Clinical Signs Of Polyneuropathy Research Articles

Evaluating Diagnostic Ultrasound of the Vagus Nerve as a Surrogate Marker for Autonomic Neuropathy in Diabetic Patients.

Background and Objectives: Diagnostic ultrasound of the vagus nerve has been used to examine different polyneuropathies, and it has been suggested to be useful as a marker of autonomic dysfunction in diabetic patients. Materials and Methods: We analyzed the cross-sectional area (CSA) of the right vagus nerve of 111 patients with type 2 diabetes in comparison to 104 healthy adults and 41 patients with CIDP (chronic inflammatory demyelinating polyneuropathy). In the diabetes group, sympathetic skin response (SSR) was measured as an indicator for autonomic neuropathy. Carotid intima-media thickness (CIMT) was measured as a surrogate for atherosclerosis. Clinical symptoms of polyneuropathy were assessed using the Neuropathy Symptom Score and the Neuropathy Disability Score. Results: In total, 61.3% of the diabetes patients had clinical signs of polyneuropathy; 23.4% had no SSR at the feet as an indicator of autonomic neuropathy. Mean vagus nerve CSA did not differ in patients with and without diabetic polyneuropathy or in diabetic patients with and without SSR at the feet. No significant correlation was found between vagus nerve CSA and CIMT or SSR parameters in diabetic patients. Mean CSA of the right vagus nerve was slightly larger in diabetic patients (p = 0.028) and in patients with CIDP (p = 0.015) than in healthy controls. Conclusions: Effect sizes and mean differences were rather small so that a reliable diagnosis cannot be performed based on the vagus nerve measurement of a single person alone. Vagus nerve CSA seems not suitable as an indicator of autonomic dysfunction or cardiovascular risk in diabetic patients.

Small fiber involvement is independent from clinical pain in late-onset Pompe disease

BackgroundPain occurs in the majority of patients with late onset Pompe disease (LOPD) and is associated with a reduced quality of life. The aim of this study was to analyse the pain characteristics and its relation to a small nerve fiber involvement in LOPD patients.MethodsIn 35 patients with LOPD under enzyme replacement therapy without clinical signs of polyneuropathy (19 females; 51 ± 15 years), pain characteristics as well as depressive and anxiety symptoms were assessed using the PainDetect questionnaire (PDQ) and the hospital anxiety and depression scale (HADS), respectively. Distal skin biopsies were analysed for intraepidermal nerve fiber density (IENFD) and compared to age- and gender-matched reference data. Skin biopsies from 20 healthy subjects served as controls to assure validity of the morphometric analysis.ResultsPain was reported in 69% of the patients with an average intensity of 4.1 ± 1.1 on the numeric rating scale (NRS; anchors: 0–10). According to PDQ, neuropathic pain was likely in one patient, possible in 29%, and unlikely in 67%. Relevant depression and anxiety symptoms occurred in 31% and 23%, respectively, and correlated with pain intensity. Distal IENFD (3.98 ± 1.95 fibers/mm) was reduced in 57% of the patients. The degree of IENFD reduction did not correlate with the durations of symptoms to ERT or duration of ERT to biopsy.ConclusionsPain is a frequent symptom in treated LOPD on ERT, though a screening questionnaire seldom indicated neuropathic pain. The high frequency of small nerve fiber pathology in a treated LOPD cohort was found regardless of the presence of pain or comorbid risk factors for SFN and needs further exploration in terms of clinical context, exact mechanisms and when developing novel therapeutic options for LOPD.

POEMS syndrome with vascular transformation of the lymph node sinuses: A case report.

POEMS syndrome is a rare multisystem disorder associated with the clinical signs of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. However, there is often a delay in the diagnosis due to a lack of overall consideration of the symptoms collectively. For this reason, POEMS syndrome is frequently mistaken for other diseases, such as chronic inflammatory demyelinating polyneuropathy. The present study reports the case of a 40-year-old female patient, who presented with a progressive lack of strength in the lower limbs and a unilateral cervical lump. The patient's enlarged cervical lymph nodes were mistaken for local hemangioma. However, subsequently POEMS syndrome with vascular transformation of the lymph node sinuses (VTS) was diagnosed. The patient received glucocorticoid treatment (20 mg prednisone acetate, daily), which is ongoing. The most recent follow-up examination revealed that the patient's strength had improved and at the time of writing the patient remained alive. The study discusses the clinical manifestations, auxiliary examinations and reason for the misdiagnosis. Hematoxylin and eosin staining and cluster of differentiation 31 immunostaining were adopted to identify the VTS. To the best of our knowledge, this is the first report of POEMS syndrome with VTS.

