Pulsed high-dose dexamethasone treatment of polyneuropathy associated with monoclonal gammopathy.
Explore the source record for details and available documents.
Aged↗
Biomedical subjects
N C Notermans
Publications and source records attributed to N C Notermans.
Indications for an immune-mediated etiology of idiopathic sensory neuronopathy.
To investigate immune mechanisms in the etiology of idiopathic sensory neuronopathy (ISN), we studied neurite outgrowth inhibition and antibody binding to neuronal tissue of serum from 4 patients with ISN. Rat dorsal root ganglion (DRG) cells were cultured in the presence of serum from ISN patients and controls. After 48 h of incubation, neurite outgrowth was quantified with a neurofilament ELISA. Serum from ISN patients significantly inhibited DRG neurite outgrowth compared to controls. ISN serum also strongly immunostained fixed cultured and cryostat rat DRG neurons (at dilutions up to 1:10,240), whereas serum from controls did not. Western blots showed unique binding patterns to DRG proteins in 3 ISN patients compared with controls, but a single band corresponding in all ISN patients was not found. The inhibitory effect of ISN serum on neurite outgrowth and the presence of circulating anti-DRG antibodies in the acute phase of the disease supports an immune-mediated pathogenesis of ISN.
Aged↗
Miyoshi-type distal muscular dystrophy. Clinical spectrum in 24 Dutch patients.
Miyoshi-type distal muscular dystrophy has now been found to be more frequent outside Japan than was previously thought. We studied 24 Dutch patients with Miyoshi-type distal muscular dystrophy and focused on its clinical expression and natural history, muscle CT-scans and muscle biopsy findings. Our study shows that Miyoshi myopathy is a heterogeneous, slowly progressive disorder. The disease starts with weakness and atrophy of the calves and progressively involves the proximal leg and hip muscles and, in a later stage the shoulder and upper arm muscles. After 10 years disease duration, one-third of the patients are dependent on wheelchairs for out-of-door transportation. Disease progression is related to disease duration and not to early age of onset of symptoms. Onset may be at any age and is asymmetrical in roughly half of the cases. Four cases had been initially diagnosed as idiopathic hyper-CK-aemia.
Adult↗
Differences between hereditary motor and sensory neuropathy type 2 and chronic idiopathic axonal neuropathy. A clinical and electrophysiological study.
To evaluate whether chronic idiopathic axonal polyneuropathy (CIAP) should be considered as hereditary motor and sensory neuropathy type 2 (HMSN type 2), we compared the clinical features of 48 patients with CIAP with those of 47 patients with HMSN type 2. In addition, we studied electrophysiological data in 20 patients with CIAP and in 20 patients with HMSN type 2. We found, in patients with HMSN type 2, that the initial symptoms were predominantly motor and that weakness and handicap were more severe and skeletal deformities more frequent, compared with those of CIAP patients. Electrophysiologically, the tibialis anterior muscle showed more denervation in patients with HMSN type 2, consistent with the predominance of motor symptoms. There was no important effect of age of onset on clinical features in HMSN type 2 patients. We conclude that in an individual patient with a sensory or sensorimotor idiopathic axonal polyneuropathy and no family history of polyneuropathies, the diagnosis HMSN type 2 is unlikely. However, if motor symptoms predominate, the diagnosis of HMSN type 2 should be considered.
Adult↗
Sjögren's syndrome in patients with chronic idiopathic axonal polyneuropathy.
