Biomedical subjects

Hessel Franssen

Publications and source records attributed to Hessel Franssen.

11 recordsLinked to original sources

PubMed2006

Length dependence in polyneuropathy associated with IgM gammopathy.

OBJECTIVE: In polyneuropathy associated with monoclonal IgM gammopathy, nerve conduction studies may show disproportionate distal slowing consistent with segmental demyelination. This was suggested to represent a length-dependent demyelinating process, starting in distal and proceeding to proximal segments. Because the evidence for this is incomplete, we assessed whether length dependence occurs in IgM neuropathy. METHODS: In 22 patients with IgM neuropathy, 20 disease controls with chronic inflammatory demyelinating polyneuropathy (CIDP) and 36 normal controls, we investigated motor conduction, sensory conduction, and needle electromyography for nerves with short, intermediate-length, and long axons as well as conduction in short segments of the ulnar nerve from proximal to distal. To compare variables in nerves of different length, we normalized individual values with respect to the median in normal controls. RESULTS: In IgM neuropathy, distal slowing and features of axon loss increased with nerve length, and ulnar nerve conduction became gradually slower from proximal to distal when the elbow segment was excluded. In CIDP, no clear length dependence was found except for distal amplitude. INTERPRETATION: The disproportionate distal slowing in IgM neuropathy may be part of a length-dependent process, assuming that this process is randomly distributed due to a generalized exposure to IgM.

Aged↗

PubMed2006

Nerve conduction studies in polyneuropathy: practical physiology and patterns of abnormality.

Nerve conduction studies are an essential part of the work-up of peripheral neuropathies. Many neuropathic syndromes can be suspected on clinical grounds, but optimal use of nerve conduction study techniques (in combination with needle electromyography) allows diagnostic classification and is therefore crucial to understanding and separation of neuropathies. Multifocal motor neuropathy, for example, may clinically present as ALS. Detection of evidence of demyelination (conduction blocks) leads to the correct diagnosis and to proper treatment. Nerve conduction studies provide essential information on (1) the spatial pattern of neuropathy, (2) the pattern of abnormalities distinguishing between primarily axonal and demyelinating pathology, and (3) the severity of neuropathic damage. This information is very comprehensive since many nerves and long segments of individual nerves can be sampled. Moreover the information is extremely detailed to the extent that the cellular pathology of a patient's neuropathy is usually defined best by physiological testing rather than by biopsy. Neuropathies can be generalized, focal, or multifocal; they can be symmetric or asymmetric; they can be distally predominant or proximal and distal. Primarily axonal neuropathies mainly affect sensory nerve and compound muscle action potential amplitudes, whereas demyelinating neuropathies lead to slowing of nerve conductions, and to increased temporal dispersion or conduction block. Usually, the pattern of demyelination allows to distinguish hereditary (uniform demyelination) from acquired (segmental demyelination) neuropathies. Electrodiagnostic criteria for primary demyelination are helpful to identify acquired demyelinating neuropathies.

Adult↗

PubMed2005

Short-segment nerve conduction studies in ulnar neuropathy at the elbow.

The aim of the study was to assess the diagnostic value of short-segment nerve conduction studies (NCS) at 2-cm intervals from 4 cm above to 4 cm below the medial epicondyle in a large group of patients with ulnar neuropathy at the elbow (UNE). Furthermore, we wanted to compare electrodiagnostic and clinical findings. We evaluated 73 arms in 70 patients with UNE and observed the following abnormalities on short-segment NCS: focal conduction block (CB) in 1, focal CB with increased latency change in 34, and increased latency change alone in 25. Short-segment NCS had an additional localizing value in 28 arms of the 37 patients (76%) with motor conduction velocity (MCV) slowing across the elbow only or with nonlocalizing electrodiagnostic findings. The lesion was located above the elbow in 32 arms (53%), at the epicondyle in 16 arms (27%), and below the epicondyle in 12 (20%) of the 60 arms with focal CB or increased latency change on short-segment NCS. Patients with CB on routine and short-segment NCS had muscle weakness significantly more often than patients without CB. Thus, short-segment NCS are useful in localizing the lesion in patients with UNE and CB on routine NCS and have additional diagnostic value in patients with MCV slowing across the elbow or with nonlocalizing signs on routine nerve conduction studies. We recommend its use in all patients in whom UNE is suspected.

