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Most doctors have vague and imprecise notions of what is meant by the terms olivopontocerebellar atrophy, Shy-Drager syndrome, striatonigral degeneration, multiple system atrophy and multisystem degenerations. This article attempts to tame this nosological jungle.
Five cases of petrous apex epidermoid cysts, six cases of petrous apex cholesterol granuloma cysts, and seven cases of intradural cerebellopontine angle (CPA) or intracranial epidermoid cysts are reviewed. Petrous epidermoid cysts (cholesteatomas) and cholesterol granuloma cysts cannot be differentiated definitely from each other on CT scans. On MR imaging, epidermoid cysts exhibit long T1 and long T2 characteristics and can be differentiated from cholesterol granuloma cysts, which exhibit short T1 and long T2 characteristics. Intradural epidermoid cysts involving the CPA or other portions of the brain, also demonstrate long T1 and long T2 characteristics on MR scans. Intradural epidermoid cysts exhibit curvilinear areas of higher intensity on T1-weighted images, which were best evaluated on T1-weighted MR images obtained with more averages (6 to 8).
Some of the clinical hallmarks of lateral medullary infarction--Wallenberg's syndrome--are saccadic dysmetria, smooth pursuit deficit, and lateropulsion of the body. Similar movement disorders are seen in monkeys after local unilateral injection of GABAergic drugs in the caudal fastigial nucleus of monkeys. These include an ipsilateral saccadic hypermetria and a contralateral saccadic hypometria as well as cogwheel smooth pursuit eye movements toward the contralateral side and an ipsiversive lateropulsion of the body. It was previously suggested that the lateral medullary infarction causes a lesion of climbing fibers to the cerebellum. This lack of climbing fiber input increases the activity of ipsilateral Purkinje cells, which consequently provide too much inhibition of the deep cerebellar nuclei.
PURPOSE: To evaluate MR patterns in ophthalmoplegia plus and correlate them with clinical symptoms. METHODS: MR was performed on a 1.5-T whole-body scanner with T2-weighted gradient-echo and spin-echo images. The retrospective analysis included 19 patients with clinically established diagnoses of ophthalmoplegia plus. RESULTS: Two types of cerebral MR abnormalities were found in ophthalmoplegia plus: brain atrophy and hyperintensities restricted to the white matter and basal ganglia, which appeared as either focal or diffuse areas of high signal intensity and were of strictly supratentorial location. No specific distribution was found. These findings differ markedly from infarction-like lesions found in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. CONCLUSIONS: MR is sensitive for the detection of central nervous system involvement in ophthalmoplegia plus, but findings are nonspecific. However, cerebral MR in ophthalmoplegia plus is different from other mitochondrial encephalomyopathies and underlines the clinical differentiation of mitochondrial encephalomyopathies.
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Explore the source record for details and available documents.
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Acute encephalopathy attributable to 5-fluorouracil (5-FU) is rare. A patient experienced this reaction associated with a continuous 5-FU infusion. The etiology of the event remains uncertain, but it is generally reversible and does not preclude retreatment with 5-FU at reduced dosages. Steroids and thiamine may expedite neurologic recovery.
In a case of pediatric Whipple disease confined to the central nervous system, white matter lesions initially appeared as areas of very low signal intensity on T1-weighted MR images and as areas of hyperintensity on proton density-weighted and T2-weighted images, and showed slight peripheral enhancement on delayed contrast-enhanced T1-weighted images. On MR studies obtained 3 and 6 months after antibiotic therapy, the lesions had decreased in size and no longer enhanced. They became progressively less hypointense on T1-weighted images and less hyperintense on T2-weighted images.
The objective of the present study was to evaluate the safety and efficacy of gadodiamide injection, a non ionic MRI contrast medium in comparison with the ionic agent gadopentate dimeglumine. Two groups of 50 patients with known or suspected lesions of the brain or head and neck were enrolled in a double -blind, randomised trial. In the gadopentate dimeglumine group three patients reported four adverse events, and in the gadodiamide injection group, four patients reported four side effects. All events were minor. Two radiologists analyzed pre and post-contrast MR images. The parameters evaluated were the number of lesions, delineation of the lesion, gain of diagnostic information, and final diagnosis. Both contrast media gave identical diagnostic information.
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The authors report a case of a primary extraskeletal osteosarcoma arising within an epidermoid cyst in the parenchyma of the cerebellum in a 64-year-old woman. On initial presentation, the tumor involved the midline cerebellum without attachment to the surrounding dura mater or calvarium. Complete medical and radiologic evaluation failed to reveal a primary skeletal or other extraskeletal osteosarcoma. To our knowledge, this is the first reported case of a primary extraskeletal osteosarcoma within the cerebellum. Osteosarcoma as a primary brain tumor is exceedingly rare, and only three cases (all occurring within the cerebral hemispheres) have been reported previously. The histogenesis of primary sarcomas of the brain is not evident. The associated finding of an epidermoid cyst suggests the tumor originated from a teratoma.
Dizziness and imbalance are uncommon in children, but often alarming for their families. A detailed interview and otoneurological examination are important for reaching a specific diagnosis and treatment. The purpose of our study was to obtain our own etiological, clinical and diagnostic statistics, and to review the major related pathologies. We report a series of 76 children with imbalance or dizziness as the presenting symptom. Paroxysmal benign vertigo of childhood was the most common pathology in our study, followed by vestibular neuronitis. Our findings were compared to those of other authors. Early diagnosis is emphasized as the most important factor for achieving an effective vestibular rehabilitation in children.
We report the imaging findings in five patients with a unique dysmyelinating disorder. MR studies of these infants showed obstructive hydrocephalus caused by mass effect produced by an enlarged cerebellum. The white matter of an enlarged cerebrum and cerebellum showed delayed myelination. Proton spectroscopy showed normal N-acetylaspartate (NAA) levels. While the dysmyelinating disorder was clearly differentiated from Canavan disease by an absence of elevated NAA and differing histopathologic findings and autosomal-dominant inheritance pattern, there were similarities to this disease in the presentation and, to some extent, in the initial imaging findings.
Dementia is a clinical syndrome and is diagnosed on clinical grounds. Various types can be distinguished: the Alzheimer-type, frontal lobe dementia and subcortical dementia syndromes. Neuropsychological examination can contribute to the clinical diagnosis. Differentiation from delirium and depression, which may co-exist with dementia, is necessary. Once a dementia syndrome has been diagnosed its cause has to be ascertained. Alzheimer's disease is the most common cause and can often be diagnosed clinically. The clinical suspicion of vascular dementia has to be confirmed by imaging methods. Drug intoxication may cause or contribute to dementia. Blood tests should be performed routinely, but EEG, CT or MRI, SPECT and genetic tests can be carried out on clinical indication. Subsequently the need for care of the patient has to be established, as well as the ability of the carers to meet it. Regular follow-up is necessary. A definite diagnosis can only be made post-mortem when neuropathological examination has been performed. The organisation of diagnosis in the dementia syndrome should preferably take place in specialised multidisciplinary teams.
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