Cases reported "Gliosis"

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1/15. Craniofacial surgery for giant frontonasal encephalocele in a neonate.

    A 5-day-old neonate with a frontonasal encephalocele is reported. He was referred to our institute with a swelling on the glabella not associated with cerebrospinal fluid (CSF) leak. The baby was the first born of a non-consanguinous marriage. The baby had a swelling over the glabella, 7 cm in diameter. The swelling had healthy covering with a raw area at the centre without any CSF leak. A magnetic resonance imaging scan showed a soft tissue swelling containing tissue iso-intense to normal brain. The internal bony defect was at the junction of the frontal and ethmoid bones, in front of the crista galii, in the floor of the anterior cranial fossa. The baby was operated on the 11th day after birth. A one-stage repair of encephalocele was performed, along with correction of hypertelorism and reconstruction of the nasal bridge. The postoperative period was unremarkable. The baby was discharged from hospital on the 10th postoperative day.
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2/15. Rasmussen encephalitis associated with Parry-Romberg syndrome.

    Parry-Romberg syndrome is a rare disorder associated with unilateral facial atrophy involving skin, subcutaneous tissue, skeletal muscle, and bone. Occasionally, there is CNS involvement with epilepsy being the most common CNS manifestation. The authors report a child with Parry-Romberg syndrome with a course strongly suggestive of Rasmussen encephalitis. The boy underwent hemispherectomy, and pathology showed the typical findings of Rasmussen encephalitis, suggesting that these two conditions may share common etiologic factors.
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keywords = bone
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3/15. MM2-thalamic-type sporadic Creutzfeldt-Jakob disease with widespread neocortical pathology.

    We report an autopsy case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) with widespread cerebral neocortical pathology. Initial symptoms were progressive insomnia and mental disturbance. magnetic resonance imaging revealed no high-signal intensity lesions on diffusion-weighted images and later showed gradually progressive cerebral atrophy. Periodic synchronous discharges and myoclonus were not observed. Upon neuropathologic examination, widespread cerebral neocortical involvement with fine vacuole-type spongiform change was observed. Severe degeneration with almost complete neuronal loss, tissue rarefaction, numerous fat-laden macrophages and hypertrophic astrocytosis of the medial thalamic nucleus was evident. The inferior olivary nucleus showed severe involvement with neuronal loss and hypertrophic astrocytosis. In the cerebellar cortex, moderate depletion of Purkinje neurons was evident, with no spongiform change in the molecular layer and no neuronal loss in the granule cell layer. immunohistochemistry for prion protein (PrP) revealed widespread synaptic-type deposits with some primitive plaque-type deposits in the cerebral neocortex, basal ganglia and cerebellar cortex. PrP deposition was also observed in the brainstem, particularly the tegmentum, substantia nigra and pontine nucleus, and spinal cord, particularly the posterior horn. In the medial thalamus and inferior olivary nucleus, PrP deposition was sparse. Analysis of the PrP gene showed no mutation but did show methionine homozygosity at polymorphic codon 129. Western blot analysis of protease-resistant PrP indicated the presence of type 2 PrP. We believe that this patient suffered from MM2-thalamic-type sCJD (sporadic fatal insomnia) with widespread cerebral neocortical pathology due to prolonged disease duration. The present case showed different patterns of spongiform degeneration and PrP deposition in the cerebral neocortex than those in previously reported MM2-thalamic-type sCJD cases.
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ranking = 9655.8918957814
keywords = macrophage
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4/15. Degeneration of the substantia nigra in familial amyotrophic lateral sclerosis.

    We have studied a large family with familial amyotrophic lateral sclerosis (FALS) and examined the brain and spinal cord of a 40-year-old male family member at autopsy three years after clinical onset of the disease. The most unusual finding was a severe degree of neuronal loss of the substantia nigra accompanied by gliosis and numerous pigment-laden macrophages. There was marked degeneration of the upper and lower motor neurons and the corticospinal tracts. In addition, there was an unusually severe degree of diffuse degeneration throughout the anterolateral columns of the spinal cord. The posterior columns and Clarke's nucleus were not involved. Nigral degeneration has rarely been reported in FALS. The findings in this case emphasize the great variability of morphologic changes encountered in FALS and raise the question of a relationship between FALS and extrapyramidal disorders such as Parkinson's disease. We conclude that careful postmortem examinations of further cases of FALS are needed to fully define the extent of degenerative changes in this disease.
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ranking = 9655.8918957814
keywords = macrophage
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5/15. Acute parkinsonian syndrome with demyelinating leukoencephalopathy in bone marrow transplant recipients.

    A syndrome of rigidity, bradykinesia, spasticity, and often myoclonus and dementia developed acutely in 5 patients who had undergone successful engraftment of bone marrow transplants for the treatment of various hematologic diseases. magnetic resonance imaging demonstrated widespread changes in white matter; brain biopsy disclosed mild demyelination associated with active phagocytosis of myelin. One patient, who was not treated, remains severely demented. patients treated with very high-dose methylprednisolone had complete clinical recovery.
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keywords = bone
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6/15. Disturbances in neuronal migration and laminar cortical organization associated with multicystic encephalopathy in the Pena-Shokeir syndrome.

