Biomedical subjects

H Franssen

Publications and source records attributed to H Franssen.

At least 37 records · Page 2Linked to original sources

PubMed2002

Is cardiovascular disease a risk factor in the development of axonal polyneuropathy?

OBJECTIVES: To determine if cardiovascular disease may be a risk factor in the development of chronic idiopathic axonal polyneuropathy (CIAP). METHODS: In this incidence case-control study, the prevalence of cardiovascular disease and risk factors in 97 patients with CIAP (mean age 67.5 (SD 7.9) years) and the prevalence of neuropathic features in 97 patients with peripheral arterial disease (PAD) (mean age 67.1 (SD 7.3) years) were investigated. The results were compared with those for 96 age and sex matched controls without diagnosed PAD or polyneuropathy (mean age 67.5 (SD 9.1) years). In a randomly chosen subgroup of 23 patients with CIAP, 42 patients with PAD, and 48 controls, an electrodiagnostic investigation was performed. RESULTS: Patients with CIAP more often had manifest cardiovascular disease and cardiovascular risk factors than controls (stroke 18% v 6% of patients, odds ratio (OR) 3.2 (95% confidence interval (CI) 1.8 to 5.9); heart disease 29% v 15%, OR 2.4 (95% CI 1.2 to 4.9); family history of cardiovascular disease 42% v 21%, OR 2.8 (95% CI (1.5 to 5.2); hypertension 56% v 39%, OR 2.0 (95% CI 1.1 to 3.6); hypercholesterolaemia 46% v 21%, OR 3.3 (95% CI 1.5 to 7.3); current smoking 38% v 23%, OR 2.1 (95% CI 1.1 to 3.9)). The prevalence of cardiovascular disease and cardiovascular risk factors was lower than in patients with PAD. Patients with PAD more often had polyneuropathy than controls (15% v 5%, OR 3.3 (95% CI 1.1 to 10.0)). There was a trend towards lower nerve conduction velocities and lower amplitudes on electrodiagnostic investigation compared with controls. CONCLUSION: This study shows that cardiovascular disease and CIAP often coexist, and therefore cardiovascular disease may be a cofactor in the development of CIAP.

Aged↗

PubMed2001

[An infant with pain and meningeal irritation due to Guillain-Barré syndrome].

In a five-month-old girl, who for a week had cried and displayed a relapse in the motor development, generalised hypotonia and meningism were found. Eventually, the results of the cerebrospinal fluid analysis and electromyography indicated Guillain-Barré syndrome. She was treated with immunoglobulins and made a quick recovery. Guillain-Barré syndrome is an acute or subacute inflammatory demyelinating polyradiculoneuropathy that can occur at any age. In children, the classic symptoms such as a flaccid paralysis and areflexia are not always predominant. Instead, pain is often the most prominent symptom, along with meningism. These symptoms are often insufficiently recognised in practice and as a result of this, delays in the diagnosis of this potentially life-threatening disease often occur.

Diagnosis, Differential↗

PubMed2001

MRI of the brachial plexus in polyneuropathy associated with monoclonal gammopathy.

On magnetic resonance (MR) imaging of the brachial plexus increased signal intensity and swelling of the brachial plexus has been found in chronic inflammatory demyelinating polyneuropathy (CIDP). Whether these proximal abnormalities are also present in the distal polyneuropathy associated with monoclonal gammopathy is unknown. Therefore, we performed MR imaging of the brachial plexus in 21 patients with polyneuropathy associated with IgM monoclonal gammopathy (11 IgM with anti-MAG antibodies, 10 IgM without anti-MAG antibodies). For comparison we studied 9 patients with polyneuropathy associated with IgG monoclonal gammopathy and 8 patients with CIDP. Among the 30 patients with monoclonal gammopathy, 24 patients had demyelinating polyneuropathy. Among these 24 patients, there was increased signal intensity of the brachial plexus on the T2-weighted images regardless of whether clinical deficits were generalized or purely distal in location. No association was found with the isotype of the monoclonal gammopathy. Of the 8 patients with CIDP, 5 had brachial plexus abnormalities. None of the 6 patients with axonal polyneuropathy associated with monoclonal gammopathy had such abnormalities. Thus, MR imaging of the brachial plexus shows that the distal demyelinating polyneuropathy associated with monoclonal gammopathy is more generalized than presumed.

