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N C Notermans

Publications and source records attributed to N C Notermans.

At least 37 records · Page 2Linked to original sources
PubMed2001

Surgery in adults with tethered cord syndrome: outcome study with independent clinical review.

OBJECT: The authors conducted a study to evaluate the risks and short-term benefits of surgical treatment for tethered cord syndrome (TCS) in patients older than 18 years of age. METHODS: The authors studied a series of 57 consecutive adult patients with TCS of varying origins. Patients were examined by the same neurologist in a standardized fashion before and after surgery, and most were followed for at least 2 years postoperatively. Patient age ranged from 19 to 75 years. The mean age at onset of symptoms and diagnosis was 30 years and 37 years, respectively. Muscle strength improved (15 cases) or showed no change postoperatively (38 cases) in a large majority of patients (93%). In four patients a minor decrease in muscle strength was demonstrated, and there was significant deterioration in two (3.5%). In the two latter patients, a rapid decline in motor function was present preoperatively. Subjective assessment of pain, gait, sensory function, and bladder/bowel function at 4 weeks, 6 months, and 2 years postsurgery revealed improvement in a substantial percentage of patients. No major surgery-related complications occurred. CONCLUSIONS: This is the largest series to date in which adult patients with TCS comprise the report. Untethering procedures in these patients were safe and effective, at least in the short term. Patients with rapid loss of motor function, lipomyelomeningocele, or split cord malformation seem to be at a higher risk of postsurgery deterioration. A follow-up period of many more years will be necessary to determine whether aggressive surgery is beneficial in the long term.

Adult↗
PubMed2000

Three families with polyneuropathy associated with monoclonal gammopathy.

OBJECTIVE: To report familial occurrence of polyneuropathy associated with monoclonal gammopathy. DESIGN: Case reports. PATIENTS: We describe 6 patients (3 pairs) with a polyneuropathy associated with IgM monoclonal gammopathy. Four of the 6 patients had a demyelinating polyneuropathy on neurophysiological examination. Three patients had elevated antibodies against myelin-associated glycoprotein. No duplication on chromosome 17 or a mutation on chromosome 1 was found in any family. CONCLUSION: Familial occurrence of polyneuropathy without the presence of hereditary motor and sensory neuropathy type I is a reason to search for the presence of monoclonal gammopathy.

Aged↗
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

Benign prognosis in idiopathic hyper-CK-emia.

We report on the long-term follow-up in 31 patients with idiopathic hyper-CK-emia. At referral, all patients underwent a neurological interview and examination. Ancillary investigations included an open muscle biopsy and electromyography (EMG) in almost all, and other ancillary tests in some patients. After a follow-up period of 7.2 (mean; range 4-18) years, 74% of the patients had a final evaluation. The most common complaints at referral were fatigue and myalgia. EMG and muscle biopsy demonstrated minor, non-diagnostic abnormalities in 30 and 71% of patients, respectively. At follow-up, the pattern and the number of complaints had not changed substantially. One patient developed a sensory polyneuropathy. Neurological abnormalities were absent in all other patients. In conclusion, long-term follow-up of patients with idiopathic hyper-CK-emia does not reveal clinical deterioration. It seems justifiable to refrain from routine long-term follow-up in these patients.

Adolescent↗
PubMed2000

Thickness of endoneurial vessel basal lamina area in chronic idiopathic axonal polyneuropathy.

For chronic idiopathic axonal polyneuropathy (CIAP), even after extensive evaluation, no cause has yet been found. Considering the age and sex distribution of patients with this disease, it is possible that vascular disease plays a role in the development of this polyneuropathy. As endoneurial vessel abnormalities can be related to ischemia, we investigated endoneurial vessels in sural nerve biopsies of 18 patients with CIAP. As controls we used sural nerves of 4 patients with diabetes mellitus, 6 patients with hereditary motor and sensory neuropathy type II (HMSN type III) and 10 autopsy cases. Basal lamina area thickness, endothelial cell area, lumen area, and the number of basal laminae, endothelial cells and periendothelial cell nuclei were investigated. Basal lamina area thickness, endoneurial cell area and number of endothelial cell nuclei in CIAP were increased in comparison with HMSN type III, whereas the basal lamina area thickness of patients with CIAP and diabetes mellitus were in the same range. The structure of the basal lamina area in CIAP differed from diabetes mellitus; in diabetes mellitus there was a larger number of lamellae, whereas in CIAP there was an increase in collagen. There was no correlation between basal lamina area thickness and age. In CIAP patients with peripheral vascular disease of the legs, basal lamina area thickness was increased. The relation between basal lamina area thickening and peripheral vascular disease of the legs in CIAP may indicate a role for ischemia in the development of this polyneuropathy.

