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J H Voormolen

Publications and source records attributed to J H Voormolen.

At least 19 recordsLinked to original sources
PubMed2000

Intrathecal baclofen for the treatment of dystonia in patients with reflex sympathetic dystrophy.

BACKGROUND AND METHODS: Patients with reflex sympathetic dystrophy (also known as the complex regional pain syndrome) may have dystonia, which is often unresponsive to treatment. Some forms of dystonia respond to the intrathecal administration of baclofen, a specific gamma-aminobutyric acid-receptor (type B) agonist that inhibits sensory input to the neurons of the spinal cord. We evaluated this treatment in seven women who had reflex sympathetic dystrophy with multifocal or generalized tonic dystonia. First, we performed a double-blind, randomized, controlled crossover trial of bolus intrathecal injections of 25, 50, and 75 microg of baclofen and placebo. Changes in the severity of dystonia were assessed by the woman and by an investigator after each injection. In the second phase of the study, six of the women received a subcutaneous pump for continuous intrathecal administration of baclofen and were followed for 0.5 to 3 years. RESULTS: In six women, bolus injections of 50 and 75 microg of baclofen resulted in complete or partial resolution of focal dystonia of the hands but little improvement in dystonia of the legs. During continuous therapy, three women regained normal hand function, and two of these three women regained the ability to walk (one only indoors). In one woman who received continuous therapy, the pain and violent jerks disappeared and the dystonic posturing of the arm decreased. In two women the spasms or restlessness of the legs decreased, without any change in the dystonia. CONCLUSIONS: In some patients, the dystonia associated with reflex sympathetic dystrophy responds markedly to intrathecal baclofen.

Adult↗
PubMed1999

Clinimetric issues of screening for responsiveness to intrathecal baclofen in dystonia.

OBJECTIVES: In this study we address clinimetric issues that pertain to the screening of responsiveness to intrathecal baclofen (ITB) in dystonia. METHODS: Eight patients with severe dystonia, who did not respond to oral medication, were evaluated in a double-blind placebo controlled ascending dose screening procedure, which included a randomised sequence of injections of 25, 50 and 75 microg baclofen and placebo. Self-assessments of dystonia severity on a visual analogue scale (VAS) and the Dyskinesia Rating Scale (DRS) were carried out at baseline 1, 4 and 8 hours after a bolus injection. RESULTS: Compared to the VAS, the DRS lacked responsiveness in all patients. Baseline scores of the VAS scores varied considerably between and within patients and underscore the need to express response scores in relation to the baseline. After placebo administration some patients showed a persistent improvement of about 30% across the day, while at some assessments improvements of >50% were noted. Based on the aforementioned findings, a responsiveness coefficient was used which relates the baclofen effect size to the non-specific score changes that may occur as a placebo effect or as random fluctuations in dystonia. Four patients with a responsiveness coefficient >2 received pump implantation and did well on continuous infusion of ITB. Several side effects occurred during the screening procedure, but none interfered with the execution of the screening procedure. CONCLUSIONS: This study demonstrates important clinimetric issues that need to be taken into account when screening for responsiveness to ITB.

Adolescent↗
PubMed1999

Cervical spondylarthrotic myelopathy with early onset in Down's syndrome: five cases and a review of the literature.

Progressive walking difficulties and bladder dysfunction may be attributed to Alzheimer disease or atlanto-axial subluxation in people with Down's syndrome (DS). The present authors describe five patients with DS suffering from the above symptoms as a result of cervical spondylarthrotic myelopathy. Clinical and radiological data were collected from all patients with DS who underwent surgery for cervical spondylarthrotic myelopathy at the Leiden University Medical Centre during the period between 1991 and 1995. Five patients with DS (four males and one female) were identified. Their mean age at diagnosis was 42 years. The main clinical features were weakness of the arms and legs, ataxic gait, hyperreflexia and bilateral Babinski signs. Radiological examination showed spondylarthrosis, compression of the spinal cord and myelomalacia. The mean delay in diagnosis was 3 years. All five individuals showed clinical stabilization after laminectomy. Cervical spondylarthrotic myelopathy seems a rather frequent disorder in DS, occurring at a relatively young age. Early diagnosis may prevent irreversible neurological deficits.

Adult↗
PubMed1996

Immunocytochemical localization of neurofilaments in the fibre systems of the developing rat spinal cord white matter.

