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Purpose: Restorative proctocolectomy with ileo neo rectal anastomosis (INRA) combines cure of ulcerative colitis (UC) or familial adenomatous polyposis (FAP) with restoration of intestinal continuity. Evaluation of long-term results was needed to determine if there is a place for INRA in the armamentarium of a surgeon besides the ileal pouch anal anastomosis (IPAA)., Methods: All patients with INRA were included in the analysis. Patient demographics and clinical and follow-up data (morbidity, dietary problems, defecation frequency, fecal continence, anal and neorectal physiology, and neorectal mucosa assessment) were registered prospectively., Results: Seventy-nine patients were enrolled, and in 58 patients (50 UC, 8 FAP), INRA was successful. In 21 patients, intraoperative conversion to IPAA was needed. In 49 patients with INRA, a functional reservoir was achieved. No pelvic sepsis or bladder or sexual dysfunction occurred. Thirteen patients experienced episodes of reservoir inflammation. Median bowel movements of six (5, 8) with a nocturnal defecation frequency of one were recorded with fecal continence or minor incontinence. Anal manometry and neorectal physiology showed a decrease in resting pressure and an increase in squeeze pressure and maximum tolerated volume. The median follow-up was 8.1 years (6.7, 10.1)., Conclusions: This is an example of a surgical innovation with a theoretical potential to be superior to the current technique. This potential was not confirmed in short- and long-term evaluations. Hence, IPAA is currently the best available alternative to a conventional ileostomy.

Purpose: The MGMT promoter methylation status has been suggested to be predictive for outcome to temozolomide chemotherapy in patients with glioblastoma (GBM). Subsequent studies indicated that MGMT promoter methylation is a prognostic marker even in patients treated with radiotherapy alone, both in GBMs and in grade III gliomas., Experimental Design: To help determine the molecular mechanism behind this prognostic effect, we have conducted genome-wide methylation profiling and determined the MGMT promoter methylation status, 1p19q LOH, IDH1 mutation status, and expression profile on a series of oligodendroglial tumors [anaplastic oligodendrogliomas (AOD) and anaplastic oligoastrocytomas (AOA)] within EORTC study 26951. The series was expanded with tumors of the same histology and treatment from our own archive., Results: Methylation profiling identified two main subgroups of oligodendroglial brain tumors of which survival in the CpG island hypermethylation phenotype (CIMP(+)) subgroup was markedly better than the survival of the unmethylated (CIMP(-)) subgroup (5.62 vs. 1.24 years; P < 0.0001). CIMP status correlated with survival, MGMT promoter methylation, 1p19q LOH, and IDH1 mutation status. CIMP status strongly increases the predictive accuracy of survival in a model including known clinical prognostic factors such as age and performance score. We validated our results on an independent data set from the Cancer Genome Atlas (TCGA)., Conclusion: The strong association between CIMP status and MGMT promoter methylation suggests that the MGMT promoter methylation status is part of a more general, prognostically favorable genome-wide methylation profile. Methylation profiling therefore may help identify AODs and AOAs with improved prognosis.

Recent studies have shown that the clinical outcome of anaplastic oligodendroglial tumors is variable, but also that the histological diagnosis is subject to interobserver variation. We investigated whether the assessment of 1p/19q codeletion, polysomy of chromosome 7, epidermal growth factor receptor (EGFR) gene amplification (EGFR(amp)), and loss of chromosome 10 or 10q offers additional prognostic information to the histological diagnosis and would allow molecular subtyping. For this study, we used the clinical data and tumor samples of the patients included in multicenter prospective phase III European Organisation for Research and Treatment of Cancer (EORTC) study 26951 on the effects of adjuvant procarbazine, chloroethyl cyclohexylnitrosourea (lomustine), and vincristine chemotherapy in anaplastic oligodendroglial tumors. Fluorescence in situ hybridization was used to assess copy number aberrations of chromosome 1p, 19q, 7, 10, and 10q and EGFR. Three different analyses were performed: on all included patients based on local pathology diagnosis, on the patients with confirmed anaplastic oligodendroglial tumors on central pathology review, and on this latter group but after excluding anaplastic oligoastrocytoma (AOA) with necrosis. As a reference set for glioblastoma multiforme (GBM), patients from the prospective randomized phase III study on GBM (EORTC 26981) were used as a benchmark. In 257 of 368 patients, central pathology review confirmed the presence of an anaplastic oligodendroglial tumor. Tumors with combined 1p and 19q loss (1p(loss)19q(loss)) were histopathologically diagnosed as anaplastic oligodendroglioma, were more frequently located in the frontal lobe, and had a better outcome. Anaplastic oligodendroglial tumors with EGFR(amp) were more frequently AOA, were more often localized outside the frontal lobe, and had a survival similar to that for GBM. Survival of patients with AOA harboring necrosis was in a similar range as for GBM, while patients with AOA with only endothelial proliferation had better overall survival. In univariate analyses, all molecular factors except loss of 10q were of prognostic significance, but on multivariate analysis a histopathological diagnosis of AOA, necrosis, and 1p(loss)19q(loss) remained independent prognostic factors. AOA tumors with necrosis are to be considered WHO grade IV tumors (GBM). Of all molecular markers analyzed in this study, especially loss of 1p/19q carried prognostic significance, while the others contributed little prognostic value to classical histology.

