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The distribution of the messenger RNA for alpha-tubulin has been investigated by in situ hybridization in the human hippocampus and temporal cortex in normal subjects and in Alzheimer's disease. The alpha-tubulin mRNA was strongly expressed in neurons in the gyrus dentatus, in the Ammon's horn and in cortical layers of the temporal cortex. The same distribution was observed in Alzheimer's disease. An important reduction of the hybridization signal was apparent, however, in areas rich in neurofibrillary lesions, e.g. as in layer II of entorhinal cortex. Neurons containing neurofibrillary tangles exhibited a weaker hybridization signal than adjacent neurons devoid of neurofibrillary tangles. The immunoreactivity for alpha-tubulin was drastically reduced in tangles-bearing neurons. These results indicate that tubulin transcription is reduced in tangles-bearing neurons, a reduction which might play a role in the reported decrease in the number of microtubules in neurons containing neurofibrillary tangles.

Subdural haematoma (SDH) caused by meningioma is infrequent. 18 cases are described in the literature. We report 2 new cases. Intratumoural bleeding is a frequent feature of this uncommon association.

BACKGROUND: The authors studied the benefit of performing histopathologic grading and DNA ploidy characterization with respect to patient survival in a series of 206 astrocytomas (AST) for which they obtained 134 complete clinical follow-ups. METHODS: The material analyzed came from archival material, i.e., formalin-fixed paraffin-embedded tissues. DNA ploidy was assessed by means of a cell image processor computing the integrated optical density (IOD) on Feulgen-stained nuclei. RESULTS: Results showed that histopathologic diagnosis in three grades, i.e., AST, anaplastic astrocytoma (ANA), and glioblastoma multiforme (GBM), had a significant prognostic value. Patients with AST showed a mean survival time (between histopathologic diagnosis and death) of more than 36 +/- 6 months (AST versus ANA or GBM) (P less than 0.001). Patients with ANA and GBM showed a mean survival time of 15 +/- 2 and 10 +/- 1 months, respectively, (ANA versus GBM) (P less than 0.05). Patient age strongly correlated with survival. Patients younger than 40 years of age had a mean survival time of 20 +/- 4 months. Patients between 41 and 60 years of age had a mean survival time of 12 +/- 2 months, and patients older than 60 years of age had a mean survival time of 11 +/- 1 months. CONCLUSIONS: Considering DNA ploidy characterization, the authors noticed that aneuploid ANA (DNA index [DI] more than 1.30) were associated with a significantly higher mean patient survival time compared with that associated with euploid ANA. In contrast, the authors did not find this in either of the groups with AST and GBM. Recognizing six DNA histogram types (diploid, triploid, tetraploid, hyperdiploid, hypertriploid, and polymorphic), the authors observed that hypertriploid tumors were associated with greater patient survival compared with what happened in the cases of the five other DNA histogram types. This was true with respect to the three AST histopathologic types. Thus, DNA ploidy determination seemed helpful in characterizing aggressiveness in adult AST.

Between January 1984 and December 1990. 65 intramedullary spinal cord tumors were diagnosed and operated on. In this series, all patients underwent magnetic resonance imaging investigations and were operated on with the Cavitron ultrasonic surgical aspirator whenever necessary. Major surgical difficulties have been found in patients previously treated by radiotherapy with or without biopsy. We found magnetic resonance imaging to be a highly sensitive imaging procedure and the method of choice for visualizing tumors within the spinal cord. Nevertheless, accurate diagnosis may only be suggested by magnetic resonance imaging, rather than made definitively. Surgery is necessary in every case in order to obtain a definite diagnosis. Radical surgery can be performed when a plane exists between the tumor and the normal spinal cord: biopsy or debulking with the Cavitron ultrasonic surgical aspirator should be performed when the tumor is infiltrative. We have performed 33 so-called total resections, 22 partial resections, and 10 biopsies, among which 5 were performed on lipomas. Surgical results were assessed at 3 months after surgery, showing 35 improvements (53%), 24 stabilizations (37%), and 6 deteriorations (10%).

