Cases reported "Brain Diseases"

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1/38. kleine-levin syndrome and psychosis: observation from an unusual case.

    OBJECTIVE: This study evaluated the possible pathologic relation between kleine-levin syndrome (KLS) and mood disorders. BACKGROUND: A 28-year-old man with a remote history of KLS had the sudden onset of a manic episode with psychotic features after the end of hypersomnolence. METHOD: The patient received an extensive laboratory examination, including single photon emission computed tomography and magnetic resonance imaging. RESULTS: Single photon emission computed tomography showed decreased tracer perfusion in the basal ganglion, hypothalamus, and right frontotemporal region. magnetic resonance imaging revealed a cystic lesion in the pineal region. CONCLUSIONS: Hypothalamic dysfunction has been described in KLS and mood disorders, but pineal gland dysfunction has been mentioned only rarely. The clinical and neuroimaging findings suggest the need for further study of KLS.
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2/38. Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: clinicopathologic features and review of the literature.

    We present a unique case of biopsy-proven necrotizing sarcoidosis involving the central nervous system (CNS) in a 52-year-old woman. The patient presented with a 3-month history of left-sided headache and sharp, shooting pains on the left side of her face. She also has a previous history of sarcoidosis, histopathologically confirmed on parotid gland biopsy 24 years before. Imaging studies of the present lesion revealed a 1.8 x 1.4-cm mass in the left temporal lobe with signal intensity suggestive of meningioma or low-grade glial neoplasm. Surgical resection was initiated, and intraoperative consultation with frozen sections revealed granulomata. The lesion was biopsied, and surgical intervention was terminated. Permanent sections failed to reveal bacteria, mycobacteria, fungi, or foreign bodies. A diagnosis of necrotizing neurosarcoidosis was rendered. The patient was administered steroid therapy and clinically responded favorably. At the most recent follow-up almost 2 years later, there was no evidence of recurrence or progression. Necrotizing sarcoidosis has been reported most commonly in the lungs and rarely in other organ systems. We report the first histologically proven case involving the CNS as well as a rare example of sarcoidosis and necrotizing sarcoid granulomatosis in the same patient. sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of a granulomatous mass lesion involving the CNS, particularly in the context of a history of systemic disease.
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3/38. Glial cyst of the pineal gland: case report and considerations about surgical management.

    Symptomatic glial cyst of the pineal gland are rare lesions. Origin, natural history and factors leading to cyst enlargement are not completely clear; thus management remain uncertain in some cases. We report a case of symptomatic glial cyst and analyze the implication for surgery. Surgical management is indicated in patients presenting hydrocephalus, mass effect or symptoms related to mesencephalic dysfunction. The infratentorial supracerebellar approach represent the first choice for this condition allowing easy orientation with wide exposure of the tumor and good visibility of deep venous systems that may be preserved. Size of the tumor is a key element in evaluation of the treatment and the appropriate course for asymptomatic cyst less than 1 cm in size consist of conservative management. Periodic follow up is always indicated.
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4/38. amenorrhea-galactorrhea syndrome as an uncommon manifestation of isolated neurosarcoidosis.

    The involvement of the hypothalamic and/or the pituitary gland during granulomatous, infiltrative or autoimmune diseases is a rare condition of acquired hypothalamic dysfunction and non-tumoral-non-vascular hypopituitarism. sarcoidosis is a pathogen-free granulomatous disease which affects both central and peripheral nervous systems in 5-16% of patients. In most cases, nervous involvement by sarcoidosis occurs within a multi-systemic disease, although disease localization limited to the nervous system has also been reported. We observed a 26-year-old Italian woman presenting with low-grade fever, headache, visual disturbances, amenorrhea-galactorrhea syndrome and pituitary failure due to an infiltrative lesion involving the hypothalamus and the pituitary stalk. At first, the diagnosis of "probable" neurosarcoidosis was made according to the clinical picture, magnetic resonance imaging, and the study of cerebrospinal fluid lymphocyte sub-populations. In this case, hyperprolactinemic amenorrhea and galactorrhea combined with blunted responses of gonadotropins to luteinizing hormone releasing hormone and acquired growth hormone failure were to be considered as an infrequent manifestation of neurosarcoidosis limited to the medio-basal brain, without other disease localization. Forty-eight months after disease onset, the occurrence of mediastinal lymph node enlargement and increase of angiotensin converting enzyme in serum allowed us to confirm the diagnosis of sarcoidosis. Neurosarcoidosis is often hard to diagnose, especially when the neurological localization of the disease is not accompanied by other possible systemic signs, and when the lesion is too deep to obtain biopsy confirmation. The study of cerebrospinal fluid and blood lymphocyte sub-populations integrated by magnetic resonance imaging may be helpful for a correct diagnosis.
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5/38. Neuropathology of Raine syndrome.

