1/26. 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.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/26. Intrasellar neuronal hamartoma associated with pituitary adenoma. Case report.46-year-old acromegalic women presenting high level of growth hormone (32 ng/ml) in the serum underwent surgery. The intrasellar tumor, 16 mm in diameter, has been removed. The biopsy material consisted of two types of closely adjacent and intermingled tissues, one of which was growth hormone positive acidophilic adenoma, the second component were haphazardly oriented ganglion cells of various size and shape, also multinuclear, with bundles of unmyelinated fibers. The cytoplasm and processes of ganglion cells were immunopositive for neurofilaments and for synaptophysin on cellular membranes and processes. There were none glial fibrillary acidic protein positive cellular elements. The authors discuss commonly used name of choristoma for this type of tumor and the problem of possible neurosecretory stimulation of pituitary adenoma by neuronal hamartoma.- - - - - - - - - - ranking = 2keywords = ganglion (Clic here for more details about this article) |
3/26. Involuntary hand levitation associated with parietal damage: another alien hand syndrome.The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
4/26. Menkes' kinky hair disease: a light and electron microscopic study of the eye.light and electron microscopic studies of the ocular tissue of a case of Menkes' kinky hair disease are described. The copper deficiency responsible for this systemic and neurologic disease appears to cause a progressive degeneration of retinal ganglion cells, loss of nerve fibers, and optic atrophy. The pigment epithelium is also abnormal with only small and irregular melanin granules present among electron-dense inclusion bodies. Abnormal elastica is present in Bruch's membrane.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
5/26. Fatal cerebro-renal oxalosis after appendectomy.A case of a 24-year-old male with fatal cerebro-renal oxalosis assumed to be due to infusions of the sugar surrogate xylitol after appendectomy is reported. The diagnosis was established only after intensive histological investigations following the autopsy. The clinical picture was characterized by an acute seizure, coma and renal failure 2 days after the first xylitol infusion. death occurred due to cerebral dysregulation as a very rare complication after parenteral administration of xylitol. Subendothelial double refractive calcium oxalate crystals were found in the walls of cerebral blood vessels, in particular in the stem ganglion regions and in the cortical renal tubules. The most common type of primary oxalosis was excluded by sequencing analysis. The young age, the minor surgical intervention and the otherwise unremarkable history are special features of this case. Since the genetic background of xylitol intolerance is still unclear, it is suggested that it should be banned as a sugar surrogate in clinical practice.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
6/26. A case of Hashimoto's encephalopathy: association with sensory ganglionopathy.We report a case of Hashimoto's encephalopathy with prominent unsteadiness of gait. Nerve conduction studies and electromyographic findings were consistent with a sensory ganglionopathy. Symptoms and clinical findings resolved with high-dose corticosteroid therapy and paralleled the levels of anti-microsomal antibodies. Sensory ganglionopathy seems to be another aspect of the broad clinical syndrome labeled Hashimoto's encephalopathy.- - - - - - - - - - ranking = 6keywords = ganglion (Clic here for more details about this article) |
7/26. Cortical-basal ganglionic degeneration.We report our experience with 15 patients believed to have cortical-basal ganglionic degeneration. The clinical picture is distinctive, comprising features referable to both cortical and basal ganglionic dysfunction. Characteristic manifestations include cortical sensory loss, focal reflex myoclonus, "alien limb" phenomena, apraxia, rigidity and akinesia, a postural-action tremor, limb dystonia, hyperreflexia, and postural instability. The asymmetry of symptoms and signs is often striking. brain imaging may demonstrate greater abnormalities contralateral to the more affected side. Postmortem studies in 2 patients revealed the characteristic pathologic features of swollen, poorly staining (achromatic) neurons and degeneration of cerebral cortex and substantia nigra. Biochemical analysis of 1 brain showed a severe, diffuse loss of dopamine in the striatum. This condition is more frequent than previously believed, and the diagnosis can be predicted during life on the basis of clinical findings. However, as with other "degenerative" diseases of the nervous system, a definitive diagnosis of cortical-basal ganglionic degeneration requires confirmation by autopsy.- - - - - - - - - - ranking = 7keywords = ganglion (Clic here for more details about this article) |
8/26. Autosomal dominant hypoparathyroidism with intracranial calcification outside the basal ganglia.We describe a family with autosomal dominant hypoparathyroidism. The 3 affected individuals had no detectable serum parathyroid hormone on radioimmunoassay. The propositus presented with seizures and on CT scan had bilateral basal ganglion calcification and calcification in the frontal lobes. His similarly affected mother had even more intracerebral calcification. The latter manifestation has not been described previously in autosomal dominant hypoparathyroidism.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
9/26. Tumors of the neuron series presenting as cysts with mural nodules.Two cases of tumors of the neuron series presenting as cysts with mural tumor nodules are described. This radiographic and surgical aspect may be observed in astrocytomas, meningiomas or ependymomas, whereas it is more rare for neuroblastomas and ganglioneuromas. There are not CT features which allow to differentiate cystic tumors of the neuron series from the other cystic tumors. The finding of cyst with enhancing mural tumor nodule and unenhanced wall is more favorable from a surgical point of view; the complete removal of the nodule with preservation of the reactive cyst wall results in the cure of patients with ganglioneuroma and ganglioglioma and in good long-term survival in those with cerebral neuroblastomas.- - - - - - - - - - ranking = 2keywords = ganglion (Clic here for more details about this article) |
10/26. The neurological features of acute hiv infection.This paper describes the few case reports of neurological effects of acute (primary) hiv infection. Following a typical primary illness (fever, sore throat, headache, rash, lymphadenopathy, superficial oral ulcers, conjunctivitis, leukopenia and thrombocytopenia) aseptic meningitis, myelopathy, spinal myoclonus, peripheral or cranial neuropathy, neuralgia and ganglioneuronitis may occur, usually within 3 weeks. Encephalopathy with spontaneous recovery also occurs, usually without other features of acute hiv infection. diagnosis depends on demonstration of seroconversion which may be delayed by weeks. No therapy is yet available.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
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