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Biomedical subjects

W H Linssen

Publications and source records attributed to W H Linssen.

At least 19 recordsLinked to original sources
PubMed2001

Effects of medication use on health state in postictal migraineurs.

We investigated whether headache-free patients with migraine report a lower health state compared with healthy controls, and whether health state is differently affected during the postattack period after using sumatriptan versus habitual nonvasoactive medication. Mood, health state, and personality questionnaires were administered once during an interictal period and twice within 30 hours after different migraine attacks treated with sumatriptan or habitual nonvasoactive medication. Twenty migraineurs without aura, 10 migraineurs with aura, and 30 matched and headache-free controls participated in this study. During an interictal period, patients with migraine reported more problems regarding social activities and pain compared with healthy controls. During the postictal period, mood (fatigue and emotional state) was negatively affected by an attack that was treated with habitual medication, whereas health state (physical pain, social activities, current pain) was similar to the migraine-free period. Sumatriptan treatment had beneficial effects on aspects of health state and mood during the postictal period.

Adult↗
PubMed2001

Interictal and postictal contingent negative variation in migraine without aura.

Cortical hyperexcitability is thought to explain the more enhanced contingent negative variation (CNV) amplitudes and impaired CNV habituation that have been found during the interictal period in migraine without aura. These CNV characteristics have been shown to normalize to the level of healthy controls during an attack. This study aimed to replicate the interictal findings, and additionally examine whether migraineurs show reduced CNV amplitudes during the postattack period. Of 12 patients with migraine without aura and their sex- and age-matched healthy controls, CNV characteristics were recorded once in an interictal period, once during the postattack period within 30 hours after an attack that was treated with sumatriptan, and once after an attack that was treated with habitual nonvasoactive medication (counterbalanced). The present results did not confirm the enhanced CNV early and late wave amplitudes or impaired habituation, and cortical hyperexcitability that have previously been reported in the interictal period in patients with migraine without aura. During the postattack period, a decrease in CNV early and late amplitudes was found but only after sumatriptan use. This reduction in CNV amplitudes was most prominent over the frontal cortex and could reflect cortical hypoexcitability, possibly related to a suppression of central catecholaminergic activity by sumatriptan.

Adult↗
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

Neuromuscular findings in thyroid dysfunction: a prospective clinical and electrodiagnostic study.

OBJECTIVES: To evaluate neuromuscular signs and symptoms in patients with newly diagnosed hypothyroidism and hyperthyroidism. METHODS: A prospective cohort study was performed in adult patients with newly diagnosed thyroid dysfunction. Patients were evaluated clinically with hand held dynamometry and with electrodiagnosis. The clinical features of weakness and sensory signs and the biochemical data were evaluated during treatment. RESULTS: In hypothyroid patients 79% had neuromuscular complaints, 38% had clinical weakness (manual muscle strength testing) in one or more muscle groups, 42% had signs of sensorimotor axonal neuropathy, and 29% had carpal tunnel syndrome. Serum creatine kinase did not correlate with weakness. After 1 year of treatment 13% of the patients still had weakness. In hyperthyroid patients 67% had neuromuscular symptoms, 62% had clinical weakness in at least one muscle group that correlated with FT4 concentrations, but not with serum CK. Nineteen per cent of the patients had sensory-motor axonal neuropathy and 0% had carpal tunnel syndrome. The neuromuscular signs developed rapidly, early in the course of the disorder and were severe, but resolved rapidly and completely during treatment (average time 3.6 months). CONCLUSIONS: Neuromuscular symptoms and signs were present in most patients. About 40% of the hypothyroid patients and 20% of the hyperthyroid patients had predominantly sensory signs of a sensorimotor axonal neuropathy early in the course of thyroid disease. Weakness in hyperthyroidism evolved rapidly at an early stage of the disorder and resolved completely during treatment, suggesting a functional muscle disorder. Hand held dynamometry is sensitive for the detection of weakness and for the clinical evaluation of treatment effects. Weakness in hypothyroidism is more difficult to treat, suggesting myopathy.

Adult↗
PubMed1999

Interictal and postictal cognitive changes in migraine.

The question whether symptom-free migraine patients show cognitive impairments compared to matched control subjects is addressed, and also whether migraine patients show transient cognitive impairments induced by an attack. The Neuropsychological Evaluation System (NES2) was administered once in an interictal period and twice within 30 h after different migraine attacks. Since cognitive impairments could be related to attack duration or severity, cognitive performance was compared during a postictal period after sumatriptan use and during a postictal period after habitual nonvasoactive medication use. Twenty migraineurs without aura, 10 migraineurs with aura, and 30 matched headache-free controls participated in the study. During a headache-free period, migraineurs without aura responded as quickly as controls, while migraineurs with aura were slower than controls during all tasks specifically requiring selective attention. These effects were not aggravated by a preceding migraine attack, irrespective of medication use and attack duration.

Adult↗
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↗
PubMed1997

Severe irreversible optic neuritis following Mantoux tuberculin skin test in a child with multiple sclerosis--a case report.

A 12-year-old girl was diagnosed as suffering from multiple sclerosis. At age 14, just after recovery from an exacerbation of MS with left-sided optic neuritis, she underwent a Mantoux tuberculin skin test. Within 30 minutes she developed complete irreversible optic neuropathy of the left eye. This case illustrates the urge for caution to perform vaccinations and tuberculin skin tests not only during progressive disease activity of MS, but also in the reconvalescent phase after an acute exacerbation.

Child↗
PubMed1997

Miyoshi-type distal muscular dystrophy. Clinical spectrum in 24 Dutch patients.

