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At least 19 recordsLinked to original sources
PubMed1999

Chronic inflammatory demyelinating polyneuropathy in diabetics: motor conductions are important in the differential diagnosis with diabetic polyneuropathy.

OBJECTIVE: It is important to recognize CIDP occurring in diabetics because, unlike diabetic polyneuropathy, it is treatable. The aim of this study was to find out whether there are clues which help to differentiate CIDP in diabetics from diabetic polyneuropathy. METHODS: We compared the electrophysiological and pathological findings of 7 diabetics, who developed a predominantly motor polyneuropathy with the features of CIDP, with a group of diabetics referred for symptomatic polyneuropathy. RESULTS: Of the 7 diabetics we believe developed CIDP, 6 met at least 3 and one patient two of the 4 electrophysiological criteria of demyelination. Of the 100 patients referred for diabetic polyneuropathy, only 4 fulfilled two criteria and none 3. Nerve biopsy findings were not helpful in differential diagnosis, as segmental demyelination and remyelination, onion bulbs and inflammatory infiltrates, which are the histologic features of CIDP, were also present in diabetic polyneuropathy. CONCLUSIONS: CIDP can be diagnosed in a diabetic patient when motor symptoms are predominant, are more severe than expected in diabetic polyneuropathy and 3 of the 4 electrophysiological criteria for demyelination are fulfilled. When only two criteria are met, we believe that a trial with one of the established treatments for CIDP may be helpful in confirming the diagnosis.

Adult↗
PubMed1998

Chronic symmetric symptomatic polyneuropathy in the elderly: a field screening investigation of risk factors for polyneuropathy in two Italian communities. Italian General Practitioner Study Group (IGPST).

A total of 4191 patients 55 years and older were screened for clinical features and risk factors of polyneuropathy by 27 general practitioners (GPs) in two areas of Italy (Varese and San Giovanni Rotondo). Polyneuropathy was diagnosed in the presence of two or more symptoms associated with bilateral impairment of at least two of the following: strength, sensation, tendon reflexes. A risk factor for polyneuropathy (associated disease or neurotoxic agent) was recorded based on its presence in the GP's records, the presence of specific treatments, or an affirmative answer to the interviewer's question. The prevalence of polyneuropathy among patients with no recognized exposure to diseases or neurotoxic agents was 1.6% (Varese 1.6%; San Giovanni Rotondo 1.8%). The corresponding values were 11.8% (Varese 11.8%; San Giovanni Rotondo 11.9%) for patients with one risk factor, and 17.3% (Varese 19.2%; San Giovanni Rotondo 13.0%) for patients with two risk factors. Combining the two populations, the prevalence of polyneuropathy was highest in diabetics (18.3%), followed by patients with a diagnosis of alcoholism (12.5%), non-alcoholic liver disease (10.9%), and tumor (7.1%). Diabetes was the commonest independent risk factor for polyneuropathy (odds ratio 11.3), followed by alcoholism (7.4), non-alcoholic liver disease (4.9), and tumor (2.6).

Aged↗
PubMed2001

A case of familial amyloid polyneuropathy homozygous for the transthyretin Val30Met gene with motor-dominant sensorimotor polyneuropathy and unusual sural nerve pathological findings.

OBJECTIVE: To report a case of familial amyloid polyneuropathy homozygous for the amyloidogenic transthyretin (ATTR) Val30Met gene with motor-dominant sensorimotor polyneuropathy and unusual sural nerve pathological findings. METHODS: Mass spectrometry analysis and polymerase chain reaction-restricting fragment length polymorphism were performed. A right sural nerve biopsy specimen was obtained for histological investigation. SETTING: Academic medical center. RESULTS: A 56-year-old Japanese man living in a local town (Nakajima, Japan) in Ishikawa Prefecture, a nonendemic area of type I familial amyloidotic polyneuropathy, had vitreous amyloidosis, motor-dominant sensorimotor polyneuropathy, erectile dysfunction, and urinary incontinence. He had neither orthostatic hypotension nor indolent diarrhea. Restriction enzyme analysis with EcoT22 I of amplified DNA and mass spectrometry analysis revealed homozygosity for ATTR Val30Met. Of 8 family members, 5 were evaluated and found to be heterozygous for ATTR Val30Met; a family history found no relative with the similar neurologic disorders. The sural nerve biopsy specimen showed focal edema and an amyloid deposit in the subperineural tissue, associated with moderate loss of myelinated and unmyelinated fibers. CONCLUSIONS: In addition to the findings characteristic of homozygosity for ATTR Val30Met such as vitreous amyloidosis and relatively less autonomic involvements, this case had the unique findings of motor-dominant sensorimotor polyneuropathy and unusual sural nerve biopsy specimen results.

