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Biomedical subjects

H Franssen

Publications and source records attributed to H Franssen.

At least 55 records · Page 3Linked to original sources
PubMed1998

The long-term effect of intravenous immunoglobulin treatment in multifocal motor neuropathy.

We studied the long-term effect of intravenous immunoglobulin (IV Ig) treatment in seven patients with multifocal motor neuropathy. In six patients, treatment with a full IV Ig course (0.4 g/kg for 5 consecutive days) improved muscle strength but for not longer than 12 weeks. These patients received IV Ig maintenance treatment consisting of one infusion every week for 2-4 years. One patient in whom the effect of the initial full IV Ig treatment lasted for more than 1 year received incidental IV Ig treatment when muscle strength deteriorated. In all patients IV Ig treatment had a beneficial effect on most muscle groups during the follow-up period. However, in three of the seven patients muscle strength deteriorated during IV Ig maintenance treatment in four of the 28 muscle groups that had initially shown an improvement of muscle strength after the start of IV Ig treatment, and in two muscle groups with normal strength at the start of IV Ig treatment. The electrophysiological follow-up studies indicated that there was an improvement of conduction block, but also that there were new sites of conduction block and ongoing axonal degeneration during IV Ig maintenance treatment.

Adult↗
PubMed1997

Multifocal motor neuropathy.

We present a review of the literature on multifocal motor neuropathy (MMN), a rare neurological disorder which has features in common with both chronic inflammatory demyelinating neuropathy and lower motor neuron disease. Clinically, MMN is characterised by slowly progressive asymmetrical limb weakness, usually most prominent in the forearms. Weakness may be associated with muscle wasting, fasciculations and decreased tendon reflexes. Serum anti-GM1 ganglioside antibody titres may be increased. The diagnostic hallmark of MMN is the electrophysiological demonstration of persistent localised motor conduction blocks, with otherwise normal or near-normal motor and sensory conduction velocities. The pathogenesis of MMN has not been elucidated completely. There is, however, substantial evidence for an autoimmune mechanism. Based on the possible involvement of the immune system in the pathogenesis of MMN the therapeutic efficacy of several immunomodulatory drugs has been tested. Treatment of MMN patients with human immunoglobulin or cyclophosphamide is usually followed by a marked improvement of strength. The finding that MMN is a potentially treatable disorder underscores the importance of distinguishing MMN from lower motor neuron disease, for which no effective therapy is currently available.

Biopsy↗
PubMed1997

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↗
PubMed1997

Intravenous immunoglobulin treatment in lower motor neuron disease associated with highly raised anti-GM1 antibodies.

The effect of intravenous immunoglobulin (IVIg) treatment was studied in five patients with lower motor neuron disease associated with highly raised anti-GM1 antibodies but without evidence of conduction block on neurophysiological examination. The patients received IVIg treatment (0.4 g/kg for five consecutive days) in an open study. Only one patient responded to IVIg treatment, which was confirmed in a double blind, placebo controlled study (two placebo treatments and two IVIg treatments in a randomised order). However, after six months of maintenance IVIg treatment (0.4 g/kg weekly) muscle weakness gradually deteriorated below pretreatment levels despite continued treatment. It is concluded that the presence of raised anti-GM1 antibodies does not identify a subgroup of patients with lower motor neuron disease who respond to IVIg treatment and although some patients with lower motor neuron disease may initially respond, IVIg treatment does not seem to be sufficient as long term treatment.

Adolescent↗
PubMed1997

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↗
PubMed1996

Modification of phytohormone response by a peptide encoded by ENOD40 of legumes and a nonlegume.

The gene ENOD40 is expressed during early stages of legume nodule development. A homolog was isolated from tobacco, which, as does ENOD40 from legumes, encodes an oligopeptide of about 10 amino acids. In tobacco protoplasts, these peptides change the response to auxin at concentrations as low as 10(-12) to 10(-16)M. The peptides encoded by ENOD40 appear to act as plant growth regulators.

Amino Acid Sequence↗
PubMed1996

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↗
PubMed1996

Source modeling of esophageal evoked potentials.

Evoked potentials can be recorded from the scalp after stimulation of the esophagus by balloon distension. The purpose of this study was to estimate the number and localization of sources contributing to the esophageal evoked potential (EEP). The EEP was recorded from 32 scalp electrodes in 5 healthy subjects. Spatio-temporal dipole modeling was performed in the time interval from 185 msec to 525 msec after stimulation (mean values). The EEP was best explained by the combined activity of 1 dipole located relatively high in the midline and 2 lateral dipoles. Given the anatomical projection of esophageal sensory fibers and the location of these dipoles the sources were probably located in the cingulate gyri and insular cortex. There was no evidence that sources in the lower brain-stem contributed to the scalp recorded EEP.