Paragangliomas of the cauda equina are not so rare as said in the literature. Two additional cases are presented with a global analysis of the 59 cases from the literature. The diagnosis of this pathology greatly benefit of the use of immunostainings as the cells are often neuron-specific enolase, neurofilament protein and somatostatin positive so that electron microscopy is thus no longer mandatory for establishing the diagnosis. In addition, we report the first magnetic resonance images of this tumor at this location.

A cDNA encoding epitopes present in paired helical filaments (PHF) in Alzheimer's disease has been characterized by DNA sequencing and by immunochemical analysis of the encoded recombinant protein fragment. The cDNA encodes 773 amino acids specific of the rat high molecular weight microtubule-associated protein MAP2. This amino acid sequence is localized in the side arm projection domain of MAP2. An antiserum raised against the expressed fusion protein encoded by this cDNA labels the high molecular weight MAP2 but not the 70 kDa MAP2c abundant in developing brain. This antiserum labels PHF in situ but only a proportion of isolated PHF. In situ hybridization on human brain sections did not demonstrate an increase in mRNA for MAP2 in tangle-bearing neurones. We suggest that the association of this MAP2 fragment with PHF in situ results from posttranslational modifications of MAP2.

A case of cylindrical spinal meningioma is reported. This most unusual morphology suggested a preoperative diagnosis of either lymphoma, metastatic epidural tumor, or meningioma. At operation, the tumor was found to be hard and extremely adherent to the cord, so its anterior part had to be left in place. Transient paraplegia was observed postoperatively, but the patient recovered normal stance and gait within 6 months. Three years after the operation, magnetic resonance imaging demonstrated a very slow progression of the remaining tumor with an estimated increase of 5%. The differential diagnosis of this lesion by magnetic resonance, its clinical postoperative evolution, and the surgical strategy are discussed.

Review of 500 consecutive MRI studies was undertaken to assess the frequency and the appearances of cystic pineal glands. Cysts were encountered in 2.4% of cases. Follow-up examination demonstrated no change in these cysts and they were considered to be a normal variant. Size, MRI appearances and signs associated with this condition are reported in order to establish criteria of normality.

✓ High-grade astrocytic tumors constitute the most serious as well as the most common group of primary brain tumors. Although several prognostic factors have been proposed, little is known about the prognostic value of deoxyribonucleic acid (DNA) ploidy in adult astrocytic tumors. In a series of 146 adult patients, aged 16 to 82 years, the individual prognostic values of six variables were studied, namely: tumor histopathological grade, treatment, patient age, extent of tumor, ploidy level, and DNA histogram type. Cox's proportional hazard model was then applied to the data to ascertain which factors might independently determine patient survival. Univariate analyses revealed that histopathological grade, age, and DNA histogram type were very powerful prognostic factors. The statistical significance of the influence of adjuvant radiotherapy and chemotherapy was at a borderline level, and the two remaining variables (tumor extent and ploidy level) had no prognostic relevance. Multivariate analyses showed that age, histopathological grade, and DNA histogram type were independent, statistically significant prognostic factors. A prognostic score was calculated from Cox's polynomial function in which those factors were introduced. The best score corresponded to a patient aged 16 years with a hypertriploid low-grade astrocytoma, while the worst score corresponded to a patient aged 82 years with a diploid high-grade astrocytoma. The worst score:best score ratio revealed a risk 71 times higher for a bad prognosis. It is concluded that patient age, histopathological grade, and DNA histogram type are very powerful prognostic factors for adult astrocytic tumors. A prognostic score including those factors could be used to characterize astrocytic tumor aggressiveness presurgically on fine-needle aspirates, and to monitor the patient's postsurgical evolution to define the appropriate therapy.

Neurofibrillary tangles are a neuronal change observed in various conditions, linked with dementia when affecting the cerebral cortex as in Alzheimer's disease. They may be found locally close to fibrous or vascular tumors, or affect extensive regions of the neocortex while the cerebellum and the medulla are not affected. Recent immunological and biochemical studies demonstrate that the MT-associated protein tau is the main component of the tangles, in an abnormally phosphorylated state. A consequence of the formation of tangles is a decreased assembly of MT in axons and dendrites, with disturbances of neuroplasmic flow. The relations between tangles and amyloid, as seen in Alzheimer's and Down's diseases are topographical, tangles accumulating in particular in neurites close to the amyloid in the senile plaques (but also at distance in cell bodies and neurites). Genetically and biochemically A4 or beta-amyloid and tau differ. The exact relation between the beta-pleated proteins of tangles and amyloid remain poorly understood.