Adult↗

PubMed2001

Diagnostic value of sural nerve demyelination in chronic inflammatory demyelinating polyneuropathy.

The objective of the study was to determine the diagnostic value of features of demyelination and inflammation in sural nerve biopsy specimens of patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The features of (i) demyelination, (ii) axonal de- and regeneration and (iii) inflammation were investigated by measuring the number of onion bulbs, g ratio (axon diameter/total nerve fibre diameter), myelinated nerve fibre density, number of clusters and endoneurial area in 21 patients with CIDP, as well as in 13 patients with chronic idiopathic axonal polyneuropathy (CIAP) and six autopsy controls. In addition, teased fibres were classified and lengths of internodes measured. We found no difference in demyelinating features between patients with CIDP and CIAP, as the percentage of fibres with segmental de- and remyelination and the number of onion bulbs were similar in both polyneuropathy groups. The g ratio, expected to be higher in a demyelinating disease due to thinner myelin sheaths, was significantly lower in CIDP than CIAP. Evidence for axonal degeneration was found in both CIDP and CIAP, as both showed a decrease in myelinated nerve fibre density. There was no evidence of endoneurial oedema in CIDP, as the endoneurial area did not differ between CIDP, CIAP and the autopsy controls. Although significant differences of features of demyelination, axonal degeneration and inflammation were found in sural nerve biopsy specimens, there was a considerable overlap between abnormalities in CIDP and CIAP patients. In the majority of patients, quantitative analysis of light microscopical abnormalities in sural nerves was similar in CIDP and CIAP. Therefore, a sural nerve biopsy is of limited diagnostic value in CIDP.

Adult↗

PubMed2000

Treatment of multifocal motor neuropathy with interferon-beta1A.

Nine patients with multifocal motor neuropathy who had previously responded favorably to IV immunoglobulins (IVIg) were treated with interferon-beta1a. Muscle strength and disability were evaluated. In six patients there was no effect of treatment. Four patients deteriorated in such a way that IVIg had to be restarted during the study. Three patients showed an improvement that was more pronounced than on IVIg. These patients had a shorter disease duration and were less affected clinically and electrophysiologically than those who did not respond.

Action Potentials↗

PubMed2000

Multifocal inflammatory demyelinating neuropathy: a distinct clinical entity?

BACKGROUND: Several patients have been reported with an asymmetric sensory or sensorimotor demyelinating neuropathy not fulfilling the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy or multifocal motor neuropathy. OBJECTIVE: To present the clinical, electrophysiologic, radiologic, and pathologic features of six patients with an asymmetric sensory or sensorimotor demyelinating neuropathy. RESULTS: All six patients were initially affected in only one limb; in four patients the neuropathy progressed to other limbs in an asymmetric fashion during several years. On electrophysiologic examination, evidence of multifocal demyelination and conduction block in motor and sensory nerves was found in all patients. MRI of the brachial plexus revealed swollen nerves and an increased signal intensity on T2-weighted imaging in four patients. A biopsy sample taken from the brachial plexus of one patient revealed evidence of inflammation. All patients showed a beneficial response to IV immunoglobulin treatment. Thirty-four similar patients have been reported previously, many of whom were initially diagnosed as having various other (nontreatable) diseases. CONCLUSIONS: The authors propose calling this neuropathy "multifocal inflammatory demyelinating neuropathy" and considering it as a distinct clinical entity to facilitate early diagnosis of this treatable disorder.

Adult↗

PubMed2000

Diagnostic criteria for demyelinating polyneuropathy associated with monoclonal gammopathy.