The chromosome (chr) 1p deletion is a favorable biomarker in oligodendroglial tumors and is even more powerful a marker when combined with chr 19q loss. As a result, the 1p deletion is taken into account more and more in clinical trials and the management of patients. However, the laboratory technique implemented for detection of this biomarker has been a topic of debate. To illustrate the usefulness of evaluating multiple loci, we here report two anaplastic oligodendrogliomas that were investigated using fluorescent in situ hybridization (FISH) and bacterial artificial chromosome (BAC)-array-based comparative genomic hybridization (aCGH). Indeed, segmental analysis using FISH, limited to chr 1p36 was unable to discriminate between complete and partial deletions of chrs 1p. However, complete and partial deletions of 1p are reported to have distinct clinical outcomes. Our results illustrate that aCGH (or other multiple loci technologies) provide complementary information to single locus technologies such as FISH because multiple loci technologies can evaluate the extent of the chr 1p deletion.

Rhabdoid tumour predisposition syndrome (RTPS) is a rare syndrome caused by inheritance of a mutated INI1 gene for which only two multigeneration families have been reported. To further characterise the genotype and phenotype of RTPS, we present a third family in which at least three cousins developed an atypical teratoid/rhabdoid tumour (AT/RT) at a young age. Two of these patients showed unusual long survival, and one of these developed an intracranial meningioma and a myoepithelioma of the lip in adulthood. Mutation analysis of INI1 revealed a germline G>A mutation in the donor splice site of exon 4 (c.500+1G>A) in the patients and in their unaffected fathers. This mutation prevents normal splicing and concomitantly generates a stop codon, resulting in nonsense-mediated mRNA decay. Biallelic inactivation of INI1 in the tumours, except for the meningioma, was confirmed by absence of nuclear INI1-protein staining. The myoepithelioma of one of the patients carried an identical somatic rearrangement in the NF2 gene as the AT/RT, indicating that both tumours originated from a common precursor cell. In conclusion, this study demonstrates for the first time transmission of a germline INI1-mutation in a RTPS family via nonpenetrant males, long-term survival of two members of this family with an AT/RT, and involvement of INI1 in the pathogenesis of myoepithelioma.

In view of recent progressive insight in the diagnosis and treatment of leptomeningeal metastases of solid tumours, a new guideline has been designed on the initiative of the Dutch Association of NeuroOncology and the Netherlands Society of Neurology, with methodological support from the Dutch Institute for Healthcare Improvement (CBO). - There are no neurological symptoms or signs, nor MRI characteristics that are unique to leptomeningeal metastasis. However, clinical suspicion of leptomeningeal metastasis in a patient known to have cancer, in combination with specific MRI characteristics is sufficient to make the diagnosis. If MRI or CT results are negative or inconclusive cerebrospinal-fluid assessment should be conducted. - Management of care of patients with leptomeningeal metastasis without brain metastases can be based on a series of categories that have been developed using prognostic factors such as Karnofsky performance status, serious encephalopathy or neurological dysfunction, systemic disease, sensitivity of the tumour for chemotherapy or hormonal treatment - In the context of meaningful palliation, systemic treatment, if necessary in combination with radiotherapy to clinically relevant sites, is preferable to intrathecal chemotherapy. - Intrathecal chemotherapy combined with local radiotherapy is recommended if effective systemic treatment is not available, and if the tumour is potentially sensitive to methotrexate, cytarabine or thiotepa. The combination of intrathecal methotrexate and whole-brain radiotherapy should be avoided.