OBJECTIVE: To assess the presence of symptoms and signs of Sjögren's syndrome in patients with otherwise idiopathic axonal polyneuropathy and to develop guidelines for the diagnostic approach with respect to Sjögren's syndrome in these patients. METHODS: Sixty five patients with axonal polyneuropathy in whom an aetiological diagnosis could not be made underwent (1) a standard interview focusing on ocular and oral sicca symptoms, (2) physical examination, (3) tests for objective assessment of keratoconjunctivitis sicca, (4) extensive serological investigations, and (5) a sublabial salivary gland biopsy. RESULTS: In forty nine patients a sublabial salivary gland (SSG) biopsy was performed, thereby completing the whole investigation for Sjögren's syndrome. Three of these 49 patients (all women) had an SSG biopsy specimen suggestive of Sjögren's syndrome, which, in combination with other symptoms and signs, led to a diagnosis of primary Sjögren's syndrome. CONCLUSIONS: None of the three patients with primary Sjögren's syndrome had spontaneously complained about sicca symptoms and the clinical neurological picture of them did not differ from the other patients in the study. Therefore, in patients with chronic idiopathic axonal polyneuropathy, especially in women, a systematic investigation for Sjögren's syndrome should be done, because the presence of Sjögren's syndrome may have implications for treatment and justifies a clinical follow up on a regular base.
Aged↗
Magnetic resonance imaging of the brachial plexus in patients with multifocal motor neuropathy.
We studied whether magnetic resonance (MR) imaging of the brachial plexus is useful to distinguish multifocal motor neuropathy (MMN) from lower motor neuron disease (LMND) and whether abnormalities resemble those of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We compared MR images of the brachial plexus of nine patients with MMN with scans from five patients with CIDP, eight patients with LMND, and 174 controls. In two patients with MMN, and in three patients with CIDP, the MR images showed an increased signal intensity on the T2-weighted images of the brachial plexus. Two other patients with MMN demonstrated a more focal, increased signal intensity on the T2-weighted images, occurring in one patient only in the axilla, and in the other patient in the axilla and in the ventral rami of the roots. MR images of the brachial plexus of eight patients with LMND were normal. The distribution of the MR imaging abnormalities corresponded with the distribution of symptoms of the patients: asymmetrical in MMN and symmetrical in CIDP. These findings demonstrate that MR imaging abnormalities of the brachial plexus in patients with MMN resemble those seen in CIDP and may be useful to distinguish MMN from LMND.
Adult↗
Cisplatin-induced autonomic neuropathy: does it really exist?
The neurotoxic side-effects of cisplatin affect predominantly the large, myelinated fibres of peripheral nerves, leading to a sensory neuropathy. Several reports of cisplatin-associated autonomic neuropathy have been published. Autonomic dysfunction however, is caused by a neuropathy of small unmyelinated nerve fibres. By using the absolute pupil diameter as a parameter of autonomic nervous system function, we studied autonomic neuropathy in the eye of cisplatin-intoxicated rats. In addition, we examined autonomic cardiovascular function by measuring the change in heart rate (HR) and mean arterial blood pressure (MAP) in response to intravenous phenylephrine (PHE) and tyramine (TYR). No significant differences in mean pupil diameter developed in cisplatin-intoxicated rats (n = 12) in the course of 9 weeks (total cumulative dose cisplatin 18 mg/kg) compared with normal controls (n = 9) MANOVA, F1,19 = 0.88, P < 0.36). The PHE- and TYR-induced changes in MAP and HR were virtually the same in cisplatin-intoxicated rats when compared with normal controls. We conclude that cisplatin probably does not cause autonomic dysfunction, at least not in rats, in doses commonly used and which are known to cause a peripheral sensory neuropathy.
Analysis of Variance↗
Response to intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy with only sensory symptoms.
In an open prospective study we analysed the effect of treatment with intravenous immunoglobulin (IvIg) in three patients with clinically pure sensory neuropathy, two of whom met the clinical, electrophysiological, pathological and cerebrospinal fluid research criteria of the American Academy of Neurology for chronic inflammatory demyelinating polyneuropathy. In all patients, subclinical signs of demyelination were present in motor nerves. Treatment with IvIg resulted in improvement of neurological functions in all three patients and in improvement of the disability score in two of them.
Demyelinating Diseases↗
Chronic idiopathic axonal polyneuropathy. Comparison of patients with and without monoclonal gammopathy.