Between January 1984 and December 1990, 65 intramedullary spinal cord tumors were diagnosed and operated on. In this series, all patients underwent magnetic resonance imaging investigations and were operated on with the Cavitron ultrasonic surgical aspirator whenever necessary. Major surgical difficulties have been found in patients previously treated by radiotherapy with or without biopsy. We found magnetic resonance imaging to be a highly sensitive imaging procedure and the method of choice for visualizing tumors within the spinal cord. Nevertheless, accurate diagnosis may only be suggested by magnetic resonance imaging, rather than made definitively. Surgery is necessary in every case in order to obtain a definite diagnosis. Radical surgery can be performed when a plane exists between the tumor and the normal spinal cord: biopsy or debulking with the Cavitron ultrasonic surgical aspirator should be performed when the tumor is infiltrative. We have performed 33 so-called total resections, 22 partial resections, and 10 biopsies, among which 5 were performed on lipomas. Surgical results were assessed at 3 months after surgery, showing 35 improvements (53%), 24 stabilizations (37%), and 6 deteriorations (10%).

Immunolabelling for synaptophysin and chromogranin A, two polypeptides associated with small clear and large dense core synaptic vesicles respectively, has been performed on tissue sections of the temporal cortex in Alzheimer's disease in combination with anti-A4 amyloid labelling. The dystrophic neurites in many senile plaques were observed to be labelled by the anti-synaptophysin or anti-chromogranin A antibodies. Some diffuse amyloid deposits, demonstrated by antibodies against synthetic amyloid A4 peptides, were associated with a punctuate increase in synaptophysin or chromogranin A immunoreactivity. The labelling of dystrophic plaque neurites may reflect the accumulation in these processes of synaptic vesicles or material derived from them. We suggest also that the punctuate increase in synaptophysin and chromogranin A immunoreactivities associated with some A4 amyloid deposits may be an early event reflecting neuronal dysfunction., Journal Article, Research Support, Non-U.S. Gov't, info:eu-repo/semantics/published

The study reported was intended to compare the impressions and analyses of investigators from 11 different laboratories on 2 slides, each from 6 cases with varying quantities of neuropathological change of the type found in Alzheimer's disease and normal ageing. The material came from 6 selected female patients over 75 years of age all of whom had been examined in detail and assessed by the Blessed Test Score. Two were severely demented, 2 mildly demented and 2 were considered to be normal. Unstained paraffin-embedded slides were sent to the investigators, the choice of the staining techniques being left to each laboratory. A quantitative evaluation of the changes was requested in 2 specified areas of the hippocampus and in the first temporal gyrus. Subjective scores of severity and a final guess about the pre mortem intellectual status (demented or not) were asked. The 11 replies were analyzed. A total of 14 different staining techniques were used. Absolute values of density differed much from one investigator to another, for senile plaques as well as for neurofibrillary tangles. Statistical analysis showed that concordance might be improved by the use of corrective factors which would standardize the scales of measurement. The ranking of the slides in increasing order of severity was in good agreement for 9 out of 11 observers concerning the neurofibrillary tangles and 3 out of 9 observers concerning the senile plaques. The correlation between the intellectual status and the density of lesions was higher for neurofibrillary tangles than for senile plaques. The subjective scores were in better agreement for the severely affected cases than for the mildly affected ones. The lowest correlation with intellectual deficit was obtained with the quantitative scores which took into account only the senile plaques or only the hippocampal lesions. The highest correlation coefficients were obtained with the subjective scores. The observers guessed correctly the intellectual status of the 2 most affected cases and often disagreed for the intermediate and normal cases. Neuropathology is mandatory for the diagnosis of definite Alzheimer's disease. Quantitative assessment is useful in cases with few lesions and light dementia but the neuropathological diagnostic procedure has to be more strictly standardized before quantitative histopathological criteria can be reliably transferred from one laboratory to another, especially when mildly affected cases are involved. Concordance seems presently easier to obtain by ranking the lesions and the cases in increasing order of severity than by using quantitative values of density.(ABSTRACT TRUNCATED AT 400 WORDS)

