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N C Notermans

Publications and source records attributed to N C Notermans.

64 records · Page 4Linked to original sources

Polyneuropathy associated with monoclonal gammopathy of undetermined significance. A prospective study of the prognostic value of clinical and laboratory abnormalities.

The natural course of polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS) is not well known. We therefore studied 32 untreated patients for a period of 5 years. Fifteen patients had an IgM M-protein, 15 an IgG and two an IgA. There was a male predominance, a mean age of onset at the end of the sixth decade and sensory signs were more pronounced than motor deficits. On entry into the study and during the 5 years of follow-up, we quantified the neuropathy in a standard way: totals of motor and sensory scores; vibration perception threshold; tapping tests; quantified Romberg test; electrophysiological parameters. A significant difference in the natural history between the polyneuropathy associated with IgM-MGUS and IgG/IgA-MGUS was found for the motor and sensory sum scores, the vibration perception threshold and the tapping tests. The polyneuropathy in IgM-MGUS is more progressive, with significantly more weakness and sensory signs, indicating that the neuropathies associated with IgM-MGUS and IgG/A-MGUS may be two different entities. A rapid progression of the neuropathy was found in five patients. We found no predictive factors for this severe progression of the neuropathy of these five patients. Of these five, three (two IgM, one IgG) developed a non-Hodgkin lymphoma.

Electrophysiology↗

Measuring ataxia: quantification based on the standard neurological examination.

To quantify ataxia in a simple way four tests were developed and analysed, based on the neurological examination: a tapping test for the arms (test 1), another one for the legs (test 2), a quantified finger-to-nose test (test 3), and a modified Romberg test (test 4). All tests were performed by 115 volunteers, 13 patients with cerebellar ataxia and 25 patients with sensory ataxia due to neuropathy. The test-retest repeatability was excellent. Tests 1, 2 and 4 were age-dependent, with lower scores above age 65. On test 1, 2 and 4 both groups of patients performed worse than controls; the correlation with the ataxia scale of Nobile-Orazio and the modified disability Rankin scale was good (P < 0.05). Although test 3 could differentiate between sensory and cerebellar ataxia, it was not useful for quantifying the degree of ataxia. To determine the practical value of the four tests, 11 patients performed the tests for a second time after a follow up period of 16 months. The results indicate that tests 1, 2 and 4 are sensitive for the detection of ataxia and of changes in its severity.

Adult↗

Chronic idiopathic axonal polyneuropathy: a five year follow up.

Seventy five patients with chronic idiopathic axonal polyneuropathy (CIAP) were studied for five years. The standardised and quantified neurological examination shows that progression of CIAP is slow, and handicap, if present, is not severe. During the follow up period a definite cause of the neuropathy was found in only four patients (two hereditary motor and sensory neuropathy type 2, one sensory chronic inflammatory demyelinating polyneuropathy, one alcoholic neuropathy). At the end of the follow up CIAP was not related to malignancy or gammopathy. Routine repetition of laboratory tests was not informative and these tests should be performed on clinical grounds only.

Aged↗

Experimentally-induced autonomic neuropathy: beneficial effect of a topical ACTH4-9 analogue on oculomotor nerve regeneration.

Melanocortins, peptides related to corticotropin (ACTH) and melanocyte-stimulating hormone, are known to exert beneficial neurotrophic effects in peripheral sensorimotor neuropathies. This has been demonstrated after both systemic and local administration of the peptides. By photographing the rat's pupil under standardized conditions, the authors have previously shown that systemic administration of a synthetic ACTH4-9 analogue can also be beneficial in autonomic neuropathies. The present study demonstrates that topical application of a synthetic ACTH4-9 analogue incorporated in a two-component fibrin glue enhances the speed of recovery of the parasympathetic nerve fibers in the oculomotor nerve after a crush lesion. This may have implications for future use in neurosurgery.

Administration, Topical↗

Chronic idiopathic polyneuropathy presenting in middle or old age: a clinical and electrophysiological study of 75 patients.

The clinical and electrophysiological features were prospectively studied of 75 patients (46 men and 29 women) with chronic polyneuropathy presenting in middle or old age in whom a diagnosis could not be made even after extensive evaluation and a follow up of six months. The mean age at the onset of symptoms was 56.5 years. The clinical features of chronic idiopathic polyneuropathy are heterogeneous. On clinical grounds 44 patients had a sensorimotor, 29 patients a sensory, and two patients a motor polyneuropathy. The overall clinical course in chronic idiopathic polyneuropathy was slowly progressive. None of the patients became severely disabled. Electrophysiological and nerve biopsy studies were compatible with an axonal polyneuropathy. Antibodies against myelin associated glycoprotein, gangliosides, and sulphatides were assessed in 70 patients and found to be negative.