In order to define diagnostic criteria for the demyelinating polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS), we compared 30 patients with idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) without a monoclonal gammopathy, with 29 patients with polyneuropathy associated with MGUS. All 59 patients fulfilled research criteria for CIDP. In the patients with MGUS, sensory symptoms and signs predominated, there was usually no cranial nerve involvement, and the neuropathy was symmetrical with a slowly progressive course. On electrophysiological examination, an abnormal median nerve sensory action potential in combination with a normal sural nerve action potential (AMNS) was not found. In idiopathic CIDP patients, a preceding infection was frequent, motor features predominated, there was often cranial nerve involvement, the neuropathy could be asymmetrical, and AMNS was frequently found. Diagnostic criteria for demyelinating polyneuropathy associated with MGUS are presented.

Action Potentials↗

PubMed2000

Unmyelinated nerve fiber degeneration in chronic inflammatory demyelinating polyneuropathy.

To determine whether unmyelinated nerve fibers escape degeneration as one might expect in an immune response exclusively directed at myelin, we performed a morphometric examination of unmyelinated axons and myelinated nerve fibers in sural nerve biopsy specimens of 14 patients with a chronic inflammatory demyelinating polyneuropathy (CIDP) and of 12 age-matched normal controls. The numbers of unmyelinated axons, myelinated nerve fibers, denervated Schwann cell units and collagen pockets were quantified and related to the clinical and electrophysiological data of the patients with CIDP. In 4 patients with a rapid onset of the neuropathy and a highly elevated CSF protein, the numbers of both unmyelinated axons and myelinated nerve fibers were decreased equally. In 8 patients we found that the unmyelinated axons were relatively spared compared with the loss of myelinated nerve fibers. In these patients, however, the presence of denervated Schwann cell units and of collagen pockets was increased. We conclude that unmyelinated nerve fibers are affected in patients with CIDP.

Adult↗

PubMed2000

Multifocal motor neuropathy: diagnostic criteria that predict the response to immunoglobulin treatment.

As multifocal motor neuropathy (MMN) is a potentially treatable disorder, its differentiation from lower motor neuron disease is important. Evidence of conduction block (CB) is considered one of the relevant criteria for the diagnosis of MMN. Strict criteria for CB may lead to underdiagnosis of MMN, however. Using a standardized examination, we studied the clinical, laboratory, and electrophysiological characteristics of 37 patients presenting with a lower motor neuron disorder and electrophysiological features compatible with segmental demyelination. We propose a set of clinical, laboratory, and electrophysiological criteria for the diagnosis of MMN, which has been verified by follow-up and response to treatment with intravenous immunoglobulins. Based on the clinical, laboratory, and electrodiagnostic features, 21 patients were diagnosed with definite MMN (17 responders), 7 were diagnosed with probable MMN (5 responders), and 9 were diagnosed with possible MMN (1 responder). Age at onset, the number of affected limb regions, and the number of patients with a creatine kinase level greater than 180 U/L were significantly lower in responders than in nonresponders. Elevated anti-GM1 antibodies and definite CB were found significantly more often in responders. The proposed diagnostic criteria may be useful in clinical practice and therapeutic trials.

Adult↗

PubMed1999

Diagnostic value of myotactic reflexes in axonal and demyelinating polyneuropathy.

In 11 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and 11 patients with chronic idiopathic axonal polyneuropathy (CIAP), absent myotatic reflexes were significantly associated more often with CIDP than with CIAP, an absent biceps-reflex having the highest sensitivity and specificity for the diagnosis of CIDP. In CIDP, the latencies of electromyographically recorded myotatic reflexes often indicated demyelination, notwithstanding normal clinically assessed myotatic reflexes. Myotatic reflexes may therefore be useful for the distinction between axonal and demyelinating polyneuropathy.

Aged↗

PubMed1999

The diagnostic value of sural nerve T cells in chronic inflammatory demyelinating polyneuropathy.