Abnormal filaments (PHF) accumulate in neurons in Alzheimer’s disease in target areas. They fill the pericaryon but are also found in dendrites and axons. Their presence is associated with a disappearance of microtubules and neurofilaments, and an accumulation of dense bodies, altered mitochondria and smooth endoplasmic reticulum. Their ultrastructure differs from normal cytoskeletal fibers but one of their main components are the tau proteins. Senile plaques are composed of a core of amyloid fibers surrounded by abnormal neurites containing PHF and accumulations of organelles, reactive astrocytes and microglia.

We report a case of isolated intramedullary sarcoidosis. The patient developed progressive signs that indicated a spinal tumor, which were investigated with contrast-enhanced magnetic resonance imaging scans. Magnetic resonance imaging clearly revealed an intramedullary lesion, but the diagnosis of sarcoidosis was made on the pathological analysis of the surgical specimen. Magnetic resonance imaging with contrast enhancement is reported in a histologically proven case of intramedullary sarcoidosis. Only 12 other cases of isolated intramedullary sarcoidosis have been reported. We review and discuss these cases according to their clinical presentation, the segmental location of the granulomas in the spinal cord, preoperative and operative diagnoses, and signs for systemic sarcoidosis. In none of the cases was the diagnosis of intramedullary sarcoidosis made before surgery. We think that surgical therapy for intramedullary lesions is the best way to diagnose rare instances of benign lesions like sarcoidosis and to treat them in an appropriate manner.

The authors describe the case of an elderly woman exhibiting the clinical signs of occlusion of the anterior spinal artery at the cervical level. Pathological findings indicate that the material occluding not only this vessel but also the satellite veins is fibrocartilage. The authors review the literature concerning this unusual situation and discuss the mechanism by which the fibrocartilaginous emboli, in their case, are both arterial and venous. Large arteriovenous shunts within and around the spinal cord are likely to explain such a disturbing pathologic finding.

A striking enlargement of peripheral nerves was observed in a patient with myotonic dystrophy (Steinert's disease). Clinical findings disclosed a polyneuropathy which was confirmed by electrophysiological and morphological investigations. Myopathic as well as neurogenic alterations were found on electromyography in wasted hand muscles. The prominent finding on ultrastructural study of a sural nerve biopsy was an important collagen infiltration with rather few “onion bulb” formations. This extensive endoneural collagen formation appeared to be responsible for the nerve hypertrophy. Evidence of segmental demyelination and remyelination was also found. Glycogen deposits were present in some myelinated axons and in some Schwann cells. Peculiar crystalline inclusions were observed in fibroblasts processes. The relation between myotonic dystrophy and polyneuropathy remains a matter of discussion.

SummaryA white male patient 38 years old has contracted leprosy in Africa. After several years of incubation, dermatological manifestations occured (erythemato-squamous patches mainly located on the trunk) together with neurological signs (distal anesthesia of lower limbs going up to the thighs and localized zone of thermoanalgesia around the right forearm). Histological and immunological findings enabled to classify the disease within the « border-line » group, with a lepromatous tendency. A review of recent data concerning leprous neuritis emphasizes its close relationship with the immunological response of the host. The therapeutic prescriptions resulting from this relationship are described.

Enriched fractions of "tangles" obtained from cases of Alzheimer's disease have been observed by negative staining and platinum: iridium shadowing, and also after treatment with sodium dodecyl sulfate and urea. Abnormal filaments with a 80-nm periodic constriction exhibited a substructure consisting of six protofilaments. They also showed a periodic right-handed twist. Some fibrils with a 160-nm periodic constriction were made up of four protofilaments. These features are discussed in view of known fibrous protein models, especially neurofilaments.