Adolescent↗

PubMed2005

Multifocal motor neuropathy.

Multifocal motor neuropathy (MMN) is an immune-mediated disorder characterised by slowly progressive, asymmetrical weakness of limbs without sensory loss. The clinical presentation of MMN mimics that of lower-motor-neuron disease, but in nerve-conduction studies of patients with MMN motor-conduction block has been found. By contrast with chronic inflammatory demyelinating polyneuropathy, treatment with prednisolone and plasma exchange is generally ineffective in MMN and even associated with clinical worsening in some patients. Of the immunosuppressants, cyclophosphamide has been reported as effective but only anecdotally. Various open trials and four placebo-controlled trials have shown that treatment with high-dose intravenous immunoglobulin leads to improvement of muscle strength in patients with MMN. Although clinical, pathological, imaging, immunological, and electrophysiological studies have improved our understanding of MMN over the past 15 years, further research is needed to elucidate pathogenetic disease mechanisms in the disorder.

Clinical Trials as Topic↗

PubMed2003

Disease course of Charcot-Marie-Tooth disease type 2: a 5-year follow-up study.

BACKGROUND: Charcot-Marie-Tooth disease (CMT) type 2 is the axonal variant of an inherited, sensorimotor polyneuropathy. To our knowledge, the clinical course of CMT type 2 has never been prospectively studied in a large group of patients. OBJECTIVE: To prospectively evaluate the disease course of patients with CMT type 2. METHODS: We prospectively evaluated the disease course in patients with CMT type 2. Forty-three patients (24 men) of 27 families with CMT type 2 were included. All patients were analyzed by the same investigator at entry and after 5 years. The standardized protocol included manual muscle testing, which could lead to a motor sum score of 140 points, and quantification of sensory deficit. Disability was assessed using the modified Rankin scale, and quality of life was assessed using the RAND 36-item health survey questionnaire. Eighteen families were tested for known mutations in the MPZ, PMP22, and GJB1 genes. RESULTS: At entry, the mean +/- SD age of the patients was 52 +/- 14 years, and the mean +/- SD duration of disease was 12 +/- 8 years. The median motor sum score deteriorated from 135 to 128 points (P =.02). Progression was never rapid. There was no sensory deterioration. The Rankin score decreased by 1 point in 16 patients. At follow-up, more patients needed walking aids, but most patients remained ambulant. The number of patients with claw toes increased, whereas the number of patients with foot deformities such as pes cavus and short calf muscles remained stable. There was no correlation between deterioration and age. Analysis of quality of life did not show any changes. In one family, a mutation in the GJB1 gene was found. CONCLUSION: This prospective study shows a slow deterioration of muscle strength and increase in disability in CMT type 2 during a 5-year follow-up period.

Adult↗

PubMed2003

A randomized sequential trial of creatine in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a fatal disease with no cure. In a transgenic mouse model of ALS, creatine monohydrate showed a promising increase in survival. We performed a double-blind, placebo-controlled, sequential clinical trial to assess the effect of creatine monohydrate on survival and disease progression in patients with ALS. Between June 2000 and December 2001, 175 patients with probable, probable-laboratory supported, or definite ALS were randomly assigned to receive either creatine monohydrate or placebo 10 gm daily. A sequential trial design was used with death, persistent assisted ventilation, or tracheostomy as primary end points. Secondary outcome measurements were rate of decline of isometric arm muscle strength, forced vital capacity, functional status, and quality of life. The trial was stopped when the null hypothesis of indifference was accepted. Creatine did not affect survival (cumulative survival probability of 0.70 in the creatine group vs 0.68 in the placebo group at 12 months, and 0.52 in the creatine group vs 0.47 in the placebo group at 16 months), or the rate of decline of functional measurements. Creatine intake did not cause important adverse reactions. This placebo-controlled trial did not find evidence of a beneficial effect of creatine monohydrate on survival or disease progression in patients with ALS.