BACKGROUND: T-cell infiltrates in sural nerve biopsy specimens of patients with inflammatory neuropathies have been reported, suggesting a role for T cells in the pathogenesis, but the specificity of the presence and localization of sural nerve T cells in chronic inflammatory demyelinating polyneuropathy (CIDP) is unknown. OBJECTIVE: To study the diagnostic value of the number and distribution of sural nerve T cells in CIDP. METHODS: We performed a quantitative immunohistochemical examination of T cells in sural nerve biopsy specimens taken from 23 patients with a CIDP and compared them with sural nerves of 15 patients with a chronic idiopathic axonal polyneuropathy (CIAP), 5 patients with a vasculitic neuropathy, and 10 normal controls. RESULTS: T cells were found in sural nerves of all CIDP patients as well as in all disease and normal controls. Only six CIDP patients had increased numbers and densities of T cells compared with CIAP patients and controls. Based on the distribution of endoneurial or epineurial T cells, it was not possible to differentiate CIDP patients from CIAP patients or normal controls. In patients and controls perivascular epineurial T cells predominated. Increased numbers and densities of sural nerve T cells in patients with CIDP were associated with female sex, a more severe disease course, worse outcome, highly elevated CSF protein level, and a larger sural nerve area, but not with loss of myelinated nerve fibers in the sural nerve biopsy sample or demyelinating features on electrophysiologic examination. CONCLUSIONS: In the majority of CIDP patients, the number and distribution of T cells in sural nerve biopsy samples were similar to patients with noninflammatory neuropathies and normal controls. Only large numbers of sural nerve T cells are specific for inflammatory neuropathies and therefore of diagnostic value for CIDP.

Adult↗

PubMed1999

The influence of temperature on conduction block.

In nerve conduction studies, it may be difficult to distinguish conduction block from increased temporal dispersion. We assessed whether a better distinction is possible by warming, because this increases the number of blocked nerve fibers and decreases temporal dispersion. We investigated 11 patients with conduction block in the median or ulnar nerve. Motor conduction was studied at 25 degrees C and at 40 degrees C. The amplitude reduction and area reduction were significantly greater at 40 degrees C than at 25 degrees C. Significantly more nerve segments fulfilled criteria for conduction block at 40 degrees C than at 25 degrees C. The duration prolongation was slightly smaller at 40 degrees C. These results indicate that, in demyelinating neuropathies, an increase in temperature increases the number of blocked nerve fibers and decreases temporal dispersion. Conduction block may therefore be missed when the investigated limb is too cold. Based on our study, we also propose a criterion for conduction block that is based on the influence of temperature.

Adult↗

PubMed1999

Motor and somatosensory evoked potentials in asymptomatic spondylotic cord compression.

To assess whether electrophysiological tests are of use in differentiating between patients with asymptomatic cervical stenosis and patients with clinical evidence of myelopathy, we studied motor evoked potentials (MEPs) to magnetic brain stimulation and somatosensory evoked potentials (SEPs) in patients with asymptomatic cervical cord compression and compared the results to healthy age-matched controls. The MEPs were normal in 23 of 25 patients and SEPs in 22 of 23 patients. Thus, MEPs and SEPs are normal in most cases of asymptomatic cervical stenosis. As previous studies have shown MEPs, and to a lesser extent SEPs, to be sensitive in the detection of spondylotic myelopathy, our data indicate that MEP and SEP may be clinically useful for differentiating patients with cervical stenosis who have myelopathy from those who have not.

Adult↗

PubMed1999

Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: a long-term follow-up study.

This long-term follow-up study examined patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and only sensory symptoms at first presentation, with emphasis on the development of motor symptoms and long-term disability. From all CIDP patients referred to our Department between 1987 and 1995, seven had only sensory symptoms at first clinical presentation. These were investigated according to a standard protocol, including a quantified clinical neurological examination and nerve conduction studies. The mean duration of the disease before weakness developed was 3.1 years, but varied considerably (0.8-6.3 years). At follow-up, weakness developed in five patients and persisted in three of them. Five patients were not seriously incapacitated by their disease (Rankin 1 or 2), four of them being in remission now and one showing a very slow progression of disease. Two patients were moderately disabled (Rankin 3); one had severe persistent sensory ataxia and only weakness during relapses and one had stepwise progression and moderate weakness. Motor nerve conduction studies revealed that the most notable worsening in the entire group of patients was a decrease in distal compound muscle action potential amplitudes, indicating the development of distal conduction block or axonal degeneration. These findings show that CIDP with only sensory symptoms is a transient clinical stage that precedes the appearance of weakness in about 70% of patients. The long-term prognosis does not differ from that of patients with CIDP who have weakness at the beginning of the disease.