The appearance and localization of the protein subunits of neurofilaments in the ascending and descending fibre systems of the developing rat spinal cord white matter were studied. The monoclonal antibody NF-90 (specific for the phosphorylated NF-L, NF-M and NF-H subunits) was used as neurofilament marker in fresh cryostat and Bouin fixed paraffin sections. The results were compared with Nissl and Bodian stained sections. Within the white matter, phosphorylated neurofilament proteins were expressed with regional variations. At embryonal day 12 (E12), the first positive fibres were found in the lateral funiculus. During further development, the peripheral region of the lateral funiculus showed an intense neurofilament staining, due to the presence of a higher number of fibres. From postnatal day 12 (P12) on, an increased amount of neurofilaments was found in the region close to the periphery, probably due to the presence of large calibre fibres. The dorsolateral part of the lateral funiculus filled in with fibres after birth, which indicated the extended development of the rubrospinal tract. At E13, positive fibres were present in the ventral commissure and the ventral funiculus. At E14, an increased amount of neurofilaments was detected in the periphery of the ventral funiculus. At maturity, an intense staining in the subsurface region could be found, due to the presence of large calibre fibres of the fasciculus longitudinalis medialis. At E13, the first neurofilament positive fibres were present in the dorsal funiculus. At this day, a concentration of fibres was found in the dorsal part of the dorsal root bifurcation zone and three days later, more fibres were detected in the medial part of the dorsal funiculus. At E18, a higher number of fibres was present in the dorsal region of the fasciculus gracilis. The mature fasciculus cuneatus showed an intense neurofilament staining, which was mainly present in large calibre fibres. The ventral part of the dorsal funiculus filled in with neurofilament positive fibres after birth. This indicated the relative late arrival of the corticospinal tract.

Animals↗
PubMed1994

The choice of treatment of single brain metastasis should be based on extracranial tumor activity and age.

PURPOSE: To determine if in patients with single brain metastasis the addition of neurosurgery to radiotherapy leads to lengthening of survival or to better quality of life. METHODS AND MATERIALS: From 1985 to 1990, 66 patients with single brain metastasis from a solid tumor were entered in a randomized trial of neurosurgery plus radiotherapy vs. radiotherapy alone. Patients were stratified for lung cancer vs. other sites of cancer and for progressive vs. stable systemic cancer. Radiotherapy was given to the whole brain by a novel scheme of two fractions of 2 Gy per day for a total dose of 40 Gy in 2 weeks, to obtain a relatively high total dose and short overall time, with minimal risk of late damage to normal tissue in long-term survivors. RESULTS: In the whole group of 63 evaluable patients, both with lung cancer as with other tumors, the combined treatment led to a better duration of survival (median 10 vs. 6 months; p = 0.04). The largest difference between both treatment arms was observed in patients with inactive extracranial disease (median 12 vs. 7 months; p = 0.02). Patients with active extracranial disease had an equal median survival of only 5 months, irrespective of given treatment. Age proved to be a strong and independent prognostic factor: patients older than 60 years had a hazard ratio of dying of 2.74 (p = 0.003) compared with younger patients. Following treatment, most patients remained functionally independent until a few weeks before death. In the majority of patients the cause of death was systemic tumor progression. CONCLUSION: Patients with single brain metastasis and with controlled or absent extracranial tumor activity should be treated with surgery and radiotherapy, especially when they are younger than 60 years. For patients with progressive extracranial disease, radiotherapy alone seems to be sufficient. The accelerated radiotherapy scheme of 40 Gy in 2 weeks to the whole brain is tolerated well and should also be considered for patients in a good performance status with surgically unaccessible single metastasis or even with multiple brain metastases.

Adult↗
PubMed1994

Aneurysmal bleeding. A plea for early surgery in good-risk patients.

From 1985 onwards we have aimed at operating on good-risk patients, i.e., those graded I-III on the WFNS SAH Scale, within 3 days after the aneurysmal bleed. We report on a series of 100 consecutive operations for saccular aneurysm, covering a period of 5 1/2 years. Early operations (in the above sense) were done in 57 good-risk but otherwise unselected patients. After a one year follow-up, 47 of them (82%) were found to have made a good recovery (Glasgow Outcome Score I). The outcome of (mostly early) surgery in 15 selected poor-risk patients (WFNS SAH Scale IV and V) was much less favourable. Late surgery (4 or more days after SAH) was performed in 28 good-risk patients, most of whom had been admitted several days or weeks after the bleeding. Almost all of these patients had a good outcome. It is argued that the known management results of delayed surgery, which during the deliberately chosen interval exposes the patient to the risk of rebleeding and vasospasm, have by now been surpassed by those of early surgery. However excellent the surgical results of delayed operations may be, early operation should become the treatment of choice in good-risk patients.

Aged↗
PubMed1994

Microvascular decompression for trigeminal neuralgia: a critical reappraisal.