Adult↗
PubMed1996

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↗
PubMed1996

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↗
PubMed1995

A new variant of sensory ataxic neuropathy with autosomal dominant inheritance.

We describe a Dutch family with sensory ataxia in two generations, late onset of symptoms (over the age of 40 years) and slow progression. Clinical, electrophysiological and sural nerve biopsy findings revealed a sensory polyneuropathy due to axonal degeneration of myelinated nerve fibres in four of five investigated siblings. Other neurological abnormalities in the affected family members consisted only of mild eye movement disturbances, probably due to cerebellar involvement. Five other family members were investigated and found unaffected. As the disease is inherited from the affected father to his sons and daughters, this is the first description of a probably autosomal dominant form of late onset hereditary sensory neuropathy with predominant sensory ataxia and minor other neurological abnormalities.

Cerebellar Ataxia↗
PubMed1995

Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double blind, placebo controlled study.

The effect of high dose intravenous immunoglobulin (IVIg) treatment was studied in six patients with multifocal motor neuropathy (MMN). All patients responded to treatment (0.4 g/kg for five consecutive days) in an open trial. The effect of IVIg treatment was confirmed for each patient in a single patient, double blind, placebo controlled trial. Four patients received two IVIg treatments and two placebo treatments, and two patients received one IVIg and one placebo treatment in a randomised order. Five out of six patients responded to IVIg but not to placebo. One patient responded to IVIg in the same manner as to placebo treatment. Thus IVIg treatment can lead to improvement of muscle strength in patients with MMN.

Adult↗
PubMed1995

Improvement of multifocal motor neuropathy during long-term weekly treatment with human immunoglobulin.

In multifocal motor neuropathy (MMN), little is known of the long-term effect of human immunoglobulin (HIG) infusions as a maintenance therapy. We report a patient who improved after an initial HIG infusion of 2 g/kg and continued to improve both clinically and electrophysiologically during maintenance HIG treatment consisting of one infusion of 0.4 g/kg every week. Our findings suggest that maintenance HIG therapy may lead to further improvement of MMN, and that more frequent HIG infusions at a lower dosage may be more effective.

Adult↗
PubMed1994

[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↗
PubMed1994

Transcranial magnetic stimulation in patients with cervical spondylotic myelopathy: clinical and radiological correlations.

Motor evoked potentials (MEPs) were studied in 28 patients with cervical spondylotic myelopathy. MEPs after cortical stimulation were abnormal in 27 patients, the responses in the leg muscles being affected the most often. Clinically asymptomatic motor lesions were detected in 7 patients (25%). The central motor conduction time (CMCT) for the abductor digiti minimi muscles correlated significantly with the clinical disability, whereas the radiological findings did not correlate with the clinical and neurophysiological parameters. In 9 patients MEPs were also recorded in the biceps muscles. The 7 patients with an abnormal CMCT for the biceps muscles had the most severe stenosis at the C-4-C-5 level or higher. The 2 patients with normal MEPs of the biceps muscles both had a stenosis at the C-5-C-6 level. The results of this study suggest that MEPs are useful for detecting spinal cord dysfunction and for localizing the level of the lesion. Some recommendations regarding the possible use of MEPs in the clinical evaluation of patients with cervical spondylotic myelopathy are given.

Adult↗
PubMed1994

Nerve conduction and temperature: necessary warming time.

The purpose of this study was to estimate the time needed to warm an extremity prior to measuring nerve conduction. In 8 normal subjects tibial and sural nerve conduction variables were measured during cooling and warming of the leg in water of 18 degrees C and 36 degrees C, respectively. During cooling, nerve conduction velocity (NCV) decreased and distal motor latency (DML), duration, and area of the compound muscle action potentials (CMAP), and compound nerve action potentials (CNAP) increased. The reverse occurred during warming. During cooling or warming the change in these variables became progressively smaller with time. The time course could therefore be described by an exponential relation, the parameters of which were determined. On the basis of these data, correlations were calculated between the skin temperature at the start of the investigation and the warming time needed to obtain a good estimate of nerve conduction variables at 36 degrees C. The use of correction factors, instead of actual warming, yielded acceptable errors only for NCV and not for the other variables.

Action Potentials↗