1/75. fluorouracil-induced neurotoxicity.OBJECTIVE: To report a case of acute neurologic adverse effects related to fluorouracil administration and to review the neurotoxicity of this agent. CASE SUMMARY: A 73-year-old white man with a history of esophageal carcinoma was treated with fluorouracil 1,500 mg iv daily for four days. After completing treatment, he presented with sudden onset of confusion, cognitive disturbances, a cerebellar syndrome, and repeated seizures. A magnetic resonance image of the brain showed no structural abnormalities, and cerebrospinal fluid examination was normal; none of the other laboratory tests provided an explanation for his symptoms. The patient was treated with anticonvulsants, and the cognitive changes resolved in 72 hours. The cerebellar signs, however, did not resolve completely and persisted when the patient was examined two weeks after discharge. DISCUSSION: fluorouracil can cause both acute and delayed neurotoxicity. Acute neurotoxicity manifests as encephalopathy or as cerebellar syndrome; seizures, as seen in our patient, have rarely been reported. Acute neurotoxicity due to fluorouracil is dose related and generally self-limiting. Various mechanisms for such toxicity have been postulated, and treatment with thiamine has been recommended. Delayed neurotoxicity has been reported when fluorouracil was given in combination with levamisole; this form of subacute multifocal leukoencephalopathy is immune mediated and responds to treatment with corticosteroids. CONCLUSIONS: Clinicians should be aware of the adverse neurologic effects of fluorouracil and should include them in the differential diagnosis when patients receiving the drug present with neurologic problems.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/75. Acute confusional state as presenting feature in aneurysmal subarachnoid hemorrhage: frequency and characteristics.In many patients with subarachnoid hemorrhage (SAH) there is a delay between the onset of symptoms and admission to hospital. An important cause for the delay is an initially erroneous diagnosis. The goal of this study was to determine the frequency of acute confusional state (ACS) as a presenting symptom of SAH and to describe the clinical and radiological characteristics of these patients. We studied all 717 patients registered from January 1989 to July 1997 in the SAH database of the University Medical Center Utrecht. For patients who presented with ACS we reviewed the computed tomography scans for baseline characteristics: the amount of cisternal blood, intraventricular or intracerebral hemorrhage, and hydrocephalus. Details about features of onset were known for 646 patients. Nine patients (1.4%) presented with ACS. In five patients ACS was either preceded by a period of loss of consciousness or accompanied by severe headache. Subtle focal deficits were found at initial neurological examination in four patients. Computed tomography demonstrated a frontal hematoma in three patients and hydrocephalus in four. The site of the ruptured aneurysm was at the anterior communicating artery in four patients, at the internal carotid artery in two, and at the basilar artery in two. In our series, one per 70 patients with SAH presents with ACS. Keys to early diagnosis of SAH in patients presenting with ACS are a preceding period of loss of consciousness and severe headache on neurological assessment.- - - - - - - - - - ranking = 56.197193556417keywords = cerebral, intracerebral (Clic here for more details about this article) |
3/75. Postoperative confusion preceded by decreased frontal lobe haemoglobin oxygen saturation.We describe a 58-year-old male patient with confusion and prolonged recovery after liver transplantation. A cause was not apparent for the confusion, but during surgery, monitoring of the frontal lobe cerebral haemoglobin oxygen saturation by near-infrared spectrophotometry showed cerebral hypo-oxygenation despite optimization of conventional cardiovascular parameters. It is possible that intraoperative cerebral ischaemia is the cause of postsurgical confusion and with near-infrared spectrophotometry this hypothesis may be tested clinically.- - - - - - - - - - ranking = 143.01131213272keywords = cerebral (Clic here for more details about this article) |
4/75. Novel prion protein gene mutation in an octogenarian with Creutzfeldt-Jakob disease.BACKGROUND: The transmissible spongiform encephalopathies constitute a fascinating and biologically unique group of invariably fatal neurodegenerative disorders that affect both animals and humans. Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia represent the more common human phenotypes. Excluding the small number of iatrogenically transmitted cases, approximately 85% to 90% of patients develop CJD without identifiable explanation, with an increasing number of different mutations in the prion protein gene (PRNP) recognized as probably causative in the remainder. OBJECTIVE: To report on an 82-year-old woman with pathologically confirmed CJD found unexpectedly to harbor a novel mutation in PRNP. methods: Routine clinical investigations were undertaken to elucidate the cause of the rapidly progressive dementia and neurological decline manifested by the patient, including magnetic resonance imaging of the brain, electroencephalography, and cerebrospinal fluid analysis for the 14-3-3 beta protein. Standard postmortem neuropathological examination of the brain was performed, including immunocytochemistry of representative sections to detect the prion protein. Posthumous genetic analysis of the open reading frame of PRNP was performed on frozen brain tissue using polymerase chain reaction and direct sequencing. RESULTS: Concomitant with