Amyloid Neuropathies, Familial↗
PubMed1998

Mandibular nerve involvement in diabetic polyneuropathy and chronic inflammatory demyelinating polyneuropathy.

Sensory complaints in the area of the mandible and mouth often escape notice or remain undiagnosed. Using electromyographic recording of the trigeminal reflexes and motor responses, we sought trigeminal dysfunction in 50 patients with peripheral neuropathy, and tried to gain pathophysiological information on the mechanisms provoking trigeminal damage. Trigeminal reflex recordings (early and late blink reflex after supraorbital stimulation, early and late masseter inhibitory reflex after mental stimulation, and jaw jerk) disclosed abnormalities caused by sensory trigeminal neuropathy in 8 out of 15 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 13 out of 23 patients with severe diabetic polyneuropathy, and in none of 12 patients with mild diabetic polyneuropathy. Six patients had abnormal motor responses in facial or masseter muscles. The response affected most frequently was the masseter early inhibitory reflex (also called first silent period, SP1) after mental nerve stimulation, its latency being strongly delayed. We found these long delays not only in patients with CIDP, but also in diabetic patients with severe polyneuropathy. We conclude that peripheral polyneuropathies often cause subclinical damage to the trigeminal nerve, especially to its mandibular branch. We believe that the nerve fibers running along the alveolar-mandibular pathway are more exposed to damage because of their cramped anatomical route in the mandibular canal and below the internal pterygoid muscle and fascia.

Adolescent↗
PubMed2000

F-wave latency serves as the most reproducible measure in nerve conduction studies of diabetic polyneuropathy: multicentre analysis in healthy subjects and patients with diabetic polyneuropathy.

AIMS/HYPOTHESIS: For use in future drug development for diabetic polyneuropathy, we conducted multicentre trials to assess the reproducibility of nerve conduction studies. METHODS: All measurements were repeated twice at a time interval of 1-4 weeks in 132 healthy subjects (63 men) and 172 patients (99 men) with diabetic polyneuropathy. Using a standardised method, 32 centres participated in the study of control subjects and 65, in patients with diabetic polyneuropathy. Motor nerve conduction studies consisted of stimulating the left median and tibial nerves and recording the compound action potential from abductor policis and adductor hallucis for measuring amplitude, terminal latency and minimal F-wave latency. For sensory conduction studies, sensory nerve action potentials were recorded antidromically from the second digit and the posterior aspect of the lateral malleous after distal stimulation of the left median and sural nerves. We also calculated motor conduction velocity, F-wave conduction velocity and sensory conduction velocity. The relative intertrial variation and intraclass correlation coefficient were used as an index of reproducibility. RESULTS: Of all the measurements, F-wave latency yielded the highest intraclass correlation coefficient with the smallest relative intertrial variation for both median and tibial nerves in both groups. CONCLUSION/INTERPRETATION: Median and tibial F-wave latency provide the most reproducible measures for a nerve conduction study, serving as one of the best measures in multicentre drug trials for diabetic polyneuropathies.

Adult↗
PubMed2002

[A sporadic case of late-onset familial amyloid polyneuropathy type I (transthyretin Met 30-associated familial amyloid polyneuropathy) inborn habitant of Ehime prefecture].

We reported a 62-year-old man of late-onset familial amyloid polyneuropathy type I(transthyretin Met 30-associated familial amyloid polyneuropathy) from Ehime Prefecture. There was no family history related to endemic Japanese foci (Nagano and Kumamoto foci). He demonstrated paraesthesia in the legs and mild autonomic symptoms at the age of 52. These symptoms gradually developed. Analysis of the transthyretin gene from his leucocytes demonstrated he had Met 30 transthyretin mutation. Therefore, he was diagnosed with late-onset familial amyloid polyneuropathy type I(FAP 1). In some families, asymptomatic carriers with the mutant transthyretin gene were diagnosed. In early stage, this patient's polyneuropathy and autonomic nervous system dysfunction were less serious than those of FAP 1 patients from endemic Japanese foci. These symptoms of this patient was slowly progressive. He hoped liver transplantation (brain death or living-related) treatment if possible. Now he became 68-year-old and bed-ridden.

Amyloid↗
PubMed1982

Congenital hypomyelination polyneuropathy. Pathological findings compared with polyneuropathies starting later in life.