Age of Onset↗

Anti-sulphatide antibodies in peripheral neuropathy.

A study was carried out on 135 patients with chronic idiopathic neuropathy (63), neuropathy associated with monoclonal gammopathy (51, including eight with anti-MAG antibody activity) and the Guillain-Barré syndrome (GBS) (21). Serum IgM, IgG and IgA anti-sulphatide antibody titres were compared with titres in 304 patients with other neurological or immunological diseases and in 50 normal subjects. Titres were presented a) as the highest serum dilution at which reactivity could be detected, and b) in the linear region of the optical density curve. A substantial number of patients with neurological or immunological diseases had higher titres than normal subjects. Compared with normal and disease controls, five patients with neuropathy associated with IgMk monoclonal gammopathy had raised titres of IgM anti-sulphatide antibodies and one patient with GBS had raised IgM, IgG and IgA anti-sulphatide antibodies in the acute phase of the disease. Two patients had a predominantly axonal sensory neuropathy with presenting symptoms of painful paresthesiae and minimal neurological deficit. Three patients had a predominantly demyelinating sensorimotor neuropathy associated with anti-MAG antibody activity. The patient with GBS had extensive sensory loss and antibody titres returned to normal within three weeks. Raised titres of anti-sulphatide antibodies occurred in several types of neuropathy, but all had some degree of sensory impairment and associated immunological abnormality.

Adult↗

Cranial nerve palsy as a delayed complication of attempted infanticide by insertion of a stylet through the fontanel. Case report.

A child suffered a sixth and seventh cranial nerve palsy due to intracerebral insertion of a stylet. The stylet was introduced through the anterior fontanel, most probably in an attempt at infanticide. The migration of the stylet through the brain was monitored because the child was first examined 6 years earlier. At operation the cranial part of the stylet lay in the fourth ventricle, compressing the facial nerve as well as the nucleus of the abducens nerve. The lower part of the stylet had reached the C-5 level.

Abducens Nerve Injury↗

[A drowsy child: benzodiazepine poisoning?].

In The Netherlands accidental intoxications in children due to benzodiazepines are regularly encountered. In 1987 of 1630 requests for information at the National Poison Control Centre about probable benzodiazepine intoxications 144 (8.8%) concerned children 0-12 years of age. The symptoms of this type of intoxication are non-specific and if the physician does not think of benzodiazepine intoxication extensive diagnostic procedures may be performed. If children show symptoms e.g. unconsciousness, ataxia and hypotonia, the physician should always think of benzodiazepine intoxication and try to confirm or to exclude this possibility by toxicological analysis. We discuss ways and means of the diagnosis and how to avoid pitfalls on the way.

Anti-Anxiety Agents↗

Acute MCPP intoxication: report of two cases.

1. Two cases of serious intoxication with phenoxy herbicides (MCPP) are reported. 2. Both patients had central nervous system involvement, became unconscious and had an inadequate respiration. Muscle cramps and rhabdomyolysis with renal failure were noted in both. Shortly after admission both patients developed a serious decrease in arterial blood pressure (160/80 mmHg to 80/45 mmHg). In one patient this was demonstrated to be caused by a reduction in peripheral vascular resistance. 3. Plasma concentration of MCPP in patient 2 was 298 mg/l (3-4 h after ingestion). The plasma t1/2 was about 17 h. MCPP plasma elimination probably follows first-order kinetics.

2-Methyl-4-chlorophenoxyacetic Acid↗

Amenorrhea after immunosuppressive treatment of multiple sclerosis.

Interviews on changes in the menstrual cycle were taken from 38 women of fertile age, several years after immunosuppressive treatment (IS) with prednisone and cyclophosphamide (CP) for definite multiple sclerosis (MS). Serum FSH, LH and 17-beta-oestradiol levels were determined at the time of interview. MS in itself did not change the experience of menstrual cycles; 17 patients developed hypergonadotrophic amenorrhea during or after IS. Symptoms related to climacterium (c. q. flushing) were present in 15 of these patients. The onset of amenorrhea depended on the age at the time of IS and on the cumulative dose of CP. Older patients developed amenorrhea at a lower cumulative dose of CP than did younger patients. High estrogen oral contraceptives are advocated in oncology to prevent disturbance of ovarian function by anti-mitotic treatment. This policy is advisable in female MS patients treated with drugs like CP or azathioprine.

Adult↗