    We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.
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6/38. Lead encephalopathy: CT and MR findings.

    Lead is toxic to many organ systems, among them bone marrow, muscles, kidneys, endocrine glands, joints, and nervous system. Encephalopathy is a rare but severe complication of lead poisoning. Lead toxicity is much less common in adults. adult lead poisoning results primarily from exposure by inhalation in the workplace. In this report, two cases of adult toxic encephalopathy due to lead poisoning are presented with CT and MR findings.
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7/38. Submandibular and intracranial hydatid cyst in an adolescent.

    OBJECTIVES: An unusual case of hydatid disease is reported. review of the pertinent literature did not reveal any hydatid disease located simultaneously in both the intracranial and submandibular glands. This is the first case with hydatid disease occurring in both locations at the same time. STUDY DESIGN: The case of an 18-year-old is presented; the symptoms, findings, methods of diagnosis, and our approach for treatment are discussed; and the literature is reviewed. RESULTS: The intracranial lesion was completely excised by left-sided frontoparietal craniotomy, and the mass in the right side of the submandibular gland was removed through a submandibular approach at the same session. The intact cyst was completely excised. Histological examination of both lesions confirmed the diagnosis of hydatid cyst by. Postoperative recovery was uneventful, and the patient was discharged on the seventh day. CONCLUSIONS: Hydatid cyst should be suspected during the evaluation of cervical masses, particularly in endemic regions. Hydatid disease infestations are best treated with complete excision of the intact cyst.
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8/38. Postirradiation necrosis of the temporal lobe presenting as a glioma. Case report.

    A patient is reported who presented with manifestations of a space-occupying lesion in the left temporal lobe thought to be a metastasis, but on radiological examination and surgical exploration appeared to be a diffuse infiltrative glioma. Some 21 months earlier he had received a course of fast neutron therapy to a carcinoma of the left parotid gland. diagnosis by microscopic examination revealed changes characteristic of delayed radiation necrosis.
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9/38. Wegener's granulomatosis presenting with otologic and neurologic symptoms: clinical and pathological correlations.

    A case of Wegener's granulomatosis in a 59-year-old woman is reported. The disease first involved the parotid gland, the brain stem and the spinal cord, then running a rapidly progressive course as systemic pathology. Anti-neutrophil cytoplasmic antibody (cANCA) levels raised as renal failure set in. Renal biopsy confirmed the diagnosis of Wegener's granulomatosis and an appropriate therapy was adopted.
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10/38. Successful treatment of multiple sclerosis with magnetic fields.

    The present communication concerns a 50 year-old woman with a 15 year history of chronic-progressive multiple sclerosis (MS) in whom extracranial application of picoTesla magnetic fields (MF) produced a dramatic and sustained improvement in disability. In contrast, administration of melatonin (3 mg, P.O.) produced in this patient a rapid exacerbation of disability which was reversed subsequently by treatment with MF. It is hypothesized that the therapeutic effects of picoTesla MF involve the mediation of the pineal gland which is known to act as a magnetosensor. The report demonstrates, for the first time, the remarkable efficacy of weak MF in the symptomatic treatment of chronic-progressive MS and underscores the pivotal role of the pineal gland in the pathophysiology of MS. If confirmed by a larger cohort of patients, extracranial application of picoTesla MF may prove as an extremely efficacious, nonpharmacological modality for the treatment of MS.
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