The results of posterior fossa explorations for trigeminal neuralgia over the period 1980-1990 in 58 patients and in 59 procedures were studied retrospectively. In 51 procedures vascular compression was treated by microvascular decompression (MVD). In the absence of such a compression, partial sensory rhizotomy (PSR) was performed in 5 cases and only adhesiolysis of thickened arachnoidea in 2 cases. In one additional case the procedure was terminated prematurely due to the development of cerebellar edema. Two months postoperatively a good or fair result was obtained in 80% of the procedures. At long-term follow-up (mean 77.3 months, range 8-146 months), a good or fair result was maintained in 71%. There was no significant difference in outcome between the MVD group and the other procedures, or between the three groups formed according to the type of vascular compression. However, in the group of 10 patients with a history of a procedure affecting the trigeminal ganglion or nerve root the result was worse. In the group of 41 MVD patients rendered free of pain at 2 months postoperatively, 8 patients perceived a recurrence. The annual recurrence rate was calculated to be 2.6%. There was no mortality in this series but the morbidity rate was 22% including 1.7% persistent neurological deficit. Compared to the literature results of percutaneous controlled differential thermocoagulation (PCDT), the recurrence and failure rates in the present series appear to be more or less the same. As neither of the two is an unequivocally more effective treatment, we are of the opinion that the less invasive procedure should be preferred.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

Treatment of single brain metastasis: radiotherapy alone or combined with neurosurgery?

Most patients treated for single or multiple brain metastases die from progression of extracranial tumor activity. This makes it uncertain whether the combination of neurosurgery and radiotherapy for treatment of single brain metastasis will lead to better results than less invasive treatment with radiotherapy alone. The effect of neurosurgical excision plus radiotherapy was compared with radiotherapy alone in a prospectively randomized trial with 63 evaluable patients with systemic cancer and a radiological diagnosis of single brain metastasis. Radiotherapy was given to the whole brain by a novel scheme of 2 fractions per day of each 2 Gy for a total of 40 Gy. Before randomization, patients were stratified by site (lung cancer vs nonlung cancer) and status of extracranial disease (progressive vs stable). Survival as such and functionally independent survival (FIS; defined as World Health Organization performance status < or = 1 and neurological function < or = 1) were compared between both treatment arms. The combined treatment compared with radiotherapy alone led to a longer survival (p = 0.04) and a longer FIS (p = 0.06). This was most pronounced in patients with stable extracranial disease (median survival, 12 vs 7 mo; median FIS, 9 vs 4 mo). Patients with progressive extracranial cancer had a median overall survival of 5 months and a FIS of 2.5 months irrespective of given treatment. Improvement in functional status occurred more rapidly and for longer periods of time after neurosurgical excision and radiotherapy than after radiotherapy alone.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1992

Arachnoid cysts of the middle cranial fossa. Surgical management for headache.

To answer the question whether headache in association with a middle cranial fossa arachnoid cyst without other signs warrants surgery, an analysis of five cases is performed. Three of 5 patients became free of symptoms, compared with 15 of 19 (79%) patients that have been collected in the literature. Both failures were treated with a low pressure cysto-peritoneal shunt without fenestration. They complained of headache resembling postural headache after lumbar puncture. Surgical treatment in the form of a craniotomy with fenestration can be advised, but is major neurosurgery. Alternatively, the merit of a cysto-ventriculo-peritoneal shunt should be considered in this group of patients.

Adolescent↗
PubMed1992

Interphase cytogenetics: a new tool for the study of genetic changes in brain tumors.

Interphase cytogenetics is the application of nonradioactive in situ hybridization with chromosome-specific DNA probes to interphase nuclei. In this study, interphase cytogenetics was used to investigate 66 primary brain tumors (33 gliomas, 30 meningiomas, and three medulloblastomas) for numerical chromosomal aberrations of chromosomes 1, 6, 7, 10, 11, 17, 18, X, and Y. Of the 33 gliomas (17 astrocytomas grades II, III, and IV, five oligoastrocytomas, seven oligodendrogliomas, and four ependymal tumors), 22 were near diploid, while the remaining 11 showed a significant triploid or tetraploid component. The predominant specific aberrations in gliomas were an over-representation of chromosome 7 (13 cases) and an under-representation of chromosome 10 (16 cases). These changes were observed in grade III and grade IV astrocytomas, as well as in oligodendrogliomas. Other frequent numerical changes were a gain of chromosome 17 (six cases) and a loss of chromosome 18 (seven cases). This loss of chromosome 18 seemed relatively specific for gliomas with an oligodendroglial component (six cases). Only two of 33 gliomas displayed no genetic abnormality with the probes used. Seven patients with astrocytomas died of their brain tumor during the clinical follow-up period. Their astrocytomas did not show a different chromosomal constitution compared to the other gliomas. For the meningiomas, the probe panel was extended with a probe specific for chromosome 22. Loss of chromosome 22 was obvious in 21 of the 30 meningiomas, and was the sole abnormality in 11 meningiomas; in the other 10, this loss was associated with other chromosomal changes. Five of these tumors with additional aberrations were recurrent or atypical meningiomas. It is suggested that interphase cytogenetics can contribute to a better understanding of the biological behavior of these tumors and possibly result in better insights into prognosis and strategies for therapy.