Background/aims: 150 cases of Brunner's gland hamartoma (BGH) have been reported in the literature. BGHs are benign and are thought not to cause bile obstruction., Methods: In this case report, a 60-year-old male is presented with unexplained obstructive jaundice who was also known for over 17 years with diffuse adenomatous hyperplasia of Brunner's glands in the duodenum. Despite the benign preoperative diagnosis, the choice of treatment was Whipple's procedure due to suspicion of a coexisting malignancy., Results: Pathological analysis of the resection specimen revealed multiple BGHs and an adenocarcinoma of the papilla of Vater (PoV). Molecular pathology using loss of heterogeneity analysis was used to confirm that both were different entities., Conclusion: It is likely that previous reports of malignant degeneration of BGHs may actually have been cases involving the coexistence of a PoV adenocarcinoma. Physicians need to be alert when a patient presents with BGH accompanied with obstructive jaundice for simultaneously occurring PoV adenocarcinoma., (Copyright (c) 2007 S. Karger AG, Basel)

Time trends in the incidence of glioma may reflect changes in the prevalence of environmental risk factors for glioma. We therefore investigated trends in the incidence of childhood and adult glioma in The Netherlands from 1989 to 2003. We used population-based incidence data from the Netherlands Cancer Registry. We calculated European standardised incidence rates for glioma, and stratified for age, gender and glioma subgroups. Changes in the incidence were estimated by calculating the Estimated Annual Percentage Change. Similar to other countries, the overall incidence of glioma was fairly stable in The Netherlands during the period 1989 to 2003, for both children and adults. In adult astrocytic glioma, a significantly increasing incidence of high-grade astrocytoma was balanced by simultaneous decreases of low-grade astrocytoma, astrocytoma with unknown malignancy grade and glioma of uncertain histology. Most of these time trends can be explained by improving detection and diagnostic precision. Stable incidence rates of adult and childhood glioma suggest that no major changes in environmental risk factors have occurred, which influenced the incidence of glioma in the studied period.

Background: Little is known about the aetiology of glioma. Research is often hampered by the low incidence and high mortality of the disease. Concomitant diseases in glioma patients may indicate possible aetiological pathways. We therefore studied comorbidity in glioma patients., Patients and Methods: We performed a case-control study using population-based data from the Eindhoven Cancer Registry. We compared prevalences of concomitant diseases in 510 glioma patients with two reference cancer populations from the same registry., Results: Compared with all other cancer patients, a significantly higher prevalence of hypertension was found in glioma patients for age categories 60-74 years [odds ratio (OR) 1.37; 95% confidence interval (CI) 1.02-1.84] and 75+ years (OR 2.37; 95% CI 1.34-4.21). The association was most pronounced in elderly men and in astrocytic glioma, with a maximum in age category 75+ years (OR 5.86; 95% CI 2.20-15.7). The prevalence of cerebrovascular disease was higher in glioma patients >45 years old (OR 1.67; 95% CI 1.12-2.47), whereas the prevalence of other cancers was lower (OR 0.64; 95% CI 0.48-0.87). No consistent associations were detected for several other concomitant diseases., Conclusions: Our data suggest an association between hypertension and glioma, although questions remain about causality and the possible mechanisms. We hypothesise that this association is mediated through potentially neurocarcinogenic effects of antihypertensive medication., (Copyright 2004 European Society for Medical Oncology)

Objective: The goal of our study was to investigate the inverse correlation between number of genetic aberrations and malignancy grade in ependymal tumors at the ploidy level., Methods: we examined seven myxopapillary ependymomas (mpEs) (WHO grade I), 28 spinal and cerebral ependymomas (Es) (WHO grade II), and 18 cerebral anaplastic ependymomas (aEs) (WHO grade III) using image DNA cytometry. The ploidy status was correlated with clinicopathological characteristics and with the results obtained by comparative genomic hybridization (CGH) analysis that we performed in about half of these tumors., Results: mpEs were exclusively located in the spinal cord and aEs in the cerebrum only, whereas Es were located in both the spinal cord and brain. We found aneuploidy or tetraploidy to be common in the group of mpEs (6 out of 7) and much less frequent in Es (6 out of 28) and aEs (4 out of 18). Three-year postoperative survival was 100% for mpEs, 100% for spinal Es, 92% for cerebral Es, and 33% for aEs. Our CGH results in a selection of these tumors revealed the highest number of genetic aberrations in the mpEs (average 16; n = 2), a lower number in Es (average 12; n = 11) and the lowest number in aEs (average 5; n = 6). Interestingly, in the group of Es and aEs, a high number of genetic aberrations as detected by CGH was not correlated with aneuploidy or tetraploidy. Three patients, all with mpEs had local seeding., Conclusion: These results underline that mpEs are distinctly different from Es and aEs at the genetic level and that extensive genomic alterations and aneuploidy in ependymal tumors are not in itself an indicator of malignant behavior.