Recently, an immunocytochemical method using glutaraldehyde fixation and an antiserum developed against a GABA--glutaraldehyde--protein conjugate has permitted direct visualization of GABAergic structures in the brains of perfused animals. This paper reports a successful use of this technique on human temporal cortex fixed by immersion. The cerebral tissue was obtained from patients operated for focal epilepsy. GABA-positive somata, fibres and terminals are observed in all layers of the temporal cortex. Terminals are particularly abundant in the superficial portion of layer I and in layers II, III and IV. Dense plexuses of fibres are located in layers II, III, IV and VI and in the underlying white matter. Somata are found in all cortical layers and in the underlying white matter; they are round, oval, fusiform or triangular and exhibit a multipolar, bitufted or bipolar dendritic pattern. This technique for the visualization of GABAergic structures in the human brain may allow a better understanding of the pathogeny of epilepsy in which the GABAergic transmission has been implicated.

The case of a solitary cerebellar abscess in a 36-year-old Rwandese male is described. First it was considered to be tuberculosis, but at microscopal examination of the lesion after neurosurgical excision the true germ was found: Blastomyces dermatitidis. This solitary intracerebellar blastomycosis abscess is only the fifth case reported worldwide and the first in African medical annals.

Spongiform lesions were observed in close association with typical presenile alterations in three of six frontal lobe biopsies taken in presenile demented patients without any familial relationship. These findings are discussed in relation with recent investigations indicating the production of spongiform lesions after inoculation of nervous tissue from an Alzheimer patient to animals and the effects of saline extract of such material on cultured nerve cells.

Several antibodies directed against the heterogeneous microtubule-associated protein group tau have been used to determine the immunocytochemical localization of these proteins in the developing rat cerebellum. Immunoblot analysis of brain extracts showed that both monoclonal and polyclonal anti-tau antibodies revealed not only the adult tau proteins (50,000-70,000 mol. wt) but also the immature (48,000 mol. wt) tau form. Immunocytochemical studies showed that, whatever the stage of development, anti-tau antibodies stained several types of axonal fibres. The Purkinje cell bodies and their dendrites were never significantly labelled. This means that immature tau is, as adult tau, localized essentially in axons. Axonal labelling seems to follow the cerebellar developmental pattern. For instance, the climbing fibres which reach the cerebellum during the embryonic life were stained soon after birth by the anti-tau antibodies. In contrast, the parallel fibres, that begin to develop perinatally, do not express tau at early (5 days) postnatal stages; a clear labelling of the deeper parallel fibres (which are more mature than the superficial ones) was seen at day 10 after birth in the vicinity of the developing dendrites of the Purkinje cells. This suggests that (1) the appearance of tau immunoreactivity reflects a certain stage of maturity of the parallel fibre; (2) both immature and mature tau microtubule-associated proteins seem to be axon specific in the developing rat cerebellum.(ABSTRACT TRUNCATED AT 250 WORDS), Journal Article, Research Support, Non-U.S. Gov't, SCOPUS: ar.j, info:eu-repo/semantics/published

Counts of neurofibrillary tangles and senile plaques were performed in two adjacent sections from the temporal lobe in 14 women aged over 75 years at death. The mental status of these cases had been prospectively assessed by the test score of Blessed, Tomlinson and Roth. They were either intellectually normal or had senile dementia of the Alzheimer type of various degrees of severity. The first section was silver-impregnated according to Bodian's method; the second one was immunolabeled with an antiserum raised against neurofibrillary tangles. Densities of neurofibrillary tangles and senile plaques evaluated by the two techniques were highly correlated but their means of differences and limits of agreement were large. The correlation with the clinical data was similar for the two methods. Both techniques can thus be used with the same accuracy in correlative studies but their results are not interchangeable without caution.

The authors present the case of a child aged 7 years who suffered from relapsing acute lymphocytic leukemia. Treatment consisting mainly of oral and intrathecal methotrexate and x-ray therapy produced remission of the hematologic symptoms. Three years after the onset of the leukemia, mental deterioration gradually appeared. Radiography of the skull revealed diffuse bilateral calcium deposits in both cerebral and cerebellar hemispheres. Four years after the onset of the disease, a hematologic relapse occurred. Behavioral disorders became more severe and the child died after a period of seizures and unconsciousness. The main pathologic data obtained by the study of a brain biopsy and after a complete postmortem examination consisted of calcifications located bilaterally in the cerebral and cerebellar cortex. No signs of leukemia were present. Cerebral calcification is an extremely rare complication in the course of the therapy of lymphocytic leukemia. Its possible causes are discussed.

