Biomedical subjects
Publications and source records attributed to J Pialat.
Diagnosis of an intracerebral mass requires a good collaboration between clinicians and pathologists. Different techniques may be necessary. The interest of cytological preparations and immunohistochemistry is reported. The main characteristic features of cerebral tumors are described and the differential diagnosis with reactive and inflammatory masses simulating neoplasia is discussed.
We report a new case of giant cell angiitis of the central nervous system associated with cerebral amyloid angiopathy (GA/CAA). A 67-year-old woman was hospitalized with a history of headaches and lapses of consciousness. After improvement with corticosteroidtherpay, treatment was stopped. She relapsed and died 33 days after first admission. Pathological examination showed unusual extension of GA/CAA lesions, in the superficial and deep layer of the cerebral cortex, and in the cerebellum. Simultaneous occurrence of GA and CAA is rare. Histopathologic findings and immunological pathogenesis of the process are discussed: 1) arguments over pre-existence of CAA, responsible for GA; 2) primitive inflammatory process inducing amyloid deposits; 3) GA/CAA may represent an association of histological lesions related to 2 different types of disease: i) neurodegenerative disease with specific lesions (such as presence of diffuse senile plaques and neurofibrillary tangles) inducing inflammatory reaction ii) inflammatory disease, with few or no degenerative lesions, responding to immunotherapy.
Primary leptomeningeal gliomatosis is rare, and the diffuse form (PLDG) is even more unusual. The following report is an example. A 17 year-old man developed a syndrome characterized by extensive basal and chronic spinal meningitis. Routine biological tests showed elevated levels of CSF proteins, and moderate mononuclear pleocytosis, with no direct evidence of neoplasia, leading to a diagnosis of chronic meningitis. A second meningeal biopsy, guided by MRI and performed in the left frontal region, led to the specific diagnosis of primary diffuse leptomeningeal gliomatosis. Treatment including ventricular and lumbar shunting, a course of cortico-spinal radiation, and three courses of an eight-drug systemic chemotherapy with intrathecal methotrexate lead to complete remission over 15 months. We believe that this is the first report of such a remission in the literature.
Numerous routes of access to the medial basal structures of the cranium have been described, largely because of the wide variety of lesions observed in deep localizations. Access can be achieve via trans-sinusal, transfacial (trans-sphenoidal rhinoseptal, mediofacial or Lefort I), trans-oro-pharyngeal and numerous other routes. An examination of the principals involved, their development and the technical modalities demonstrate the advantages and disadvantages of each and their specific indications. Access is particularly interesting with the frontal trans-sphenoid, Lefort I osteotomy and trans-oro-pharyngeal routes. The simplicity of these non-mutilating routes provide an alternative to neurosurgical access. Their development depends on progress in imaging and microscopic surgery. Used alone or in combination, they can be an useful complement to a neurosurgical access.
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We have studied 20 pineal parenchymal tumors (PPT) and 4 normal or cystic pineal glands both by light and electron microscopy and immunohistochemistry with antibodies against glial markers [glial fibrillary acidic protein (GFAP) and protein S-100] or neural/neuroendocrine markers [neurofilaments (NF), synaptophysin and chromogranin A]. Light microscopy revealed the cellular organization of pinealocytes in the normal gland and in different morphological types of pineal tumors (typical pineocytomas, PPT with intermediate differentiation, mixed PPT exhibiting elements of both pineocytoma and pineoblastoma and pineoblastomas). Immunohistochemistry showed the presence of GFAP and protein S-100 in interstitial cells in non-neoplastic pineal gland. Cell processes were labeled with anti-synaptophysin and anti-NF antibodies. No immunoreactivity was found for chromogranin A in non-neoplastic pineal gland. In pineocytomas, GFAP and protein S-100 were observed in interstitial cells. Synaptophysin and NF were present in the large rosettes of pineocytomas. Synaptophysin, NF and chromogranin A were present in pineocytomas with a lobular arrangement of cells. Anti-chromogranin A immunoreactivity was also seen in lobular areas of some PPT with intermediate differentiation. Analysis of normal human pineal gland by electron microscopy showed the presence of vesicle-crowned rodlets (VCR or synaptic ribbons), fibrous filaments (F), paired twisted filaments but few dense-core vesicles (DCV) in normal pinealocytes. Tumoral pineal cells appeared to differentiate either towards a neurosensory pathway characterized by the presence of sensory cells elements (VCR and F), or towards a neuroendocrine pathway, with the occurrence of many DCV. Immunogold labeling demonstrated the presence of chromogranin A in neurosecretory granules.