We describe the neuropathological and biochemical autopsy findings in 3 patients with autosomal dominant adult neuronal ceroid lipofuscinosis (ANCL, Parry type; MIM 162350), from a family with 6 affected individuals in 3 generations. Throughout the brain of these patients, there was abundant intraneuronal lysosomal storage of autofluorescent lipopigment granules. Striking loss of neurons in the substantia nigra was found. In contrast, little neuronal cell loss occurred in other cerebral areas, despite massive neuronal inclusions. Visceral storage was present in gut, liver, cardiomyocytes, skeletal muscle, and in the skin eccrine glands. The storage material showed highly variable immunoreactivity with antiserum against subunit c of mitochondrial ATP synthase, but uniform strong immunoreactivity for saposin D (sphingolipid activating protein D). Protein electrophoresis of isolated storage material revealed a major protein band of about 14 kDa, recognized in Western blotting by saposin D antiserum (but not subunit c of mitochondrial ATPase (SCMAS) antiserum). Electron microscopy showed ample intraneuronal granular osmiophilic deposits (GRODs), as occurs in CLN1 and congenital ovine NCL. These forms of NCL are caused by the deficiencies of palmitoyl protein thioesterase 1 and cathepsin D, respectively. However, activities of these enzymes were within normal range in our patients. Thus we propose that a gene distinct from the cathepsin D and CLN1-CLN8 genes is responsible for this autosomal dominant form of ANCL.

Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma, is a rare cerebellar lesion. It has long been regarded as avascular. We report two patients with surgically proven LDD in whom contrast enhancement was observed on MRI. Neuropathological examination revealed proliferation of veins. We suggest that peripheral enhancement of LDD probably reflects vascular proliferation of the cerebellar venous draining system, and should be considered part of the imaging features of LDD.

We describe a family with adult neuronal ceroid lipofuscinosis, with apparent autosomal dominant inheritance, observed in six affected individuals in three generations. Disease onset was usually in the fifth decade, but was earlier in the youngest generation. Early symptoms consisted of myoclonus in face and arms, epilepsy, auditory symptoms, cognitive decline, or depression. Parkinsonism occurred a few years after disease onset, with stooped posture, shuffling gait, bradykinesia, and mask face. Four subjects deteriorated to a state of severe handicap, with severe dementia, contractures, dysphagia, and dysarthria. Leg weakness evolved to flaccid paraparesis in two patients. Diagnosis was confirmed by brain biopsy in one patient and full autopsy in two patients. Abundant intraneuronal storage of autofluorescent material was found throughout the brain. Electron microscopy showed granular osmiophilic deposits and scarce fingerprint profiles. Striking loss of neurons in the substantia nigra pars compacta and reticulata was found. (123)I-IBZM Single photon emission computed tomography in two patients showed loss of postsynaptic D2 receptor binding in the striatum. We conclude that parkinsonism in ANCL is likely to be caused by both presynaptic nigral cell loss and postsynaptic striatal degeneration., (Copyright 2002 Movement Disorder Society.)

In a longitudinal prospective study a muscle biopsy was taken from 30/32 (33%) of the 98 patients who developed critical illness polyneuropathy and myopathy (CIPNM). Neuropathic changes were found in 37%, myopathic in 40%, and a combination in 23% of the biopsies. The immunohistopathology showed macrophages and Th-cells in 40% and 60% of the muscle biopsies respectively. Small mainly perivascular infiltrates contained macrophages and Th-cells. ICAM-1, VCAM and MAC were found on the vascular endothelium in 58%, 53% and 79% respectively. In all biopsies there was an upregulation of both HLA-I and HLA-DR. Proinflammatory cytokines and TNFalphaR75 were also produced locally (IL-1beta in 71%, IFN-gamma in 40%, IL-12 in 73%, TNFalphaR75 in 90%). The anti-inflammatory cytokine IL-10 was simultaneously expressed in 96% of the biopsies. HLA-DR, TNFalphaR75 and IL-10 differed significantly when compared with control muscle biopsies. Our data provide evidence that small numbers of activated leukocytes producing both pro- and anti-inflammatory cytokines infiltrate skeletal muscle of CIPNM patients. We propose that the local balance of leukocyte activities is of importance in the pathophysiology of muscle weakness in CIPNM.