Adult↗
PubMed1991

Interphase cytogenetics of brain tumors.

The development and application of a procedure for interphase cytogenetics on brain tumor material is described. Nuclei isolated from freshly removed brain tumor tissue were investigated for chromosomal aberrations by nonradioactive in situ hybridization with a panel of chromosome-specific probes. The panel consisted of nine satellite DNA probes specific for the centromeric regions of chromosomes 1, 6, 7, 10, 11, 17, 18, X, and Y. For each probe, the number of hybridization signals per cell was determined in 200 nuclei. It was inferred from the hybridization results that in 11 gliomas (seven astrocytomas grade II-IV, three oligodendrogliomas, and one ependymoma) the numerical aberrations were gains of chromosomes 1 (once), 7 (twice), 10 (once), 11 (twice), and X (twice); losses of chromosomes 1 (once), 10 (twice), 17 (twice), and Y (once); and complete tetraploidy (once). Among the 18 investigated meningiomas monosomy 18 and trisomy 17 were observed once and twice, respectively. An additional hybridization with a cosmid probe for the BCR gene on 22q11 indicated monosomy 22q in 11 meningiomas. These results show the value of interphase cytogenetics for the analysis of solid tumors for which it is relatively difficult to obtain sufficient metaphases of good quality for conventional cytogenetics.

Adult↗
PubMed1991

Should all patients with malignant astrocytoma have postoperative radiotherapy?

A retrospective analysis was carried out on 96 patients with malignant astrocytoma treated during the period 1977--1986 to evaluate the contribution of postoperative radiation treatment to survival. In this material the initial Karnofsky performance score, age and extent of resection appeared to be prognostic factors, on the basis of which suggestions are given for selection of patients for radiotherapy.

Adult↗
PubMed1990

Enzyme histochemistry of the spinal cord after experimental transection (Th 9) in the cat.

Subpial complete resection of a 10 mm segment of the spinal cord at Th 9 was performed in 9 adult cats. Topographic enzyme histochemical investigations of the terminal clubs were performed after different survival times after transection in 7 cats and three days after a subsequent one-week-delayed autologous sciatic grafting procedure in the remaining two cats. For acid phosphatase (ACP), the count of active terminal clubs was high (200 per m2) from 12 hours until day 3 after transection. Then the count of active terminal clubs decreased to a low level (20 per m2) and remained the same until day 14. Removal of necrotic tissue and subsequent grafting with autologous sciatic nerve did not change these findings. For succinate dehydrogenase (SDH), the numbers of terminal clubs showed the same pattern at a lower level. The SDH defined terminal clubs were smaller than the ACP ones. The length of the SDH positive area decreased after 7 days while the ACP positive area remained the same until day 14. The SDH active terminal clubs are overgrown by the ACP positive terminal clubs, after the 7th day. Considering that SDH is linked to constructive activity in mitochondria and ACP to destructive activity in lysosomes, this phenomenon might be responsible for the termination of the capacity of the spinal cord tissue to regenerate.

Acid Phosphatase↗
PubMed1988

Haemorrhagic necrosis of the grey matter of the spinal cord due to accidental injection of iopamidol in a patient with multiple neurofibromas; a clinico-pathological study.

Cervical laminectomy was performed in a 34-year-old man with multiple spinal neurofibromas because of a slowly progressive medullary compression. Four weeks later a rapid deterioration necessitated iopamidol myelography by left lateral cervical puncture at C2 level. Despite the establishment of adequate spinal fluid contact, resulting in imaging of the subarachnoid space, part of the contrast medium entered the spinal cord, thus delineating a syrinx from the upper cervical extending to the upper thoracic level. After the puncture the patient developed triplegia, involving the left arm and both legs and a paresis of the right arm. He died from aspiration pneumonia. Autopsy revealed haemorrhagic necrosis of the spinal grey matter. This adverse effect of myelography is argued to have been conditioned by the extreme immobility and displacement of the spinal cord due to the presence of multiple neurofibromas. The deterioration four weeks after the operation was probably caused by a further compression of the spinal cord.

Adult↗