Aged↗

PubMed2003

Influence of age of horse on results of quantitative electromyographic needle examination of skeletal muscles in Dutch Warmblood horses.

OBJECTIVE: To determine the influence of age on results of quantitative analysis of electromyographic (EMG) needle examination in the subclavian, triceps, and lateral vastus muscles of Dutch Warmblood horses. ANIMALS: 7 healthy young Dutch Warmblood horses (range, 13 to 18 months old), 7 healthy adult Dutch Warmblood horses (range, 4 to 10 years old), and 7 healthy elderly Dutch Warmblood horses (range, 18 to 21 years old). PROCEDURE: An EMG needle examination was performed to evaluate insertional activity, spontaneous activity, and motor unit action potential (MUAP) variables. Although all horses were conscious, young horses were sedated prior to examination. RESULTS: Mean insertional activity in young horses was significantly lower than in elderly horses. Pathologic spontaneous activity was rarely found in young and adult horses but was frequently evident in all muscles in all elderly horses. The MUAP duration and amplitude were significantly lower in all muscles of young horses, compared with values for adult and elderly horses. The MUAP duration and number of phases and turns were significantly lower in adult horses than in elderly horses. Group differences for percentages of polyphasic and complex MUAPs were also found. The 95% confidence intervals for MUAP duration, MUAP amplitude, and number of phases and turns for the subclavian, triceps, and lateral vastus muscles were significantly lower in young horses than in adult or elderly horses. CONCLUSIONS AND CLINICAL RELEVANCE: Age of the horse being examined should be considered when EMG examination is performed.

Action Potentials↗

PubMed2002

Chronic idiopathic axonal polyneuropathy and successful aging of the peripheral nervous system in elderly people.

BACKGROUND: Chronic idiopathic axonal polyneuropathy (CIAP) is a frequent neurologic disorder in elderly persons. In view of the aging population, it is important to know the long-term prognosis of CIAP. OBJECTIVES: To determine if CIAP is influenced by the superposition of the effects of aging and to evaluate the severity of CIAP according to the disease duration. DESIGN: Controlled cohort study. SETTING: Outpatient clinic for neuromuscular diseases at the University Medical Center Utrecht, Utrecht, the Netherlands. PARTICIPANTS AND METHODS: One hundred twenty-seven patients with CIAP and 108 age-matched control subjects were included. We defined CIAP on the basis of symmetrical distal sensory or sensorimotor symptoms and signs with evolution over at least 6 months, exclusion of causes by history taking, results of clinical and laboratory investigations, and electrophysiologic findings that agreed with the diagnosis of axonal polyneuropathy. RESULTS: No important neurologic or electrophysiologic differences were found between patients with early-onset (before the age of 65 years) and late-onset (at or after the age of 65 years) CIAP, but patients with early-onset CIAP who had a short disease duration (<10 years) experienced more disability than patients with late-onset CIAP who had a similar disease duration. Old controls (age of 65 years or older) more often had symptoms, sensory signs in the legs, absent ankle jerks, and lower mean distal amplitudes of compound muscle action potentials and sensory nerve action potentials than young controls (aged <65 years). Absence of the sural nerve sensory nerve action potentials or presence of spontaneous muscle fiber activity in the anterior tibial muscle was common in patients with CIAP (51% and 60%, respectively), but exceptional (both 2%) in controls. CONCLUSIONS: Neither aging of the peripheral nervous system nor disease duration affects CIAP to a considerable degree, but CIAP has a greater influence on the daily life of nonretired patients with early-onset CIAP. The diagnosis of axonal polyneuropathy is probably supported best by either the absence of the sural nerve sensory nerve action potentials or the presence of spontaneous muscle fiber activity in the anterior tibial muscle.

Age of Onset↗

PubMed2002

Interhemispheric reorganization of motor hand function to the primary motor cortex predicted with functional magnetic resonance imaging and transcranial magnetic stimulation.