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The P.O.E.M.S. syndrome is a rare clinical entity that has been described mainly in Japan. It is characterized by a progressive polyneuropathy with raised CSF protein content, organomegaly, endocrinopathy, skin changes and plasma cell dyscrasia. We report a new documented case associated with a solitary plasmocytoma of the 12th thoracic vertebra. Immunopathological and ultrastructural studies of the peripheral nerve did not disclose any immune-specific changes. Surgery and radiation therapy of the plasmocytoma allowed a complete recovery, with a 5-year follow-up. This case report illustrates the need for serial full skeletal survey, including scintigraphy, in middle-aged patients with progressive polyneuropathy of obscure origin.
Three patients with adult-onset neuronal-ceroid lipofuscinosis (Kufs' disease) are presented. Two cases were familial (autosomal recessive inheritance) and 1 case was possibly sporadic. The main clinical feature was progressive deterioration of cognitive functions. In 2 siblings, aged 37 and 41, dementia was associated with personality and behaviour changes, suggesting a psychotic disorder and with dysarthria and tic-like dyskinetic movements. In a third patient, dementia was only associated with an asymptomatic pigmentary retinal degeneration. CT scan revealed diffuse cerebral atrophy in all cases. Diagnosis was established by brain biopsy in 2 cases. Autofluorescence emission and absorption spectra from the abnormal pigment was studied and was not found contributive.
The phenotypic characteristics of 7 subependymal giant cell astrocytomas (GSECG) (6 of these being associated with tuberous sclerosis) are studied using morphological and immunohistochemical methods with antiserums against vimentine, glial fibrillary acid protein (GFA), S100 protein, and neurofilaments. The glycoproteic secretion of the tumor cells was also analyzed after exposure to Concanavalin A (CON A) by a direct fluorescent method. Our results suggest that some GSECG originate from specialized ependyma (circum-ventricular organs). They have the same location (foramen of Monro), present some common ultrastructural features (cytoplasm with lumen containing cilia), are positive with certain immunohistochemical markers (staining with S100 protein in 4 cases, with vimentin in 3 cases) and show a strong glycoproteic secretion (positive with CON A). Therefore, some GSECG might be considered hamartomas of specialized ependyma, with a reduced evolutivity potential.
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Choroid plexectomy was performed for chronic infected hydrocephalus in 17 children via a direct open approach. In 16 cases, the CSF was sterilized soon after the plexectomy. In 37% of cases, the hydrocephalus was arrested without a shunt. The incidence of seizures did not increase after plexectomy. Removal of the choroid plexus was controlled by scintigraphy. Neuropsychological results were not encouraging, probably related to the long history of chronic ventricular infection. Surgical mortality was 6%. Choroid plexectomy should be considered as a possible treatment of chronic infected hydrocephalus in children.
The authors report the case of a 33 year-old male with urinary disturbances referred for removal of a spinal intradural mass associated with a L4 spina bifida occulta. At operation three types of lesions were present: a tethered cord, an intradural lipoma of the cauda equina and conus medullaris and an intramedullary mature teratoma. A total removal of teratoma and lipoma and a section of the filum terminale achieved a normalization of urological function. Such mature teratoma that consists of all three germ layers only represent 2% of all intramedullary tumors. Pathogenesis is dysembryoplastic but still remains unclear: germinal cells might have been displaced into the dorsal midline during their normal migration from yolk sac to gonadal ridges. Their association with other dysgenetic lesions such as lipoma or spina bifida is not rare but the links between these different pathologies remain unknown.