Biopsies from patients with congenital hypomyelination polyneuropathy (Group I) and with late infantile (Group II) and juvenile (Group III) forms of hereditary motor and sensory neuropathy (HMSN) type III were compared, using morphometric methods and ultrastructural analysis. In congenital polyneuropathies (Group I), myelin sheaths were practically absent and onion bulbs, essentially made of multiple laminae of double layered basement membrane, surrounded every axon in the size range of normal myelinated axons. The number of these axons was markedly reduced. Serial sectioning of an isolated fibre showed that the territory of successive clusters of Schwann cell nuclei was considerably reduced when compared with the biopsies in Groups II and III and with normal controls. Fibres without myelin surrounded by multiple layers of basement membrane represented between 25 and 50 per cent of the entire population of fibres in the size range of myelinated fibres in Group II and were practically absent in Group III. The number of "myelinated' fibres (that is, fibres with myelin, and amyelinate or demyelinated fibres) was normal in Groups II and III. Although there is no indication that congenital hypomyelination onion bulb polyneuropathy is a separate entity, it can be considered as a subtype of Dejerine-Sottas disease (HMSN type III). In this disease there is a gradient of severity both in clinical expression and in the disorder of Schwann cells. In the severe congenital form, all Schwann cells are affected and are incapable of forming myelin. The diminution of the number of nerve fibres in the "myelinated' fibre size range, whether or not related to a prenatal involvement of Schwann cells, is another expression of the gravity of this form. The proportion of amyelinate fibres, i.e. with Schwann cells incapable of forming myelin, becomes less in the more benign late infantile and juvenile forms of the disease in which a process of demyelination and remyelination takes place. The literature on the congenital neuropathies, a heterogeneous assembly of diseases, is reviewed.

Adolescent↗
PubMedDate not supplied

[Evaluation of the evoked potentials in various acquired polyneuropathies (Guillain-Barré syndrome and chronic recurrent polyneuropathy). Preliminary report].

In 15 patients with acquired polyneuropathy (Guillain-Barré syndrome and chronic recurrent polyneuropathy) the conduction velocity was measured in the peripheral nerves of the upper and lower extremities, the latency of the F wave was determined, and the somatosensory evoked potentials were assessed stimulating the median enerve and posterior tibial nerve. The abnormalities were assessed in the parameters of the obtained somatosensory evoked potentials comparing them with the changes of F wave and the velocity of conduction in peripheral nerves. The sensitivity and usefulness of these methods in acquired polyneuropathies are discussed.

Adolescent↗
PubMed1998

[Polyneuropathy in the critically ill patient--critical illness 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.

Critical Care↗
PubMed1994

Antiarrhythmic drugs and polyneuropathy. The Collaborative Group for the Study of Polyneuropathy.

A total of 151 patients on chronic treatment with amiodarone and other antiarrhythmic drugs were subjected to standard clinical and electrophysiological investigation to assess the prevalence and specificity of polyneuropathy. Twenty two untreated patients with cardiac disorders and 246 normal subjects served as controls. Abnormal electrophysiological findings supporting the diagnosis of polyneuropathy were present in 38 subjects (25%) given antiarrhythmic drugs, with even distribution among drugs, and four untreated patients (18%). Concurrent clinical abnormalities were present in five treated patients (one each with amiodarone, propafenone, and flecainide, and two with multiple drugs). Therefore, electrophysiological abnormalities are a common, although non-specific, feature in patients taking antiarrhythmic drugs. Amiodarone users do not seem at higher risk of polyneuropathy than subjects treated with other drugs or even untreated patients with cardiac disorders.

Adolescent↗
PubMedDate not supplied

[Recent aspects of acute and chronic inflammatory polyneuropathies: Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy].

INTRODUCTION: In last decade many advances have occurred in knowledge of pathogenic, in the different types of clinic expression and in the therapy of both acute and chronic polyneuritis. OBJECTIVE: To review the recent advances in the childhood expression of these disorders. DEVELOPMENT AND CONCLUSIONS: Into the broad term of Guillain-Barré syndrome (GBS) several types are considered: demyelinating, motor sensory axonal and motor axonal, and the Miller Fisher syndrome (MFS). Diagnostic criteria, clinical and neurophysiological are explained. The actual modes of treatment are reviewed, especially immunoglobulins and plasmapheresis. The chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare disease in childhood but it is necessary think on it when a picture of demyelinating polyneuropathy that seems the more frequent Charcot Marie Tooth type 1 polyneuropathy, because the treatment of CIDP changes radically the prognosis. Last advances of pathogenic, diagnostic criteria and treatment with corticosteroids, immunoglobulins and plasmapheresis are reviewed.