A couple consulted the gynaecologist repeatedly because of a primary fertility disorder. When they had desired children for 8 years, no pregnancy had yet occurred. In-vitro fertilization (IVF) treatment was started. A few days after the beginning of the hormonal treatment that precedes the IVF procedure, the woman noted a swelling in the right inguinal area for which she consulted the surgeon. This swelling was found to be a metastasis of a melanoma that had been removed from her leg 6 months previously. The gynaecologist was not aware of this case history and the surgeon was not informed of the started IVF treatment. As was found later, this was in accordance with the couple's wishes. The first IVF treatment resulted in a pregnancy that ended with the birth of a healthy boy. The placenta showed melanomatous metastases and the mother died 2 months after the birth of her son.

Chemical analysis of the contents of a so-called bright epidermoid of the posterior fossa with unusual CT and MR imaging characteristics suggested that a combination of high protein content and high viscosity were responsible for the atypical imaging findings.

Deep cerebral vein thrombosis can present with acute, severe neurological symptoms and may be rapidly fatal as in the 20-year-old woman reported here. Although MRI is superior for establishing the diagnosis, CT is usually the first examination performed in the clinical setting. It is therefore important to recognise certain indicators such as extensive bithalamic low density. These and certain other less specific signs are correlated with the MRI and autopsy findings.

The authors describe the radiological and pathological features in a patient with an intracranial chondroma originating in the falx cerebri. Diagnostic procedures and management in treatment are discussed, and a review of the literature is presented.

At present a reliable and specific diagnostic test of Alzheimer's disease is not available. Thus far, diagnosis is based on clinical criteria despite their occasional inadequacy. Post mortem search for neuropathological hallmarks can establish the diagnosis with certainty. In the present case control study we performed an apoE genotyping for 21 patients, divided into an Alzheimer-positive and an Alzheimer-negative group after neuropathological search. As described in the literature, the apoE-epsilon 4 allele was overrepresented in the Alzheimer-positive group, while in the Alzheimer-negative group the apoE-epsilon 3 allele dominated. The epsilon 4 allele of the apoE gene may be considered as a biological risk factor for the development of Alzheimer's disease. Especially in geriatric patients with cognitive impairment, apoE genotyping seems to be a supplementary tool for risk assessment.

Two patients with chronic pulmonary disease, a woman of 38 and a man of 54 years old, who had developed a status asthmaticus, had difficulties being weaned from artificial ventilation. They suffered from an acute myopathy caused by a combination of high-dose corticosteroids and muscle relaxants (pancuronium, vecuronium). This acute myopathy is characterised by generalised flaccid quadriplegia with muscle atrophy and areflexia, difficulties being weaned from artificial ventilation, myoglobinuria and high levels of creatine kinase activity in serum. The prognosis is good; almost complete recovery occurs. Muscle biopsy may reveal necrotising myopathy and occasionally, selective loss of thick myofilaments.

We present a 39-year-old man with tumour of the eighth thoracic vertebra, causing compression of the spinal cord. The tumour proved to be a primary leiomyosarcoma (LMS) of bone, an uncommon neoplasm; to our knowledge this is the first report of primary LMS in the spine. The lesion was documented by plain radiography, myelography, CT, MRI and digital subtraction angiography. These investigations did help to focus on the differential diagnosis and demonstrated the extent of the bony lesion, the findings were nonspecific, and the correct diagnosis was established by pathological examination.

We present a case of so-called olfactory schwannoma in a 17-year-old male. The diagnostic procedures included computed tomography and digital subtraction angiography. Histological examination with tumor markers, S100, Leu-7 (CD57) and EM showed the tumor tissue to be a schwannoma. Arguments are raised that olfactory schwannomas are comparable to or even occur as intracerebral schwannomas with frontal localisation.