In order to study whether axonal polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS) is a distinct entity, we prospectively studied the clinical, electrophysiological and pathological features of 16 patients with chronic idiopathic axonal polyneuropathy (CIAP) with MGUS (CIAP-MGUS) and compared them with those of 71 patients who had CIAP without MGUS. In patients with CIAP-MGUS the arms were more frequently affected and the disability was worse. On electromyography there was more evidence of denervation in patients with CIAP-MGUS. All other clinical symptoms, signs, nerve conduction parameters and nerve biopsy findings, showed no differences between both groups. Antibodies against myelin associated glycoprotein (MAG), GMI-ganglioside and chondrotoin sulphate were not present. Only one patient with an immunoglobulin M (IgM)-MGUS and a sensory neuropathy had antibodies against sulphatide. In conclusion, axonal polyneuropathy in patients with and without MGUS are essentially indistinguishable rather than different, suggesting that the MGUS may be coincidental in most patients.
Axons↗
Monoclonal gammopathy and neuropathy.
There is a known coexistence between polyneuropathy and monoclonal gammopathy (immunoglobulin M, immunoglobulin G, immunoglobulin A). Antibodies to several glycoconjugates of the peripheral nervous system have been found in 50-65% of patients with immunoglobulin M monoclonal proteins, and distinct clinical syndromes have been recognized. However, if no antibodies are found, a relationship between monoclonal protein and polyneuropathy is still debatable. Therapeutic intervention is generally directed at removing the autoantibodies by reducing the number of monoclonal B-cells.
Aged↗
Intermittent cyclophosphamide and prednisone treatment of polyneuropathy associated with monoclonal gammopathy of undetermined significance.
In an open prospective study, we analyzed the effect of cyclophosphamide (300 mg/m2 body surface daily for 4 days) combined with prednisone (40 mg/m2 body surface daily for 5 days) at 4-week intervals during 6 months in 16 patients with polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS). Eleven patients had an IgM-MGUS and five an IgG-MGUS. During a follow-up period of 3 years, eight patients had improvement and six patients stabilized, based on quantitative neurologic examination, the Rankin disability scale, and electrophysiologic studies. These 14 patients had neuropathy with demyelinating and axonal features. One patient with a purely axonal neuropathy had deterioration despite therapy. One other patients developed severe leukopenia as side effect of cyclophosphamide, necessitating withdrawal of treatment. A difference in response was not present in patient with IgM- or IgG-MGUS, nor in patients with or without autoantibodies against myelin-associated glycoprotein. Nine patients had a bone marrow biopsy before and 1 year after treatment. In eight patients, the monoclonal lymphoid IgM or plasma cell IgG infiltration decreased, while in four the monoclonality disappeared after treatment. In the patient who had neurologic deterioration, repeated bone marrow biopsy showed deposits of amyloid. In conclusion, short-term treatment with intermittent cyclophosphamide and prednisone may have a long-term favorable effect in patients with demyelinating polyneuropathy associated with MGUS.
Aged↗
[The neurologic differential diagnosis of unilateral headache and facial pain].
The most frequent and relevant neurologic causes of unilateral headache and facial pain are discussed. Symptomatic headache syndromes, caused by an underlying neurologic disease or structural lesion, are distinguished from idiopathic (essential) pain syndromes, which are characterized by a typical clinical presentation and the absence of a known etiology or pathogenesis.
Diagnosis, Differential↗
Chronic idiopathic axonal polyneuropathy.
Explore the source record for details and available documents.
Axons↗
Neural influences on the iris of diabetic rats and effect of oculomotor nerve crush.
Using an animal model where the pupil diameter of the eye in anaesthetized and dark-adapted rats serves as a parameter of autonomic function, we studied the functional recovery of the parasympathetic nerve fibres in the oculomotor nerve after a crush lesion in rats with streptozotocin-induced diabetes compared with normal controls. Prior to the crush lesion, diabetic rats develop significantly (P < 0.001) smaller pupils compared with controls, and this occurs early in the course of the diabetes mellitus. As the difference in pupil diameter between control and diabetic rats persists immediately after the crush lesion, when the nervous control of the pupil is entirely due to sympathetic nerves, we suggest that the reduction in pupil diameter is due to a sympathetic neuropathy. Furthermore, we show that the functional recovery of the parasympathetic input to the iris after a crush lesion of the oculomotor nerve is not as good in diabetic rats as it is in normal control rats.