Guillain-Barre Syndrome↗
PubMed1979

[The diabetic polyneuropathy. II. Polyneuropathy, angiopathy and nerve conduction velocity].

789 patients with diabetes mellitus were studied by clinical and electroneurographical examination. Motor conduction velocity of the median and the tibial nerve and sensory conduction of the median nerve were determined. 81.1% of the patients we suffering from diabetes which began in childhood or adolescence, 13.9% were suffering from maturity onset diabetes. Average duration of the disease was 9.5 years, average age was 26.7 years. Clinical signs of polyneuropathy were found in 19.1%. Typical findings were pain and paraesthesia, lack or abolition of triceps surae reflexes, impaired pallaesthesia on lower extremities. 48.3% of 151 patients with clinical signs of polyneuropathy were suffering from combined angiopathy, 32.5% from microangiopathy, 7.9% from macroangiopathy. Severity of complicating retinopathy and macroangio,athy were found to be correlated with polyneuropathy. 58.2% of 323 diabetics with at least one delayed nerve conduction velocity exhibited signs of angiopathy. In nearly 30% of children and adolescents after comparatively short duration of the disease at least one conduction velocity was delayed. In diabetic children and adolescents metabolic disturbances are assumed to cause peripheral nerve dysfunction.

Adolescent↗
PubMed1986

Multiple myeloma with polyneuropathy and coagulopathy. A case report of the polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin change (POEMS) syndrome.

A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.

Blood Coagulation Disorders↗
PubMed2002

Can electrophysiology differentiate polyneuropathy with anti-MAG/SGPG antibodies from chronic inflammatory demyelinating polyneuropathy?

OBJECTIVES: Patients with polyneuropathy and antibodies to myelin-associated glycoprotein (MAG) and sulphated glucuronyl paragloboside (SGPG) differ from chronic inflammatory demyelinating polyneuropathy (CIDP) because of a slower, progressive course, symmetrical and predominantly sensory involvement of legs, predominantly distal slowing of motor conductions, and poorer response to therapy. We studied whether a wide set of electrophysiologic parameters may differentiate these two neuropathies. METHODS: We reviewed the electrophysiological studies of 10 patients with anti-MAG/SGPG antibodies and 22 with CIDP examining: (1) motor conduction velocity and distal compound muscle action potential amplitude; (2) conduction block (CB) and temporal dispersion; (3) distal motor latency and terminal latency index (TLI); (4) F wave and proximal conduction time; and (5) sensory conduction and occurrence of abnormal median with normal sural sensory potential. RESULTS: Anti-MAG/SGPG neuropathies showed: (1) more severe involvement of peroneal nerves; (2) more frequent disproportionate distal slowing of motor conductions (TLI< or =0.25) and absent sural potential, and (3) no CB. However 3/22 CIDP patients also had at least two nerves with TLI< or =0.25 and no CB. CONCLUSIONS: Electrophysiologic findings suggest in anti-MAG/SGPG neuropathy a length-dependent process with a likely centripetal evolution. A disproportionate slowing of conduction in distal segments of motor nerves suggests the diagnosis of anti-MAG/SGPG neuropathy, although it is not pathognomonic.

Adult↗
PubMed2004

Ten years of international experience with liver transplantation for familial amyloidotic polyneuropathy: results from the Familial Amyloidotic Polyneuropathy World Transplant Registry.

BACKGROUND: Transthyretin (TTR) amyloidosis is a group of systemic amyloidoses disorders caused by an amyloidogenic TTR variant. Untreated, it slowly leads to severely disabling symptoms that relentlessly progress until the death of the patient. Because the mutant form of TTR is produced mainly in the liver, successful orthotopic liver transplantation (OLT) results in the elimination of the source of the variant TTR molecule and is presently the only known curative treatment. OLT in patients with familial amyloidotic polyneuropathy (FAP) was first performed in 1990 at the Karolinska Institute in Sweden, and because the results were promising other centers took up the procedure. METHODS: To gain as great an experience as possible regarding this treatment, the Familial Amyloidotic Polyneuropathy World Transplant Registry (FAPWTR) was initiated in 1995, and this article presents the 10-year registry results. RESULTS: A total of 54 centers in 16 countries have performed OLT for FAP, and today approximately 60 OLTs are performed annually worldwide. During the last decade, a total of 539 patients have undergone 579 OLTs. Patient survival is excellent (overall 5-year patient survival 77%) and comparable to the survival with OLT performed for other chronic liver disorders, but longer follow-up is needed to compare the outcome after OLT with the natural course of the disease. The main cause of death was cardiac related (39%). CONCLUSIONS: We believe that the FAPWTR has become a valuable tool that will help to accurately evaluate the potential risks and benefits of OLT in patients with FAP and promote a fruitful collaboration between centers engaged in this field.