Adult↗
PubMed2000

Quality of life in patients with axonal polyneuropathy.

"Quality of life" (QOL) measurement reflects the impact of a disease on the daily life of a patient, and this can be used as an outcome measure in clinical trials. QOL measurements are rarely used in patients with neuromuscular disease. The aim of this study was to determine whether QOL is reduced in chronic polyneuropathy, whether there is a relationship between QOL and objective measures of disease severity, and whether measuring QOL is a useful addition to the assessment of severity of polyneuropathy. We measured QOL in 90 patients with chronic axonal polyneuropathy (33 with hereditary motor and sensory neuropathy type II and 57 with chronic idiopathic axonal polyneuropathy) using the RAND 36-item Health Survey questionnaire (RAND-36). We compared the results with the QOL of a reference population, with summed motor and sensory scores, and with the Rankin scale for handicap. Patients had worse scores than the reference population on seven of eight areas of the RAND-36. Patients with both low motor and low sensory scores rated lower in physical and emotional areas than less impaired patients. A low Rankin score was related only to physical domains. We conclude that in patients with chronic axonal polyneuropathy the severity of disease can be assessed with a general QOL instrument, and that this provides additional information, particularly on areas related to emotional and social functioning.

Aged↗
PubMed2000

Relationship between ischemia and neuropathy.

To evaluate what at present is known about the relationship between vascular diseases and the development of chronic polyneuropathy, the literature on ischemia and peripheral nerve damage was reviewed with emphasis on chronic ischemia.

Animals↗
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

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

[Methylprednisolone in traumatic spinal cord injuries: not proven to be beneficial to the patient at the present time].

Ever since the publication in 1990 in The New England Journal of Medicine of a multicentre, randomised, double-blind, placebo-controlled trial on the efficacy of methylprednisolone (MP) in the treatment of acute, traumatic spinal cord injury, the advice is to administer MP as soon as possible to every patient presenting a traumatic spinal cord injury. This recommendation has been followed throughout the world, especially by traumatologists, and seems to be above criticism. However, the results of most cited studies, which have had a major influence on the treatment of patients with an acute, traumatic spinal cord lesion, show that the improvements in the neurological condition due to MP cannot be translated into a specific improvement in the functional status. Until it has been proven beyond reasonable doubt that MP can play a significant role in the treatment of these patients, we advise that MP should not be administered to a patient with acute, traumatic spinal cord injury, awaiting the results of more quantitative research. Such research is being performed by the Cochrane Brain and Spinal Cord Injury Group.

Emergency Treatment↗
PubMed1998

[Clinical judgment and decision making in medical practice. A retiree with fatigue and foot drop].

A 64-year-old former civil servant consulted his general practitioner because of severe fatigue. Later he began to lose weight and gradually developed chronic sensorimotor polyneuropathy characterized by sensory nerve loss which started in his legs. After a year he needed a wheel chair and developed cachexia. IgG paraprotein was detected. Morbid-anatomical examination of enlarged supraclavicular lymph nodes revealed plasma cell angiofollicular hyperplasia, characteristic of Castleman's disease. Treatment with corticosteroids led to marked improvement of the patient's condition. He was able to walk again, using an ankle orthosis on both legs.

Anti-Inflammatory Agents↗
PubMed1998

Genetic heterogeneity in Miyoshi-type distal muscular dystrophy.

Miyoshi-type distal muscular dystrophy (MMD) is an autosomal recessively inherited progressive disorder. The putative locus of MMD is linked to the limb-girdle muscular dystrophy 2B locus on chromosome 2p12-14. In this study three of four MMD pedigrees show non-linkage to the region spanned by D2S134-D2S358-D2S145 on chromosome 2p, indicating genetic heterogeneity. A genome wide screen was performed to identify loci linked to MMD. In two non-chromosome 2-linked families, a 23 cM region on chromosome 10 segregated with MMD.