Prevalence and determinants of diabetic polyneuropathy in a sub-Saharan African referral hospital

BackgroundDiabetic peripheral neuropathy is the commonest complication of diabetes mellitus, and a major cause of limb amputations. In general however, the magnitude of diabetic neuropathy in sub-Saharan Africans with diabetes has been less reliably quantified. We assessed the prevalence and determinants of diabetic polyneuropathy in hospital settings in Cameroon. MethodsWe conducted a cross-sectional survey at the Douala Laquintinie Hospital, which is one of the main reference hospital in the economic capital of Cameroon (3 million populations). Participants included all patients with type 1 (T1DM) or type 2 (T2DM) diabetes who reported to the hospital regardless of the reason, during a 5-month recruitment period. Polyneuropathy was defined as diabetic in a patient with a Diabetic Neuropathy Examination score of >3/16 and/or a monofilament score of <5/9. ResultsA total of 306 patients were recruited, including 196 women (64%) and 294 (96%) with T2DM. The mean (standard deviation) values were 59.8 (11.2) years for age and 8.4 (8.2) years for diabetes duration. Clinical signs of polyneuropathy were present in 102 (crude prevalence rate: 33.3%) patients. The polyneuropathy was symptomatic in 79/102 (77.4%) patients. Determinants of polyneuropathy were urban residence (p=0.02), infection with hepatitis C virus (p=0.002), infection with HIV (p=0.012) and presence of albuminuria (p=0.0001). ConclusionAbout one in three patients with diabetes reporting to the hospital in our setting has prevalent diabetes related polyneuropathy. This emphasizes the importance of routine implementation of therapeutic education and other measures to limit the complications.

The Influence of CSII Treatment on the Function of the Peripheral and Visceral Nerves in Type I Diabetics*

The distal latencies, motor and sensory nerve conduction velocities, and respiratory heart arrhythmia (RHA) were measured in 14 patients with type I diabetes but without clinical signs of polyneuropathy. The electroneurographic parameters tended to improve as the HbA1 value decreased. The RHA was not affected.

Electrophysiological findings in gastrectomized patients with low serum B12.

Thirty-eight patients with vitamin B12 deficiency after gastric surgery for a benign peptic ulcer were examined electrophysiologically. Thirteen (34 per cent) had electromyographical signs of peripheral nerve involvement and the amplitude of the sensory potentials of the median nerve at wrist (16 patients) was diminished, whereas sensory and motor conduction velocities were normal. Six patients had clinical signs of polyneuropathy. The electrophysiological findings are compatible with slight loss of myelinated nerve fibres. None of the patients had clinical or electromyographical signs of myopathy.

Critical illness polyneuropathy: clinical findings and cell culture assay of neurotoxicity assessed by a prospective study

First, to evaluate the role of typical intensive care-related conditions like sepsis, prolonged ventilation, drug effects and metabolic disorders in the pathogenesis of critical illness polyneuropathy (CIP); second, to investigate the possible significance of patient serum neurotoxicity assessed by an in vitro cytotoxicity assay with respect to CIP development. Prospective study. Neurological intensive care unit. Twenty-eight patients who were on mechanical respiratory support for at least 4 days during a 21-month study period. Diagnosis of CIP was established by clinical and electrophysiological examination in 16 (57%) of 28 patients. Patients were investigated on days 4, 8 and 14 of mechanical ventilation. Two of 16 CIP patients had clinical signs of polyneuropathy at initial examination. Factors that correlated significantly with the development of CIP were: the multiple organ failure score on day 8 of ventilation, the total duration of respiratory support, the presence of weaning problems and the manifestation of complicating sepsis and/or lung failure. The in vitro toxicity assay showed serum neurotoxicity in 12 of 16 CIP patients. Electrophysiological investigations yielded false positive results of the toxicity assay in six patients (not developing CIP) and false negative results in four patients (developing clinical and electrophysiological signs of CIP). Statistical analysis did not reveal a significant correlation between the diagnosis of CIP and the finding serum neurotoxicity. The results support the hypothesis of a multi-factorial aetiopathogenesis of CIP. We observed serum neurotoxicity in the majority of CIP patients, indicating the possible involvement of a so far unknown, low-molecular-weight neurotoxic agent in CIP pathogenesis.

Sleep disturbance in spinocerebellar ataxias: is the SCA3 mutation a cause of restless legs syndrome?

To identify and to characterize sleep disturbances in patients with hereditary ataxias. We observed restless legs syndrome (RLS) and impaired sleep as a frequent yet unrecognized symptom in spinocerebellar ataxia type 3 (SCA3). A total of 89 patients with genetically defined subtypes of autosomal dominant cerebellar ataxias were investigated for sleep history and neurologic findings according to a standardized protocol. Nerve conduction studies were performed. Sleep was studied by overnight polysomnography in seven patients. RLS was present in 45% of SCA3 patients but is rare in other types of autosomal dominant cerebellar ataxias. RLS was a frequent but not the only cause of sleep impairment in SCA3. Impaired sleep in SCA3 is associated with older age, long-standing disease, and brainstem involvement. RLS tended to be more frequent in patients with clinical signs of polyneuropathy, but RLS was not restricted to patients with peripheral neuropathy. RLS was not observed in healthy members of SCA3 families. RLS is a frequent and treatable cause of disabling sleep disturbance in SCA3. This study provides evidence for the expanded CAG repeat in the SCA3 gene as a molecular factor causing RLS.