Adult↗
PubMedDate not supplied

[Comparison of electrophysiologic changes in persons with alcoholic polyneuropathy and polyneuropathy caused by carbon disulfide].

A group of 39 patients with alcoholic polyneuropathy (Alc) and another group of 33 patients with carbon disulphide polyneuropathy were studied by electrophysiological methods using an identical programme, and the results were compared. In both groups evidence was found of damage to the peripheral neuron of the type of axonal neuropathy with signs of axonal loss, with moderate slowing down of conduction velocity and with associated signs of regeneration. The dynamics of changes in the Alc group was greater. The cause of a slower rate of changes in the CS2 group seems to be related to a more gradual exposure to the damaging agent, while in alcohol-dependence the toxic agent has a more acute action, with remissions during abstinence and exacerbations during increased alcohol consumption.

Action Potentials↗
PubMed1999

Late-onset familial amyloid polyneuropathy type I (transthyretin Met30-associated familial amyloid polyneuropathy) unrelated to endemic focus in Japan. Clinicopathological and genetic features.

Clinicopathological and genetic features were assessed on 35 Japanese families affected by late-onset familial amyloid polyneuropathy type I (transthyretin Met30-associated familial amyloid polyneuropathy, FAP TTR Met30) whose siblings were unrelated to endemic Japanese foci. In these patients (50 years or older), the most common initial symptom was paraesthesias in the legs. Autonomic symptoms were generally mild and did not seriously affect daily activities. The male-to-female ratio was extremely high (10.7 : 1). A family history was evident in only 11 out of 35 families, and other patients were apparently sporadic. The rate of penetrance was very low. Symptomatic siblings of familial cases showed a late age of onset, male preponderance and clinical features similar to those of the probands. Asymptomatic carriers, predominantly female, were detected relatively late in life. The geographical distribution of these late-onset, FAP TTR Met30 cases was scattered throughout Japan. In three autopsy cases and 20 sural nerve biopsy specimens, neurons in sympathetic and sensory ganglia were relatively preserved. Amyloid deposition was seen in the peripheral nervous system, particularly in the sympathetic ganglia, dorsal root ganglia and proximal nerve trunks such as sciatic nerve. These abnormalities were milder than those seen in typical early-onset FAP TTR Met30, as observed in two Japanese endemic foci of this disease. While axonal degeneration was prominent in myelinated fibres, resulting in severe fibre loss, unmyelinated fibres were relatively preserved. Our cases of late-onset FAP TTR Met30 showed features distinct from those of typical early-onset FAP TTR Met30 that occurred in the two Japanese endemic foci. Factors responsible for clinicopathological differences between these two forms of FAP TTR Met30 need to be identified.

Action Potentials↗
PubMed1995

Plasma exchange and chlorambucil in polyneuropathy associated with monoclonal IgM gammopathy. IgM-associated Polyneuropathy Study Group.

The study compared chlorambucil alone with chlorambucil in combination with plasma exchange in patients with polyneuropathy associated with monoclonal IgM. Forty four patients were prospectively randomly assigned, in a comparative open trial, to receive either 0.1 mg/kg/day chlorambucil orally, for 12 months or chlorambucil associated with 15 courses of plasma exchange, during the first four months of treatment. They were evaluated by a neuropathy disability score and nerve conduction studies. No difference was found between the two treatment groups. The average neuropathy disability score improved by 2.1 points from baseline (21.0 to 18.9) in the chlorambucil group and by 1.8 points (20.4 to 18.6) in the chlorambucil + plasma exchange group (P = 0.70). The mean motor nerve conduction velocity decreased from 20.0 to 18.2 m/s in the chlorambucil group and increased from 20.5 to 22.5 m/s in the chlorambucil + plasma exchange group (P = 0.51). A slight improvement of the sensory component of the neuropathy disability score (from 10.5 to 8.3) was noted in both groups (P = 0.01). At the end of the study and according to self evaluation, 15 patients--eight from the chlorambucil group and seven from the chlorambucil + plasma exchange group--reported clinical improvement, whereas 15--eight from the chlorambucil group and seven from the chlorambucil + plasma exchange group--reported clinical worsening. Neuropathy remained stable in the others. Thus plasma exchange seemed to confer no additional benefit in the treatment of polyneuropathy associated with monoclonal IgM.

Aged↗