholds at the different tested body sites had decreased after treatment. Moreover, mean values of thermal and vibratory thresholds of the 20 patients improved with dialysis (p < 0.05). Tuning fork results were too coarse and failed to show a dialysis effect.(ABSTRACT TRUNCATED AT 250 WORDS)

High-dose intravenous immunoglobulin G (IVIG) was used as alternative treatment in 39 patients with crisis in myasthenia gravis (MG), acute polyneuritis (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP). Of 10 patients with MG, 3 recovered completely, 5 patients improved except for long-standing ocular signs, 1 patient was weaned from artificial ventilation, and one patient did not improve after IVIG, but did so after plasma exchange. 25 GBS patients were treated with IVIG, progression of weakness was stopped in 24; 12 showed complete or near complete recovery and 6 improved markedly. Of 4 patients with CIDP, 2 responded definitely. The majority of patients with MG, GBS, CIDP, respond to IVIG treatment. The effect seems to be equivalent to plasma exchange. In GBS, IVIG may be superior. Side effects of IVIG are negligible, and there is no risk of transmission of AIDS-, hepatitis-, or other viruses.

It is reported on the combined simultaneous treatment with haemodialysis and controlled ultrafiltration. When the treatment is performed three times five hours a week this method shows a clear improvement of the general condition of the patient with -- normalisation of blood pressure, -- increase of haematocrit by removal of chronic hyperhydration, -- an essentially more insignificant transfusion frequency, -- balance of the products of lipometabolism, -- subsidence of the polyneuritic symptoms.

On the basis of an analysis of 56 acute cases of polyneuritis of the Landry type treated in a period of 15 years at a neuropsychiatric intensive care unit, an attempt is made to establish relations between spinal fluid findings, kind of the course of the disease, rate of development of paralyses and the prognosis. According to the results of the examinations, the spinal-fluid findings cannot be attributed a prognostic significance. It appears, however, remarkable that the group of patients with a high rate of spreading of the pareses showed both the highest mortality and full remission rates. On the basis of own experiences and compilation of intensive medical literature, the most frequent complications of the disease and its therapy problems are dealt with.

Lipomatosis symmetrica benigna is a rare condition, usually found in association with the complications of chronic alcoholism. A case is reported which showed the typical clinical picture, and diagnosis and therapy will are discussed.

The most common forms of polyneuropathies are the alcoholic and diabetic polyneuropathies. They each constitute 1/3 of all polyneuropathies. The first symptoms shown by the alcoholic polyneuropathy are symmetric sensory disturbances with loss of tendon reflexes and of vibration sense in the peripheral segments of the lower extremities. At the beginning one almost always finds pressure pain in the calves. Important differential clues in diagnosis compared to the diabetic neuropathy, are the age at which the disease begins, the degree to which the autonomic nerve fibres and the cranial nerves are affected, as well as the form of manifestation. Pathogenetically, a direct toxic alcohol effect can above all be suspected in accordance with the typical electrodiagnostic findings with a neurogenic pattern in the EMG in the case of normal or slightly diminished conduction velocity, and in agreement with the morphological finding of an axonal degeneration in most of the biopsies. Possibly, in a small number of cases a vitamin deficiency or a malabsorption can play a causal role. The prognosis is good by complete abstinence from alcohol.