A series of 9 patients having a tuberous sclerosis associated to a midline ventricular tumour is reported. Microscopically, the presence of giant cells within the lesion is a major characteristic of the disease. The origin of these subependymal giant cells tumours is questionable since astrocytic, neuronal and ependymal features have been noted by several authors providing various denominations. In the literature and in our series as well, the intra-ventricular tumour presented as the initial manifestation of the disease in most of cases, usually with increased intra-cranial pressure symptoms. On CT, the tumour arises in the area of the foramen of Monro and enhances after contrast injection while the other intracranial anomalies of the disease do not enhance. In 8 patients, a direct transcortical transventricular approach was used. 1 patient was treated by shunt only. The results were evaluated according to the degree of the preoperative neuropsychological impairement: there were 3 deaths, 3 "excellent", 2 "fair" and 1 "poor" results. The problem of the surgical indication raises mainly in patients in whom the diagnosis of the tuberous sclerosis is ascertained prior to the diagnosis of the tumour. Since acute C.S.F. blockage or intra-ventricular bleeding may occur during "conservative" treatment, direct approach seems preferable.
The authors describe the case of a Marfan syndrome who presented a giant intracranial internal carotid aneurysm associated with elongation and tortuosity of internal carotid and vertebral arteries on both sides. The skin microscopic examination showed fragmentation and distorsion of elastic fibers. There was no microscopic study of the vessels. Extra and intracranial vascular abnormalities are rarely reported in marfan syndrome: cardiovascular changes are seen mostly in aorta and pulmonary artery. In the literature are reported some giant aneurysms, dissections and dilatations of carotid, basilar and vertebral arteries. Usually the microscopic examination of the vessels show cystic medial necrosis. Other connective tissue diseases (pseudo-xanthoma elasticum, Ehlers-Danlos syndrome, progeria) are described with such clinical abnormalities. Reference is made to the possibility of unknown histological and chemical lesions weakening the vessels in patients without connective tissue disorders and presenting with arterial dissection or arterial aneurysm. So vessels would be more sensible to aging, arteriosclerosis or hypertension.
A 64 year old patient with a cardiac prosthesis and receiving antivitamin K therapy presented with a right bulbar syndrome. Based on absence of meningeal signs and hemorrhage on CT scan heparin therapy was instituted. Fatal coma developed two and a half days later. Autopsy findings included a right lateral bulbar infarct and meningeal hemorrhage, predominant around a fusiform dilatation of the end of the right vertebral artery. Serial sections showed a large dissecting aneurysm between media and adventitia communicating with an intimal tear. Among intracranial dissecting aneurysms those of vertebrobasilar localization have the peculiarity of being subadventitial in some cases, with a subsequent enhanced risk of hemorrhagic complications. The use of anticoagulants appears contraindicated in these cases, even if their clinical expression is suggestive of an infarct. Their etiology is often unknown.
The authors report a recent personal case of trigeminal neurinoma with a topographical extension both in the cerebello pontine angle and the middle cerebral fossa. This 33 year-old female suffered from progressive sensory disturbances of her right hemiface associated with a right fifth nerve motor deficit, a right VI nerve palsy and a tinnitus. CT scan and angiogram were evocative of a right hourglass trigeminal neurinoma. Two successive operative stage through suboccipital route and a pteriono-temporal extra and intradural approach allowed a complete removal of the tumour. A post-operative rhinorrhea dried up with 10 days. The patient complained with a right hemiface anesthesia and a palsy of the masseter muscles; the VI nerve palsy recovered within 3 months. From the review of the literature (118 cases) the authors summarize the anatomical, clinical and radiological features of these tumours and point out. The difficulty of their surgical removal that was only complete in half of cases. The high frequency of hourglass neurinomas explains that a single suboccipital or subtemporal approach--even with opening of the tentorium--only allowed 23 complete removal among the 58 published or quoted interventions. This justifies that in a majority of cases a combined approach must be preferred, using successively a suboccipital and an intradural subtemporal route, the latted giving access to the cavernous sinus in case of its invasion.