The objective of this study was presurgical assessment of reorganization of motor hand function in an 11-year-old girl with intractable epilepsy and a right-sided hemiplegia resulting from an extensive perinatal left hemispheric stroke. Prior to a left functional hemispherectomy, functional magnetic resonance imaging (MRI) showed that both nonparetic and paretic motor hand function predominantly activated the right primary motor cortex, whereas no activation was found in the left hemisphere. Transcranial magnetic stimulation of the right central area yielded responses in both the nonparetic and the paretic hand, whereas no responses were obtained after stimulation of the affected hemisphere. Both techniques indicated that motor function was mediated by corticospinal fibers originating from the undamaged (primary) motor cortex and predicted no further loss of motor hand function after surgery. Indeed, subsequent functional hemispherectomy induced no new sensorimotor deficits. Functional MRI was repeated 22 months after surgery and matched preoperative sensorimotor functional MRI findings, confirming reorganization of the primary motor cortex. No additional reorganization was introduced by surgery.

Brain Infarction↗

PubMed2002

Quantitative analysis of motor unit action potentials in the subclavian muscle of healthy horses.

OBJECTIVE: To evaluate the application of analysis of motor unit action potentials (MUAP) in horses and to obtain values of MUAP for the subclavian muscle of horses. ANIMALS: 10 healthy adult Dutch Warmblood horses. PROCEDURE: Electromyographic examination of the subclavian muscle in conscious nonsedated horses was performed to evaluate insertional activity, spontaneous activity, MUAP variables, and recruitment patterns. Muscle and body temperatures were measured at the beginning and end of the procedure. Amplitude, duration, number of phases, and number of changes in direction (ie, turns) for all representative MUAP were analyzed to determine values for this muscle in this group of horses. RESULTS: Mean +/- SD duration of insertional activity was 471.7 +/- 33.45 milliseconds. Mean MUAP amplitude in the examined horses was 379 RV (95% confidence interval [CI], 349 to 410 microV). Mean MUAP duration of the subclavian muscle was 727 milliseconds (95% CI, 6.84 to 7.71 milliseconds). Mean number of phases was 2.9, and mean number of turns was 3.0. Prevalence of polyphasic MUAP defined as MUAP with > 4 phases, was 77%. Number of MUAP that had > 5 turns was 2.4%. Satellite potentials were found in 1.0% of the MUAP CONCLUSIONS AND CLINICAL RELEVANCE: This study revealed that electromyography including MUAP analysis can be performed in horses, and values for the subclavian muscle in healthy adult horses can be obtained. Analysis of MUAP could be a valuable diagnostic tool for use in discriminating between myogenic and neurogenic problems in horses.

Action Potentials↗

PubMed2002

Electromyographic changes of motor unit activity in horses with induced hypocalcemia and hypomagnesemia.

OBJECTIVE: To determine whether electromyographic abnormalities are evident in skeletal muscles in horses with induced hypocalcemia and hypomagnesemia. ANIMALS: 7 healthy adult Dutch Warmblood horses. PROCEDURES: Electromyographic examination was performed in the lateral vastus, triceps, and subclavian muscles before and after IV infusion of EDTA. An initial dose (mean +/- SD, 564+/-48 ml) of a 10% solution of sodium EDTA was administered IV during a period of 21+/-73 minutes to establish a blood concentration of ionized calcium of approximately 0.5 mmol/L. Average rate of EDTA infusion to maintain ionized calcium at this concentration was 6.6 ml/min. RESULTS: Mean blood concentrations of ionized calcium and magnesium were 1.39+/-0.06 and 0.84+/-0.09 mM, respectively before EDTA infusion; after EDTA infusion, concentrations were 0.48+/-0.05 and 0.44+/-0.20 mM, respectively. This state induced positive waves; fibrillation potentials; doublets, triplets, and multiplets; complex repetitive discharges; and neuromyotonia. Analysis of motor unit action potentials (MUAP) after EDTA infusion revealed an increase in prevalence of polyphasic and complex MUAP in all muscles. CONCLUSIONS AND CLINICAL RELEVANCE: None of the horses had classical signs of hypocalcemia and hypomagnesemia. In contrast, all horses had spontaneous activity in the measured muscles indicative of nerve hyperirritability. Calcium and magnesium deficits appear to have consequences, which may be subclinical, affecting functions of the neuromuscular system. This is of interest for equestrian sports in which hypocalcemia and hypomagnesemia are expected, such as during endurance rides.

Action Potentials↗