    The Pena-Shokeir syndrome is characterized by intrauterine growth retardation, camptodactyly, multiple ankyloses, facial anomalies and pulmonary hypoplasia. The condition is thought to be inherited in an autosomal recessive fashion. A detailed neuropathological analysis of the brain of a stillborn full-term male infant who exhibited the gross features of the Pena-Shokeir syndrome revealed diffuse bilateral cerebral polymicrogyria associated with multicystic encephalopathy. Abnormal brain development, which was characterized by disturbances in neuronal migration and laminar cortical organization, was clearly associated with changes of an encephaloclastic nature, namely reactive gliosis and infiltration by macrophages. These findings suggest strongly that the Pena-Shokeir syndrome may also result from teratogenic factors such as intrauterine ischemic and/or hypoxic insults to the developing brain.
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ranking = 9655.8918957814
keywords = macrophage
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7/15. Histopathology of Endolymphatic subarachnoid shunt surgery for Meniere's disease.

    The histopathological findings in the temporal bones of three patients who underwent endolymphatic subarachnoid shunt operation for Meniere's disease are reported. The postoperative follow-up period varied from three to five years. Two patients had a successful operation; the third was unsuccessful. The continuity between th mesothelial lining of the endolymphatic sac and the subarachnoid space was demonstrated in one case; no definite conclusions could be made for the other two cases. Although failure of shunt surgery was presumably due to fibrosis in the shunt area in the reported case, the human endolymphatic sac seemed to be more resistant to surgical trauma than the sac of the experimental animals.
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keywords = bone
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8/15. Hereditary diffuse leucoencephalopathy with spheroids.

    The clinical, genetic, and morphological features of a previously unknown progressive neuropsychiatric disease are presented. By genealogical investigation of the background of an uncharacteristic case of presumed organic psychosis, we traced 71 relatives from four generations. The anamnestic data showed various combinations of psychiatric symptoms (depression, anxiety, aggressiveness, and severe dementia), neurological symptoms (impaired balance with retropulsion, hyperkinesia, and epilepsy), and somatic symptoms (gastrointestinal disorders, arthritis, and gynaecological problems) in 17 (11 dead and 6 living) members of the family. Age at onset varied between 8 and 60 years. Some patients rapidly developed severe dementia and died a few months after the onset of symptoms, while in others the course was prolonged with dementia over several decades. The genetic interpretation indicated an autosomal dominant inheritance with possible full penetrance but widely variable expressivity. Morphological studies were performed on the central nervous system of four decreased family members (three siblings and their maternal uncle). The same type of widespread leucoencephalopathy was seen in the four autopsy cases. It was characterised by degeneration and loss of myelin sheaths and axons, occurrence of numerous neuroaxonal spheroids in the affected white matter, accumulation of lipid-laden macrophages, and gliosis. The bilateral, frontal, fronto-parietal, and temporal locations of the most pronounced, diffusely demarcated lesions corresponded fairly well to the symptoms of an organic psychosyndrome with its main substrate in the forebrain. For this new clinico-pathological entity, the name "hereditary diffuse leucoencephalopathy with spheroids" (HDLS) is proposed. As a working hypothesis, it is suggested that not only genetic, but also immunological and possible endocrine factors may contribute to the development of the disease.
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ranking = 9655.8918957814
keywords = macrophage
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9/15. Sclerosing leucoencephalopathy with membranocystic lesions of adipose tissue: a contribution to the pathology of Nasu disease.

    A case of sclerosing leucoencephalopathy with membranocystic lesions of adipose tissue was reported. A 38-year-old Japanese housewife, whose parents were consanguineous, developed gradually neuropsychiatric symptoms characterized by euphoria, gait disturbance and urinary incontinence, followed by spastic tetraplegia with epileptic convulsions and died eight years later. Neuropathologically a form of sudanophilic leucodystrophy with a prominent fibrillary gliosis (dissociation glio-myelinique) associated with axonal spheroids and calcospherite depositions was revealed. A peculiar membranocystic lesion of adipose tissue was recognized not only in the bone marrow but also in other areas. However, no apparent bone pathology was observed except for a ring-like pleated lamellar structure. This case was thought to be classified as heterogeneous phenotype of membranous lipodystrophy (Nasu).
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keywords = bone
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10/15. Nasu-Hakola's disease (membranous lipodystrophy). A case report.

    An autopsy case of Nasu-Hakola's disease (membranous lipodystrophy) was reported. A 29-year-old Japanese woman whose younger sister had been affected with typical Nasu-Hakola's disease with skeletal and neuropsychiatric syndromes and membrano-cystic lesions in the bones developed forgetfulness and lack of initiative. The clinical features were characterized by diminished drive, apathy, euphoria, disturbance of attention, amnestic syndrome, and gait disturbance. The clinical course of her illness was 8 years. The neuropathologic examination revealed marked symmetrical gliosis of the cerebral white matter (sclerosing leukodystrophy) predominantly in the frontal and temporal lobes with slight or moderate demyelination (dissociation glio-myelinique) and widespread axonal changes such as fragmentation and spheroid in the white matter of the cerebral hemisphere, cerebellum, basal ganglia, and brain stem. The ultrastructure of spheroids showed neurofilamentous accumulation. We discussed the importance of axonal changes with regard to the pathogenesis and etiogenesis of the disease.
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keywords = bone
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