Two cases of Menkes disease belonging to different families are reported in order to illustrate different types of central nervous system involvement. In the first case which documents the sex-linked recessive inheritance, arteriographic studies have shown that the vascularization of the brain was severely impaired. Postmortem examination revealed lesions of the elastic membranes of large and medium calibre arteries, widespread and extremely severe necroses of grey and white matter with relative sparing of the territories irrigated by the vertebro-basilar system. The dendritic tree of the Purkinje cells was atrophied, somal sprouts were not found. Electron microscopy showed the mitochondria to be morphologically normal. In the second case, there was a bilateral demyelination predominating in the temporal lobes' white matter while arterial alterations and anoxic lesions were present but less prominent. The Purkinje cells were normal.

Two muscle biopsies were made in a case of chloroquine neuromyopathy in a 57-year-old woman with discoid lupus erythematosus. Histological study demonstrates widespread granulovacuolar degeneration and alterations of terminal innervation. Ultrastructural changes include formation of myelin figures and autophagic vacuoles. Of particular interest is the amount and mode of recovery from such alterations 2 months after cessation of the treatment. © 1973 S. Karger AG, Basel., SCOPUS: ar.j, info:eu-repo/semantics/published

The axonal flow of neurosecretory elementary granules has been studied in the paraventricular neurons of the rat (PVN), with the help of three techniques: light microscopy, radioautography after labelling with 35S-L-cysteine, and electron microscopy. Colchicine treatment does not alter the uptake of 35S cysteine in the PVN but the flow of labelled neurosecretory material towards the neurohypophysis is interrupted. Interruption of the axonal flow is also evidenced by the stagnation of neurosecretory granules at the periphery of the neuronal cytoplasm and by the presence of numerous axonal swellings, heavily loaded with neurosecretory granules and often containing abnormal elongated granules, surrounded by a single membrane, oriented more or less parallely to the long axis of the axons. Other cell organelles and neurotubules are not altered. The present experiments bring further evidence of the arrest by colchicine of the axonal flow of secretory granules without apparent changes of the neurotubules.

Bilateral visual impairment, associated with amenorrhea, diabetes insipidus and mental deterioration were observed in a 28 year old woman. CT scan revealed bilateral symmetrical infiltrative processes surrounding the ventricular system, which was confirmed by postmortem examination. Histologically the lesion was a germinoma.

Tanycytes in the median eminence (ME) of the rat exhibit morphological features suggesting their possible participation in transport phenomena. After adrenalectomy, which modifies the hypothalamo-hypophyseal axis, they undergo morphological changes characterized by an accumulation of lipid droplets, an increased number of bleb-like protrusions at their apex, as well as an increased pinocytosis of intraventricularly injected horseradish peroxidase (HRP). In addition, after adrenalectomy an increased number of vacuoles appears at the level of the tubero-infundibular sulci. Their intracellular location in the tanycytes is demonstrated by an intraventricular injection of HRP. The significance of these vacuoles is discussed in relation to the hydroelectrolytic modifications associated with the state of adrenalectomy., Comparative Study, Journal Article, Research Support, Non-U.S. Gov't, info:eu-repo/semantics/published

The authors describe a case of inferior red nucleus syndrome due to a solitary intramesencephalic metastasis from a glandular epithelioma of the prostate. They emphasize the rarity of cerebral metastases of prostatic origin, particularly at the level of the brain stem, and the exceptional features of a red nucleus syndrome originating from a tumour.