Adult↗

PubMed1999

The influence of temperature on nerve conduction in patients with chronic axonal polyneuropathy.

OBJECTIVES: To determine whether the increase in conduction velocity with temperature (deltav/deltaT) is decreased in axonal polyneuropathy and to compare methods to account for low limb temperature in electroneurography. METHODS: Median nerve motor and sensory conduction and tibial nerve motor conduction were measured in 19 patients with chronic idiopathic axonal polyneuropathy at baseline temperature, after warming at 37 degrees C, and after cooling at 25 degrees C. Deltav/deltaT was determined, using the skin temperature difference and the presumed nerve temperature difference of 12 degrees C. The baseline conduction velocity was corrected for 37 degrees C, using mean deltav/deltaT values for normal subjects taken from the literature. RESULTS: Deltav/deltaT was positively correlated with the conduction velocity after warming at 37 degrees C: the lower the conduction velocity, the lower the deltav/deltaT. In nerves with a slow conduction velocity, deltav/deltaT was often zero. As slower conducting nerve fibers have smaller deltav/deltaT values, the decreased deltav/deltaT values in our study are probably related to loss of fast conducting fibers. The corrected conduction velocity was usually faster and considerably less often abnormal than the conduction velocity after warming at 37 degrees C. The latter can be considered the gold standard. CONCLUSION: Deltav/deltaT may be reduced in axonal polyneuropathy. The correction method is, therefore, not suitable to account for low limb temperature, as the conduction velocity is overestimated.

Aged↗

PubMed1998

Polymorphisms in the promoter of the tumor necrosis factor-alpha gene in coal miners.

Tumor necrosis factor-alpha (TNF) is recognized as a central mediator of mineral dust-induced lung fibrosis, and genetic polymorphisms of the TNF promoter have been reported to influence levels of TNF production. To assess whether polymorphisms within the TNF promoter gene are associated with susceptibility to coal workers' pneumoconiosis (CWP), the DNA of 78 coal miners was typed for G-to-A transitions at positions -238 and -308. Our results show that frequency of A-308 genotype (T2) is significantly overpresented in coal miners with CWP (50%), as compared with miners without CWP (25%) and controls (29%). After correction for cumulative dust exposure and smoking, the A-308 transition genotype is still associated with the presence of CWP (OR = 3.0, 95% CI = 1.0-9.0). Both A-238 and A-308 transition genotypes were related to TNF release from endotoxin-stimulated blood monocytes; only the A-238 transition and not the A-308 transition was associated to coal dust-induced TNF release. In summary, this study shows that the A-308 transition is related to CWP, but this relation is not paralleled by a different TNF release in this genotype. A larger number of patients coupled to frequent TNF release are required to evaluate genotype screening to estimate individual health risks for effects of coal mine dust exposure.

Base Sequence↗

PubMed1998

Decrease in nerve temperature: a model for increased temporal dispersion.

A decrease in nerve temperature causes a proportional decrease in conduction velocity which, in percentage terms, is equal for all nerve fibers. The absolute decrease in conduction velocity is larger for faster conducting nerve fibers. This results in a compression and a shift to lower values of the conduction velocity distribution and an increase in temporal dispersion. The purpose of this study was to determine if these effects could be detected by a combination of two collision techniques designed to obtain the motor conduction velocity distribution and refractory period distribution. In 12 healthy volunteers we measured the conduction velocity distribution in the median nerve at nerve temperatures of 25 and 40 degrees C. The results showed that our method could detect the predicted changes in conduction velocity distribution and temporal dispersion. We conclude that temperature change is a model that can be used to study temporal dispersion. This may be a first step towards distinguishing between the effects of conduction block and (abnormal) temporal dispersion in demyelinated nerve fibers.

Adult↗

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↗