Inspiration-triggered vasoconstrictive episodes in healthy subjects, in patients with diabetes mellitus and in patients with clinically manifest polyneuropathy of variable origin. A simple Doppler ultrasound test to assess the autonomic vascular functional unit of the upper extremity

A cost- and time efficient ultrasound-Doppler-test is introduced to evaluate the function of autonomic innervation of the upper limb. A voluntary inspiratory vasoconstrictor episode (VICE) after a provoked deep inspiration, transmitted via sympathetic nervous system, can be demonstrated at the radial artery using basic Doppler equipment. VICEs were investigated in 30 healthy subjects (group A), in 20 patients with diabetes mellitus without clinical signs of polyneuropathy (group B) and in 20 patients with clinically manifest polyneuropathy (group C). In all healthy subjects a two minutes arterial occlusion led to a decrease of resistance index (RI) lower than 0.9 indicating sufficient hyperemia. RI during VICE increased to 1.0 in all healthy subjects. In 50% of the patients of group B as well as in 50% of the patients of group C the test revealed abnormal findings suggesting a disorder of the functional peripheral neurovascular unity.

Early detection of distal symmetrical polyneuropathy during HIV infection by paired stimulation of sural nerve

In 203 HIV infected patients in various clinical stages neurological examination, paired stimulation (LPSS), nerve conduction velocity (NCVS) and amplitude (AMPS) of the sural nerve, distal latency (DLP), nerve conduction velocity (NCVP), amplitude (AMPP) and F waves of the peroneal nerve were recorded. Neurological examination revealed symptoms and clinical of polyneuropathy in 67 (33%) (WR 1–6) of the patients. LPSS after paired stimulation was abnormal in 25.5%, NCVS in 14.2%, AMPS in 9.8%, NCVP in 11.8%, DLP in 11.2%, AMPP in 5.9% and FWP in 14.6%. Our findings indicate a high incidence of peripheral nerve system involvement during HIV infection. In 11.5% of all patients only LPSS proved polyneuropathy. Neurophysiological results from HIV infected patients with symptoms and clinical signs of polyneuropathy were statistically significantly different from HIV infected patients without symptoms and clinical signs of polyneuropathy. The delay of LPSS represents the most sensitive neurophysiological indicator of polyneuropathy during HIV infection and announces the onset of peripheral nerve disease even in early stages of infection, according to Walter Reed staging classification 1 and 2 (approx. 20%).

Refractory period impairment in sural nerves of diabetics.

Polyneuropathy is a frequent complication in diabetics. In a non-selected group of 65 diabetics, 30 patients had clinical signs of neuropathy. These patients had more significant changes in electrophysiological parameters than patients without clinically evident polyneuropathy. Significant reduction of sural nerve conduction velocity was observed in 16 of 30 patients with and in 4 of 35 diabetics without clinical signs of polyneuropathy. Additional information was obtained when paired stimuli were applied and the test response latency was measured. Stimulus intervals of 3 ms gave the highest quota of information about involvement of the sural nerve. Significant prolongation of the test response in paired stimulation at intervals of 3 ms was found in 21 of 30 patients with clinically apparent diabetic polyneuropathy and in 16 of 35 diabetics without clinical signs of polyneuropathy.

Histochemical and electron microscopic observations on the myoneural junctions of rats with carbon disulfide induced polyneuropathy.

Myoneural junctions in the tibialis anterior muscle of rats with clinical signs of polyneuropathy induced with carbon disulfide were studied by light and electron microscopy. Histochemically demonstrable acetylcholinesterase (AChE; E.C. 3.1.1.7) activity was distributed similarly in the myoneural junctions of both the exposed and the control rats. In both groups intense enzyme activity was localized at the level of the post-synaptic membrane of the myoneural junction. The postsynaptic infoldings of the myoneural junctions of the exposed rats appeared normal. No enzyme activity was seen outside the zone of the myoneural junctions. The ultrastructure of the sub-sarcolemmal space, as well as the postsynaptic membranes of the myoneural junctions of the exposed animals, was normal. In the terminal axons signs of various degrees of degeneration were present, e.g., disappearance of the preterminal axoplasmic neurotubules, partial disappearance of synaptic vesicles, appearance of dense bodies, and even total disappearance or destruction of the terminal axons. Synaptic clefts were often widened with Schwann cell interposition. It thus seems that systemic carbon disulfide poisoning primarily alters the presynaptic structures of the myoneural junctions, while the postsynaptic side remains relatively intact, especially since the histochemical distribution of AChE in myoneural junctions was normal.