Pituicytes are peculiar glial cells found in the posterior lobe of the pituitary, in close proximity to axonal endings loaded with neurosecretory granules. They are easily recognizable, contain a large spheroid nucleus, a prominent Golgi apparatus, numerous free ribosomes, large lipid droplets, and an occasional centriole. Microtubules are present in the pituicyte cytoplasm and in their processes surrounding axonal neurosecretory endings. Since the first description of these cells by BUCY (1930), numerous studies of pituicytes have appeared (GERSH, 1939; ORTMANN, 1951; RENNELS and DRAGER, 1955; LEVEQUE and SMALL, 1959; LEVEQUE et al., 1966; WITTKOWSKI, 1968; STERBA and BRUCKNER, 1969; DELLMANN and OWSLEY, 1969; BOUDIER et al., 1970; KRSULOVIC and BRUCKNER, 1969; KRSULOVIC et al., 1970; HANSSON and NORSTROM, 1971; ERKOCAK, 1973). In these publications, however, cilia have not been mentioned. The possible role of pituicytes in the control of the release of neurohormones has been suggested in relation to the mitotic figures observed after dehydration (SELVE and HALL, 1943; DESCLIN, 1947; ORTMANN, 1951; LEVEQUE and SMALL, 1959; KRSULOVIC and BRUCKNER, 1969).

• A 39-year-old man died of acute nonlymphoblastic leukemia (ANLL). At age 35, he had undergone an operation for a frontal astrocytoma (grade 4), and received a 6,000-rad cranial irradiation, followed by intensive chemotherapy combining teniposide (VM-26) plus lomustine and 2,4-diamino-5-4'-dichlorophenyl-6-methylpyrimidine (DDMP). This new case of ANLL occurring as a second neoplasm in a patient previously treated for a malignant brain tumor is considered to be related to the antineoplastic therapy.

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Severe osteopetrosis was diagnosed in utero in two successive pregnancies resulting from an intermarriage. Hydrocephaly and skeletal hyperdensity were detected at 18 weeks of gestation, and fractures at 24 weeks. We report on extensive ultrasound, radiological, and pathological findings, including those on brain and bone. The markedly reduced number of osteoclasts observed in these sibs and the very early fetal involvement suggest that this form of osteopetrosis might represent a new entity: autosomal recessive lethal osteopetrosis.

In Alzheimer’s disease, and in a small group of other neurological disorders, the normal microtubules and neurofilaments are replaced or displaced by an accumulation of so-called“paired helical filaments” (PHF) which are parallel oriented structures, periodically twisted every 80 nm. In light microscopy, they form the so-called“tangles” in the neuronal perikarya which stain with silver methods and show a strong birefringence with a green dichroism after Congo Red staining. This property is similar to that of amyloid and has led some authors to the false conclusion that these tangles represent intracellular deposits of amyloid.

Changes in the hypothalamo pituitary tract and the pituicytes of the rat were studied after intrathecal and intraperitoneal injections of colchicine. Radioautography with 35S cysteine demonstrates that intrathecal colchicine prevents the migration of neurosecretory granules from the supraoptic and paraventricular nuclei to the posterior lobe of the pituitary. This results in accumulations of neurosecretory granules and in the formation in the axons of elongated structures resembling neurosecretory products, although they sometimes have a fibrillary content. Neurotubules appear to remain intact in these conditions. The stimulation of the posterior pituitary by dehydration, in particular after injection of the diuretic furosemide, leads to an increased activity of pituicytes. When colchicine is injected at the same time as furosemide, a considerable new formation of centrioles is observed in the pituicytes. These become associated with ciliary vesicles, and form numerous cilia of the 9 + 0 type. An increased number of centrioles is also seen in the endothelial cells of the posterior lobe of the pituitary. These apparently paradoxical results were briefly discussed in relation to the action of colchicine on neurotubules and axonal flow and to the limited data from the literature indicating a stimulation of cilia formation under the action of colchicine and other drugs., SCOPUS: NotDefined.j, FLWNA, info:eu-repo/semantics/published, The Biology of Cytoplasmic Microtubules

N, N-dimethyl-paraphenylenediamine (DMPPD) hydrochloride increases the inorganic and organic acid-soluble phosphate (Pi and POAS) uptakes in the innervated gastrocnemius muscle of the rat but not in the innervated soleus. In the denervated gastrocnemius muscle, the effects of DMPPD and denervation are not additive, Pi uptake being even lower than on the controlateral innervated side. It is suggested that DMPPD acts only on the innervated fast fibre (white fibre, type II anaerobic fibre), as far as the permeability to Pi is concerned. Histological evidence of a severe myopathic process affecting slow and fast fibres, irrespective of denervation has been documented. Severe degenerative changes were still manifest after a 20-day DMPPD treatment. There is no obvious relation between the biochemical alterations in phosphate metabolism and the morphological lesions. The increased Pi and POAS uptakes observed in the innervated gastrocnemius and soleus muscles during generalized convulsions induced by DMPPD are independent of a direct drug action on the muscle fibre.