the exclusion of alternative diagnoses, the presence of characteristic periodic sharp-wave complexes on the electroencephalogram in combination with a positive result for 14-3-3 beta protein in the cerebrospinal fluid led to a confident clinical diagnosis of CJD, confirmed at autopsy. There was no family history of dementia or similar neurological illness, but patrilineal medical information was incomplete. Unexpectedly, full sequencing of the PRNP open reading frame revealed a single novel mutation consisting of an adenine-to-guanine substitution at nucleotide 611, causing alanine to replace threonine at codon 188. CONCLUSIONS: In addition to expanding the range of PRNP mutations associated with human prion diseases, we believe this case is important for the following reasons. First, from an epidemiological perspective, the avoidance of occasional incorrect classification of patients manifesting neurodegenerative disorders that may have a genetic basis requires systematic genotyping, particularly when there are uncertainties regarding the family history. Second, the incidence of spongiform encephalopathy in elderly patients beyond the typical age range may be underestimated and does not preclude a genetic basis. Finally, as a corollary, this case highlights problematic issues in human transmissible spongiform encephalopathies, as illustrated by disease penetrance and age of onset in genotype-phenotype correlations.- - - - - - - - - - ranking = 3keywords = brain (Clic here for more details about this article) |
5/75. Early diagnostic magnetic resonance imaging in acute disseminated encephalomyelitis.We report two cases and review the literature concerning the importance of early magnetic resonance imaging (MRI) of the brain as a guide for the early diagnosis and treatment of acute disseminated encephalomyelitis (ADEM). A nonspecific term, ADEM refers to an acute disease that is postinfectious, parainfectious, postvaccinal, or of an unknown precipitating factor. Often when there is clinical suspicion of ADEM, MRI is not done before significant morbidity and mortality occur, despite the existence of adequate treatments. Primary care physicians should be aware of the importance of early MRI in ADEM.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
6/75. Acute confusion secondary to pneumocephalus in an elderly patient.PRESENTATION: an 83-year-old man was admitted to hospital with acute confusion 3 days after a direct flight from australia. OUTCOME: computed tomography (CT) brain scan and magnetic resonance imaging head scan revealed the cause to be pneumocephalus, apparently the result of barotrauma caused by Valsalva manoeuvres when he attempted to unblock his nose during the flight. After 5 days of nursing in the vertical position the patient's Abbreviated Mental Score returned to normal. A CT brain scan 6 weeks later showed complete resolution of the pneumocephalus.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
7/75. Rapid spontaneous resolution and redistribution of acute subdural hematoma in a patient with chronic alcoholism: a case report.We report a case of a 54-year-old man who had documented traumatic acute subdural hematoma. He suffered from a transient episode of confusion and a follow-up CT scan of brain 6 h after the initial scan showed resolution and redistribution of the subdural hematoma. In this case report, we review the literature for the underlying pathophysiology of this uncommon phenomenon.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/75. Paradoxical lithium neurotoxicity: a report of five cases and a hypothesis about risk for neurotoxicity.There have been many reports of probable lithium-induced organic brain syndromes occurring when serum lithium levels are within or close to the therapeutic range. The authors report on five patients who developed clinical syndromes suggestive of severe neurotoxicity during lithium treatment. In all cases lithium levels were between .75 and 1.7 mEq/liter. The patients who developed neurotoxicity had markedly higher global ratings of psychotic symptomatology and anxiety in the pretoxic period than did patients who never deveoped neurotoxicity. When the acute manic state is characterized by marked psychotic symptoms and intense anxiety, it may be associated with increased vulnerability to the development of severe lithium neurotoxicity.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/75. Leptomeningeal glioblastoma presenting with multiple cranial neuropathies and confusion.glioblastoma multiforme (GBM) is the commonest primary malignant neoplasm of the CNS. Usually, patients present with seizures and headache but in the elderly, confusion and generalised cognitive decline are more frequently the initial features. Multiple cranial nerve lesions as a manifestation of leptomeningeal meningitis is a rare presentation of GBM. The diagnosis is not often suggestive on either brain computed tomography (CT) or magnetic resonance imaging (MRI) and is usually confirmed by cerebrospinal fluid (CSF) cytology or histology. We describe the case of an 80-year-old man, who presented with multiple cranial nerve palsies and confusion secondary to leptomeningeal gliomatosis, in whom GBM was detected along the intra-ventricular lining of the left lateral ventricle at ventriculoscopy, in the absence of a distinct parenchymal lesion.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/75. Susac's syndrome: beneficial effects of corticosteroid therapy in a Japanese case.Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bi- a lateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions ti predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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