A bipolar cuff electrode for electrical stimulation of small diameter peripheral nerves is described. The cuff is made of a highly flexible rubber-impression material, and the electrode assembly is suited for chronic implantation. Its manual construction is easy and reliable, utilizing only simple tools. The cuff completely envelopes nerves of varying diameter and requires a minimal amount of manipulations of the nerve, thereby reducing the chance of surgical trauma. The snug envelope prevents the nerve from drying, and minimizes shunting between the two leads by extracellular fluids. Small outer dimensions were achieved: 1.4 x 1.1 x 2.3 mm (width x height x length) when used with nerves of 1 mm diameter, which minimizes pressure and damage to surrounding tissues. Morphometric analysis of nerves enclosed in cuffs for 28-30 h revealed a small decrease in the number of large-diameter fibers. Stimulation thresholds remained, however, constant throughout the experiments.

A family with anaplastic ependymomas, histologically verified in three cases and neuroradiologically suggested in a fourth, is presented. Two healthy brothers both had two affected sons. All four male patients were younger than 5 years at the time of diagnosis. Two boys died before the age of 3 years. Genotype analysis (with polymorphic DNA markers for chromosome 22 and interphase cytogenetic analysis) of one of the tumours showed a subpopulation of tumour cells with monosomy of (part of) chromosome 22. Non-neoplastic cells of this patient showed a normal karyotype. These findings give further evidence for the role of a tumour suppressor gene on chromosome 22 in the pathogenesis of familial ependymal tumours.

Up to now, 71 critically ill patients have been reported with neuromuscular complications after artificial respiration. The authors review the literature and present data of a personal series of 22 patients all suffering from severe flaccid tetraparesis and muscle atrophy, which developed after an average of two weeks artificial respiration. The prognosis was relatively good in those surviving the primary disease. The multiconditional causes are discussed with emphasis on the combination of polyneuropathy and myopathy. Tumor necrosis factor (TNF), a key mediator of sepsis, which also has an influence on muscle and nerves, is mentioned as a possible cause of this illness.

A 30-year-old multigravida woman was admitted to the obstetrical unit in the third trimester of gestation, because of a large-for-date uterus. Repeated ultrasonic examinations over a 2-week period revealed an increase in biparietal diameter far too large to be normal. This progressive asymmetrical hydrocephalus was diagnosed as caused by an intracranial space-occupying lesion in the middle cranial fossa. Because of the only minimal thickness of the cerebral cortex prognosis was judged so poor, that labor was induced. A stillborn female infant was delivered vaginally after an ultrasonically guided transabdominal and later transcervical encephalocentesis. Autopsy revealed hydrocephalus and section through the fixed brain showed that the mentioned mass was an undifferentiated meningeal sarcoma that filled the left middle cranial fossa. Microscopically the sarcoma was invading the brain tissue. Definitions of the term "congenital brain-tumors" vary among authors. "Congenital" can be interpreted as "derived from embryonal tissue" or as "originating in utero". Congenital intracranial neoplasms are very rare. Antenatally intracranial tumors have been reviewed by Wells, Solitare and Farwell. Only a few cases of congenital brain tumors arising in the meninges have been reported in these reviews. The genesis of the tumor is explained as derived from multipotential cells. The undifferentiated type of a meningeal sarcoma has a poor prognosis. Most meningeal sarcoma present with progressive hydrocephalus.(ABSTRACT TRUNCATED AT 250 WORDS)

The clinical and neuropathological findings are reported of two sibs with adult type PMD. Clinical features deviating from the usual pattern included: no psychosis, no measurable dementia, no dwarfism, no microcephaly, no (marked) involuntary movements, but conspicuous generalised muscle atrophy and denervation, impairment of vital and gnostic sensation, thoracolumbar vertebral anomalies, and aplasia of os coccygis. Neuropathological findings were as usual, with additional unusual features: pinhead-size areas of acute myelin-abbau products, involvement of grey in addition to white matter, and upon ultrastructure, the new finding of intra-oligodendroglial fingerprint bodies, both in neuronal satellite and in white matter oligoglia, but not in astrocytes, ganglion cells, or pericytes. This excludes the origin of the stored material in the lysosomes as to derive exclusively from demyelination and would possibly imply PMD to be an oligodendroglial lysosomal storage disease.

An alveolar soft part sarcoma localised in the pituitary gland extended into the suprasellar region. Three years after surgical removal there was a recurrency in the occipital horn of the right cerebral ventricle. This type of tumour predominantly occurs in the limbs and has never been reported in the pituitary region.