A case of diffuse meningocerebral angiodysplasia is described in which type 4 adenovirus was isolated from the brain biopsy. The pathological features were similar to those previously described by various authors (1,3,4,5) and were not indicative of an infective process. Status epilepticus in the presence of extensive arteriovenous shunting in this dysgenetic cerebromeningeal vasculature is probably responsible for the extensive necrosis observed in the cortex and white matter.

A panel of monoclonal antibodies to neurofilaments have been investigated with regard to the location of their respective epitopes on neurofilament polypeptides and their ability to label the neurofibrillary tangles and paired helical filaments (PHF) which are characteristic of Alzheimer's disease. All of the neurofilament monoclonal antibodies that label tangles and PHF are directed against epitopes in the side arm domains of the two larger neurofilament polypeptides, NF-H and NF-M, and do not recognise the alpha-helical rod domains of these proteins. Immuno-electron microscopy demonstrates that the neurofilament antibodies label the constituent PHF per se and do not simply stain neurofilaments that might be admixed with PHF. These neurofilament epitopes are differentially retained by PHF, following isolation. Thus, antibody labelling of PHF is not simply due to the presence of normal neurofilament polypeptides. We propose that in tangle-bearing neurons, neurofilaments are degraded by proteases and that it is fragments of the side arms which contribute to the composition of PHF.

Flament-Durand Jacqueline. Le cerveau : morphologie et fonctions. In: Bulletin de la Classe des Beaux-Arts, tome 64, 1982. pp. 143-157.

An 18-year-old girl developed a reversible locked-in state with bilateral ptosis and almost complete ophthalmoplegia. She later presented with a relapsing and remitting course suggestive of multiple sclerosis. Autopsy findings demonstrated bilateral capsular and tegmental demyelinating lesions. In addition to this unusual aetiology, this is the first report with pathological evidence of a locked-in syndrome due to lesions outside the ventral brainstem.

Intrathecal administration of 20 mug of vincristine sulphate in the rat induced in vivo the formation of paracrystalline inclusions mainly in axonal processes. This is associated with an impairment in the migration of neurosecretory granules as shown by their accumulation in the perikarya of the magnocellular neurons. The granules are intermixed with numerous dense bodies of various shape, sometimes with a fibrillar content, and probably of lysosomal origin. In addition to the impairment of the flow of neurosecretory granules, there is also a striking accumulation of mitochondria and synaptic vesicles, and an apparent proliferation of the smooth endoplasmic reticulum. In the posterior lobe, the axonal endings contain a large number of neurosecretory granules, intermingled with bodies of varying shapes and electron density. Occasionally, a dense membrane surrounding a group of elementary granules is observed, reacting positively for acid phosphatase. This suggests an attempted crinophagia., SCOPUS: ar.j, info:eu-repo/semantics/published

Dementia of the Alzheimer type, or Alzheimer’s disease, has a characteristic brain histopathology comprising neurofibrillary tangles, senile neuritic plaques, granulovacuolar bodies and Hirano bodies. A correlation between the number of tangles, the extent of the cholinergic deficit and the degree of dementia has been found (Wilcock et al., 1982). Tangles are composed of bundles of 10–13 nm filaments which appear to be twisted about each other in pairs with a crossover roughly every 80 nm; these filaments are known as paired helical filaments (PHFs) (Kidd, 1963; Wisniewski et al., 1976). The tangles are present in, and appear to fill, affected neuronal perikarya of the hippocampus and other cortical and subcortical areas but the PHFs are also found as small aggregates in the swollen neurites present in the periphery of the senile plaques (Kidd, 1963, 1964; Terry et al., 1964; Gonatas et al., 1967).

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