Adult↗
PubMed1998

Distribution of muscle weakness of central and peripheral origin.

According to the established clinical tradition about the distribution of weakness, the ratios of flexor/extensor strength of patients with upper motor neuron lesions are expected to be relatively high for the elbow and wrist and low for the knee. To assess the diagnostic value of these patterns of weakness, muscle strength of 70 patients with limb weakness of central or peripheral origin was measured with a hand held dynamometer. The ratios of flexor/extensor strength at the knee, elbow, and wrist did not differ significantly between patients with central or peripheral origin of muscle weakness. The examination of tendon jerks proved to be of more value as a localising feature. The traditional notion about the distribution of weakness in upper motor neuron lesions may be explained by an intrinsically greater strength in antigravity muscles, together with the effects of hypertonia.

Adult↗
PubMed1997

[Nerve circuit disorders: determination of polyneuropathy].

So many conditions, both hereditary and acquired, can lead to polyneuropathy that a systematic approach is needed to identify the cause. Finding a cause is necessary to inform the patient about prognosis, handicap and chances of treatment. Three patients with polyneuropathy are described. The first was a man aged 34 with a predominantly motor neuropathy who appeared to have a duplication in the pmp-22 gene on chromosome 17PII.2. The diagnosis was hereditary sensorimotor neuropathy type IA (hypertrophic form of Charcot-Marie-Tooth disease). The second was a woman of 58 years; she had a sensory polyneuropathy as the first manifestation of Hodgkin's disease. The last patient, a 67-year-old man, had polyneuropathy due to vasculitis. He showed a prompt response to treatment.

Adult↗
PubMed1997

[Polyneuropathy: diagnosis and management].

A polyneuropathy is characterized by a symmetrical distribution of sensory or motor abnormalities, more pronounced distally than proximally and usually more evident in the lower than in the upper limbs. Polyneuropathies may be classified on the basis of (a) clinical picture: acute/subacute/chronic, sensory/motor/autonomous, axonal degeneration/segmental demyelinization, and (b) cause: metabolic disorder, deficiency, infection, auto(dys)immunity, hereditary and toxic/iatrogenic polyneuropathy, with idiopathic polyneuropathy as the remaining group. Damaged nerves may recover as the result of spontaneous remyelinization and axonal regeneration. Treatment is particularly successful in immunomediated neuropathies. Withdrawal is often successful in intoxications and suppletion in deficiencies. Even if no treatment is possible, the diagnosis is important: the patient can be taught to accept his disease and the prognosis can be determined, in connection with possible handicaps.

Algorithms↗
PubMed1997

[Referral of patients with polyneuropathy by the family physician: influence of type of symptoms but not of age].

OBJECTIVE: To investigate the influence of the age of the patient and the nature of a polyneuropathy on the referral behaviour of general practitioners (GPs). DESIGN: Written questionnaire sent to GPs regarding paper case records of polyneuropathy. SETTING: University Hospital Utrecht, the Netherlands. METHODS: 1590 GPs were asked about their differential diagnosis regarding a paper case record of a patient with polyneuropathy. There were six case records, differing in age (53, 64 and 73 years) and nature of the disease (sensory or sensorimotor polyneuropathy). The GPs were divided into six groups with similar demographic characteristics and type of practice. To avoid focus on polyneuropathy, all GPs also received questions about three other neurological cases (amaurosis fugax, radicular syndrome and vasovagal collapse). RESULTS: The mean response of the questionnaire was 54% (n = 844). Most GPs diagnosed the polyneuropathy (analysis of variance; p < 0.0001). The age of the patient did not influence the diagnosis nor the referral behaviour. At least 73% of the patients with a sensory and 81% of the patients with a sensorimotor polyneuropathy were referred to neurologists for further investigations (chi(2)-test; p < 0.05). CONCLUSION: At least 73% of the GPs referred a patient with polyneuropathy to a neurologist; patients with muscle weakness were referred more often than patients with only sensory disturbances. Referral was not influenced by the age of the patient.

Age Factors↗