Animals↗
Beneficial effect of an ACTH4-9 analogue on experimentally induced diabetic autonomic neuropathy in the eye of the rat under general anaesthesia.
While peripheral polyneuropathy is a well-known complication in diabetes mellitus, and the subject of a great deal of study, the clinical importance of autonomic diabetic neuropathy is increasingly recognised. Using an animal model, where the pupil diameter of the eye serves as a parameter of autonomic function, we produced an age and weight curve of pupil diameter and studied the development of autonomic neuropathy in rats with streptozotocin-induced diabetes. We show that diabetic rats develop significantly (P < 0.009) smaller pupils compared with controls, most probably due to a defective sympathetic input, caused by sympathetic neuropathy. Treatment with the neurotrophic peptide Org 2766, a synthetic ACTH4-9 analogue, prevents the occurrence of this sympathetic neuropathy, as the pupil diameters in the ACTH4-9 analogue-treated group are significantly (P < 0.05) larger than the pupils of placebo-treated rats, and are comparable to the pupil diameters of the rats in the control group.
Adrenocorticotropic Hormone↗
[Course of chronic idiopathic polyneuropathy during middle age and older: 2-year follow-up study].
OBJECTIVE: To study the clinical features and natural course of idiopathic polyneuropathy. DESIGN: Prognostic and descriptive cohort study. SETTING: University Hospital, Utrecht, the Netherlands. METHOD: 75 patients (46 men, 29 women) with a mean age of 56.5 years at onset of symptoms were clinically investigated during two years. RESULTS: The features of chronic idiopathic polyneuropathy were heterogeneous. Clinically, 44 patients had a sensorimotor, 29 patients a sensory and 2 patients a motor polyneuropathy. Electrophysiological and nerve biopsy studies were compatible with an axonal polyneuropathy. The overall clinical course was slowly progressive. None of the patients became severely disabled. After one year of follow up one patient proved to have a hereditary neuropathy. In the other 74 patients no cause for the polyneuropathy was found even after 2 years of follow-up. CONCLUSION: Chronic idiopathic polyneuropathy is a heterogeneous entity, leading to only slight disability. The neuropathy is due to axonal degeneration.
Age Factors↗
Temperature dependence of nerve conduction and EMG in neuropathy associated with gammopathy.
The effects of warming on nerve conduction variables and electromyography were studied in 15 patients with a polyneuropathy associated with monoclonal gammopathy of undetermined significance. In each patient median nerve (motor, sensory) and tibial nerve (motor) conduction parameters were measured before and after warming in water at 36 degrees C. Warming: (1) increased the conduction velocity (CV); (2) decreased the distal motor latency, amplitude, and duration of the compound muscle or nerve action potential; and (3) caused fibrillations to appear in 1 patient. The increase in CV with temperature depended upon the CV after warming: the lower this CV, the smaller the increase in CV with temperature (delta CV/delta T). Correction of median nerve motor CV before warming with 2.2 m/s per degree C yielded CV values which were higher than the CV values after warming, because in most patients delta CV/delta T was less than 2.2 m/s per degree C. Because of differences in delta CV/delta T values, it is more accurate to warm the extremity than to correct for temperature.
Aged↗
Segmental fasciculations as a late sequel of spinal cord injury.
In two patients with a history of spinal cord injury many years ago fasciculations developed in muscles belonging to previously damaged segments. In both patients MRI of the cervical spine showed an area of abnormal signal intensity representing a cavity, near the level of the neurological abnormalities and the cord injury. During a 4-year follow-up period no new neurological or MRI abnormalities were detected. A hypothesis for the genesis of fasciculations following spinal cord injury is presented.
Adult↗