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H Franssen

Publications and source records attributed to H Franssen.

At least 19 recordsLinked to original sources
PubMed2003

Demyelination and axonal loss in multifocal motor neuropathy: distribution and relation to weakness.

Multifocal motor neuropathy (MMN) is characterized by a slowly progressive, asymmetric weakness of the limbs without sensory loss. The arms are usually affected to a greater extent than the legs, and distal muscles more than proximal muscles. The distribution of electrophysiological abnormalities and its correlation with weak muscle groups in MMN have not been investigated systematically. The aim of the present study was to assess whether electrophysiological abnormalities have a preferential or random distribution, whether electrophysiological abnormalities in a nerve correlate with weakness in the innervated muscles, and whether these results are relevant for the development of optimal electrodiagnostic protocols. We compared the pattern of weakness and electrophysiological abnormalities in 39 patients with a lower motoneuron syndrome and a positive response to intravenous immunoglobulins. All patients underwent an extensive standardized electrophysiological examination. Electrophysiological evidence of demyelination was found more often in the nerves of the arms and was distributed randomly over lower arm, upper arm and shoulder segments. Electrophysiological evidence of axonal loss presented more frequently in longer nerves, occurring most often in the leg nerves. For the arm nerves, it is possible that the length dependence of axonal loss is due to the random distribution of demyelinating lesions that lead to axonal degeneration. Weakness was associated with features of demyelination and axonal loss in the nerves of the arm, and with features of axonal loss in leg nerves. However, a substantial number (approximately one-third) of electrophysiological abnormalities were found in nerves innervating non-weakened muscles. These results imply that in MMN, conduction block is most likely to be found in long arm nerves innervating weakened muscles, but if conduction block cannot be detected in these nerves, the electrophysiological examination should be extended to other arm nerves including those innervating non-weakened muscles.

Action Potentials↗
PubMed2003

Sporadic lower motor neuron disease with adult onset: classification of subtypes.

The discovery of the genetic basis of hereditary lower motor neuron disease (LMND) and the recognition of multifocal motor neuropathy as a distinct clinical entity necessitate a new classification of LMND. To this end, we studied the clinical and electrophysiological features of 49 patients with sporadic adult-onset LMND in a cross-sectional study. Disease duration was more than 4 years to exclude the majority of patients with amyotrophic lateral sclerosis. Based on the pattern of weakness, we identified three groups: 13 patients with generalized weakness (group 1); eight patients with symmetrical, distal muscle weakness (group 2); and 28 patients with non-generalized asymmetrical weakness of the arms in most patients (group 3). Group 3 could be subdivided into patients with weakness in predominantly the distal (group 3a) or the proximal (group 3b) muscle groups, both with disease progression to adjacent spinal cord segments. Distinctive features of group 1 were an older age at onset, more severe weakness and muscle atrophy, lower reflexes, greater functional impairment, more widespread abnormalities on concentric needle EMG, respiratory insufficiency and serum M-protein. In groups 2 and 3, concentric needle EMG findings also suggested a more widespread disease process. Retrospectively, the prognosis of sporadic adult-onset LMND appears to be favourable, because clinical abnormalities were still confined to one limb in most patients after a median disease duration of 12 years. We propose to classify the patients in the different subgroups as slowly progressive spinal muscular atrophy (group 1), distal spinal muscular atrophy (group 2), segmental distal spinal muscular atrophy (group 3a) and segmental proximal spinal muscular atrophy (group 3b). The described clinical phenotypes may help to distinguish between different LMND forms.

Adult↗
PubMed2002

Mimic syndromes in sporadic cases of progressive spinal muscular atrophy.

Described are patients initially diagnosed with progressive spinal muscular atrophy (PSMA), in whom further evaluation established another diagnosis. The authors prospectively investigated incident and prevalent cases of PSMA. Seventeen of 89 patients, after initial registration, were later excluded because reassessment revealed a diagnosis other than PSMA. In 11 of the 17 patients with a revised diagnosis, a potential treatment was available: multifocal motor neuropathy (7), chronic inflammatory demyelinating polyneuropathy (2), inflammatory myopathy (1), and MG (1). Other misdiagnoses included myopathy, syringomyelia, ALS, idiopathic chronic axonal polyneuropathy, and idiopathic brachial plexus neuropathy. One patient with a possible herniated lumbar disk recovered spontaneously.

Adult↗
PubMed2002

Disease severity in multifocal motor neuropathy and its association with the response to immunoglobulin treatment.

Disease progression in multifocal motor neuropathy (MMN) was studied by comparing severity and duration of disease. We assessed disease severity by determining muscle weakness, disability, conduction block (CB), and distal and proximal compound muscle action potential (CMAP)-amplitude in 38 patients with MMN in whom disease duration ranged from 6 months to 34 years. As indicator for an ongoing immune-mediated process, the response to one course of IVIg treatment was measured in 34 patients and associated with disease severity. With increasing disease duration, weakness and disability became significantly more severe, and the distal and proximal CMAP-amplitude decreased significantly. The number of CBs was significantly higher in patients with a disease duration longer than 10 years than in those affected less than 10 years. Thirty of the 34 patients responded to IVIg treatment. Non-responsiveness to IVIg was not associated with any of the disease variables. Severe and widespread weakness was significantly associated with a response > or = 2 on the MRC-sum-score. Our results provide evidence for a slowly progressive disease course of MMN. The good response to IVIg treatment in patients with severe and prolonged disease indicates that progression may be the result of an ongoing immune-mediated process. These findings imply that early treatment may prevent future progression of weakness and disability in patients with MMN.

Action Potentials↗
PubMed2002

Multifocal motor neuropathy: long-term clinical and electrophysiological assessment of intravenous immunoglobulin maintenance treatment.

We performed a long-term follow-up study of 11 patients with multifocal motor neuropathy (MMN) who received maintenance treatment with intravenous immunoglobulins (IVIg). Patients were treated initially with one full course of IVIg (0.4 g/kg for 5 days) followed by one IVIg infusion (0.4 g/kg) every week. During follow-up, the frequency and dosage of IVIg infusions were determined for each patient and ranged from one infusion every 1 to 7 weeks and an average dose of 7 to 48 g per week. During the 4- to 8-year follow-up period, muscle strength was assessed by measuring the MRC (Medical Research Council) sumscore of 20 muscle groups and by performing hand-held dynamometry on a selection of weak muscle groups. Systematic electrophysiological studies were performed before treatment and each year during IVIg maintenance treatment. Disability was assessed with the upper limb and lower limb subscales of the Guy's Neurological Disability Scale before treatment, after the first full course of IVIg and at the last follow-up examination. Muscle strength improved significantly within 3 weeks of the start of IVIg treatment and was still significantly better at the last follow-up examination than before treatment, even though it decreased slightly and significantly during the follow-up period. Upper limb disability was significantly better after the first full course of IVIg than before treatment. Conduction block disappeared in six nerve segments but new conduction block appeared in eight nerve segments during the follow-up period. Changes consistent with improvement (remyelination or reinnervation) occurred in 13 nerves during follow-up and changes consistent with worsening (demyelination or axon loss) occurred in 14 nerves. Electrophysiological changes consistent with improvement were significantly associated with the presence of conduction block before IVIg treatment. In conclusion, IVIg maintenance treatment has a beneficial long-term effect on muscle strength and upper limb disability but may not prevent a slight decrease in muscle strength. The electrophysiological findings imply that IVIg treatment favourably influences the mechanisms of remyelination or reinnervation but that axon loss cannot be prevented.

Adult↗
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↗