Miyoshi-type distal muscular dystrophy has now been found to be more frequent outside Japan than was previously thought. We studied 24 Dutch patients with Miyoshi-type distal muscular dystrophy and focused on its clinical expression and natural history, muscle CT-scans and muscle biopsy findings. Our study shows that Miyoshi myopathy is a heterogeneous, slowly progressive disorder. The disease starts with weakness and atrophy of the calves and progressively involves the proximal leg and hip muscles and, in a later stage the shoulder and upper arm muscles. After 10 years disease duration, one-third of the patients are dependent on wheelchairs for out-of-door transportation. Disease progression is related to disease duration and not to early age of onset of symptoms. Onset may be at any age and is asymmetrical in roughly half of the cases. Four cases had been initially diagnosed as idiopathic hyper-CK-aemia.

Adult↗
PubMed1997

Differences between hereditary motor and sensory neuropathy type 2 and chronic idiopathic axonal neuropathy. A clinical and electrophysiological study.

To evaluate whether chronic idiopathic axonal polyneuropathy (CIAP) should be considered as hereditary motor and sensory neuropathy type 2 (HMSN type 2), we compared the clinical features of 48 patients with CIAP with those of 47 patients with HMSN type 2. In addition, we studied electrophysiological data in 20 patients with CIAP and in 20 patients with HMSN type 2. We found, in patients with HMSN type 2, that the initial symptoms were predominantly motor and that weakness and handicap were more severe and skeletal deformities more frequent, compared with those of CIAP patients. Electrophysiologically, the tibialis anterior muscle showed more denervation in patients with HMSN type 2, consistent with the predominance of motor symptoms. There was no important effect of age of onset on clinical features in HMSN type 2 patients. We conclude that in an individual patient with a sensory or sensorimotor idiopathic axonal polyneuropathy and no family history of polyneuropathies, the diagnosis HMSN type 2 is unlikely. However, if motor symptoms predominate, the diagnosis of HMSN type 2 should be considered.

Adult↗
PubMed1997

Sjögren's syndrome in patients with chronic idiopathic axonal polyneuropathy.

OBJECTIVE: To assess the presence of symptoms and signs of Sjögren's syndrome in patients with otherwise idiopathic axonal polyneuropathy and to develop guidelines for the diagnostic approach with respect to Sjögren's syndrome in these patients. METHODS: Sixty five patients with axonal polyneuropathy in whom an aetiological diagnosis could not be made underwent (1) a standard interview focusing on ocular and oral sicca symptoms, (2) physical examination, (3) tests for objective assessment of keratoconjunctivitis sicca, (4) extensive serological investigations, and (5) a sublabial salivary gland biopsy. RESULTS: In forty nine patients a sublabial salivary gland (SSG) biopsy was performed, thereby completing the whole investigation for Sjögren's syndrome. Three of these 49 patients (all women) had an SSG biopsy specimen suggestive of Sjögren's syndrome, which, in combination with other symptoms and signs, led to a diagnosis of primary Sjögren's syndrome. CONCLUSIONS: None of the three patients with primary Sjögren's syndrome had spontaneously complained about sicca symptoms and the clinical neurological picture of them did not differ from the other patients in the study. Therefore, in patients with chronic idiopathic axonal polyneuropathy, especially in women, a systematic investigation for Sjögren's syndrome should be done, because the presence of Sjögren's syndrome may have implications for treatment and justifies a clinical follow up on a regular base.

Aged↗
PubMed1997

A behavioral treatment of young migrainous and nonmigrainous headache patients: prediction of treatment success.

We evaluated the outcome of a behavioral treatment package in a clinical setting with a group of young (age: 12-22) headache patients, suffering from migrainous or nonmigrainous. Comparison between the experimental (n = 24) and the waiting-list control group (n = 15) showed a treatment effect on headache frequency and on the headache index. Using a 50% reduction in the headache activity as a criterion for clinical improvement, 52% of the participants in the experimental group had improved clinically at the end of the treatment. The treated participants were found to have maintained significant improvement at 1-year follow-up. The treatment effect was significantly high for nonmigrainous headache patients than for migraineurs. The most important background predictor of outcome was duration of headache history: youngsters with a longer headache history profited less by the treatment than youngsters with a shorter headache history. Family predictors of pre-post improvement were maternal rewarding of illness behavior and mother-child relationship Those youngsters who reported more rewarding and/or a more positive mother-child relationship profited less by the treatment than those who reported less rewarding and/or a less positive mother-child relationship. We conclude that therapists treating young headache patients should be alert to pain-rewarding patterns in the family.

Journal Article↗
PubMed1994

Deafness, sensory neuropathy, and ovarian dysgenesis: a new syndrome or a broader spectrum of Perrault syndrome?

We report on 3 sibs (2 males and one female) with sensorineural deafness. The presence of ovarian dysgenesis in the girl suggested a diagnosis of Perrault syndrome. In addition our patients have a sensory polyneuropathy and amelogenesis imperfecta. Two of the patients have mild mental retardation, fine choreatic movements, and dyspraxia. It is discussed whether these findings are part of a separate clinical entity or should be included within the spectrum of the Perrault syndrome.

Abnormalities, Multiple↗
PubMed1994

[2 patients with autosomal recessive generalized myotonia].

Two men aged 20 and 18 years complained of transient muscle stiffness of 4 and 2 years' duration, respectively. Symptoms had started in the legs. Gradually, arm muscles and eventually facial and pharyngeal muscles became affected. Neurological examination revealed myotonia without any associated symptoms. Myotonia was confirmed by electromyography. Laboratory investigations, ECG, chest X-rays, slit lamp examination in both patients, and muscle biopsy in one, revealed no abnormalities. History, physical examination and electromyography of family members disclosed no evidence of myotonic dystrophy. Based on the findings mentioned, the diagnosis of autosomal recessive generalised myotonia was made.

Adolescent↗