1/172. Subcortical arteriosclerotic encephalopathy (Binswanger's disease). A vascular etiology of dementia.A 51-yearold man with moderate intermittent hypertension had a rapidly progressive, profound dementia in the absence of significant localizing neurological signs. Postmortem examination disclosed the vascular alterations and diffuse white matter degeneration which characterize subcortical arteriosclerotic encephalopathy (SAE) or Binswanger's disease. The case underscores the need to consider vascular disease as an etiology of dementia -- even in the absence of focal neurological deficit.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/172. Aicardi-Goutieres syndrome: a genetic microangiopathy?Aicardi-Goutieres syndrome (AGS) (McKusick 225750) is an autosomal recessive disease with onset in the 1st year of life, resulting in progressive microcephaly, calcification of cerebral white matter, thalamus and basal ganglia, generalized cerebral demyelination and a chronic low-grade CSF lymphocytosis, without evidence of infection. We report the autopsy of a patient who died with this disorder at the age of 17 years. Findings were severe microencephaly, diffuse but inhomogeneous cerebral white matter loss with associated astrocytosis, calcific deposits in the white matter, thalami and basal ganglia. neocortex and cerebellar cortex were affected by wedge-shaped microinfarctions. Small vessels showed calcification in the media, adventitia and perivascular spaces. These findings are similar to some previous publications that in retrospect may have been AGS, but this is the first reported cerebral microangiopathy in which the diagnosis AGS was made during lifetime. This report provides evidence that microangiopathy plays a significant role in the pathogenesis of AGS.- - - - - - - - - - ranking = 32.112304758613keywords = microangiopathy (Clic here for more details about this article) |
3/172. Neurofibromatosis, stroke and basilar impression. Case report.Neurofibromatosis type 1 (NF1) can virtually affect any organ, presenting most frequently with "cafe au lait" spots and neurofibromas. Vasculopathy is a known complication of NF1, but cerebrovascular disease is rare. We report the case of a 51-year-old man admitted to the hospital with a history of stroke four months before admission. On physical examination, he presented various "cafe au lait" spots and cutaneous neurofibromas. neurologic examination demonstrated right-sided facial paralysis, right-sided hemiplegia, and aphasia. Computed tomography scan of head showed hypodense areas in the basal ganglia and centrum semiovale. Radiographs of cranium and cervical spine showed basilar impression. angiography revealed complete occlusion of both vertebral and left internal carotid arteries, and partial stenosis of the right internal carotid artery. A large network of collateral vessels was present (moyamoya syndrome). It is an uncommon case of occlusive cerebrovascular disease associated with NF1, since most cases described in the literature are in young people, and tend to spare the posterior cerebral circulation. Basilar impression associated with this case may be considered a pure coincidence, but rare cases of basilar impression and NF1 have been described.- - - - - - - - - - ranking = 2keywords = vascular disease (Clic here for more details about this article) |
4/172. Painless aortic dissection presenting as hoarseness of voice: cardiovocal syndrome: Ortner's syndrome.Most of the neurological manifestations of the aortic dissection are due to neuronal ischemia secondary to either extension of the dissection process into a branch artery, or compression of an artery by the false lumen of the dissecting aortic hematoma. However, the enlarging false lumen may directly compress on an adjacent nerve, causing neuronal injury resulting in neurological symptoms. This may particularly take place when a distal intimal tear does not decompress the false lumen, resulting in formation of an expanding blind pouch. About 10% of aortic dissections are painless and may present with symptoms secondary to the complications of the dissection. Although cardiovocal syndrome, or Ortner's syndrome (hoarseness of voice due to involvement of recurrent laryngeal nerve in cardiovascular diseases) has been described with aortic dissection, it has not been reported as an initial presenting feature of this disorder. This report describes the first case of painless aortic dissection presenting with hoarseness of voice, the cardiovocal syndrome. The hoarseness remained the only symptom throughout the entire course of the disease. The aortic dissection was not suspected initially. During surgical exploration, the recurrent laryngeal nerve was found compressed by the false lumen at the level of aortic arch. Aortic root replacement was performed successfully, resulting in complete resolution of the hoarseness. The neurological manifestations of aortic dissection, and the cardiovocal syndrome, are discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/172. A novel syndrome of diabetes mellitus, renal dysfunction and genital malformation associated with a partial deletion of the pseudo-POU domain of hepatocyte nuclear factor-1beta.Mutations in the homeodomain-containing transcription factor hepatocyte nuclear factor (HNF)-1beta are the cause of one form of maturity-onset diabetes of the young (MODY), type 5 (MODY5). We have studied a Norwegian family, N5, with a syndrome of mild diabetes, progressive non-diabetic renal disease and severe genital malformations. The sequence of the HNF-1beta gene ( TCF2 ) revealed a 75 bp deletion in exon 2 (409-483del) which would result in the synthesis of a protein lacking amino acids Arg137 to Lys161 (R137-K161del). This deletion is located in the pseudo-POU region of HNF-1beta, a region implicated in the specificity of dna binding. Functional studies of R137-K161del HNF-1beta revealed that it could not bind an HNF-1 target sequence or stimulate transcription of a reporter gene indicating that this is a loss-of-function mutation. The R137-K161del allele co-segregated with diabetes and renal disease in pedigree N5. In addition, two of four female carriers with this mutation had vaginal aplasia and rudimentary uterus (Mullerian aplasia). These studies strongly suggest that heterozygous mutations in the HNF-1beta gene are associated with a syndrome characterized by MODY and severe, non-diabetic renal disease. Moreover, the presence of internal genital malformations in two females suggests that additional clinical features may be associated with HNF-1beta mutations.- - - - - - - - - - ranking = 6.7863630290621keywords = diabetic (Clic here for more details about this article) |
6/172. The double-crush phenomenon--an unusual presentation and literature review.The double-crush syndrome was initially described by Upton and McComas in 1973. They postulated that nonsymptomatic impairment of axoplasmic flow at more than one site along a nerve might summate to cause a symptomatic neuropathy. This was suggested by their clinical observation that the majority of their patients had a median or ulnar neuropathy associated with evidence of cervicothoracic root lesions. They also hypothesized that one of the constraints on axoplasmic flow could be a metabolic neuropathy, and this is supported by the high association of diabetes and carpal tunnel syndrome. Other researchers have since reported series of patients supporting the frequent association of a proximal and distal nerve compression syndrome, including carpal tunnel syndrome associated with cervical radiculopathy, brachial plexus compression, and diabetic neuropathy. Subsequently, MacKinnon and Dellon have expanded the description of this syndrome to include a) multiple anatomic regions along a peripheral nerve, b) multiple anatomic structures across a peripheral nerve within an anatomic region, c) superimposed on a neuropathy, and d) combinations of the above. We present an unusual case of symptomatic nerve compression caused by two nonanatomic structures within an anatomic region.- - - - - - - - - - ranking = 3.393181514531keywords = diabetic (Clic here for more details about this article) |
7/172. popliteal artery entrapment syndrome: specific aspect.A case of popliteal artery entrapment syndrome (PAES) is reported. A non smoker, 63-year-old man, consulted for severe claudication of the lower limb, with a sudden onset. There was no past history of vascular disease. Neither the arteriography nor the arterial doppler led to definite diagnosis. In our case, only the C.T. scan was contributive to the diagnosis. The age, 63, at which this abnormality became symptomatic, the abrupt appearance of ischaemic symptoms and the embryologic type of the arterial stenosis were particular. The surgical management was the only therapeutic option.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/172. The C.C.A. syndrome (congenital contractural arachnodactyly): a new differential syndrome for Marfan's syndrome and homocystinuria.The first case in the dental literature of congenital contractural arachnodactyly (C.C.A. syndrome) is presented. This newly delineated syndrome is an autosomal dominant heritable disorder of connective tissue. Its similarities to Marfan's syndrome and homocystinuria, as well as other syndromes, are discussed. The lack of cardiovascular disease, specific ocular anomalies, and mental retardation are presented in the differential diagnosis of the C.C.A syndrome with Marfan's syndrome and homocystinuria.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
9/172. The cognitive syndrome of vascular dementia: implications for clinical trials.dementia is common among patients with cerebrovascular disease, particularly in a setting of one or more clinically evident strokes. Prior cohort and case studies have suggested that the cognitive syndrome of vascular dementia is characterized by predominant executive dysfunction, in contrast to the deficits in memory and language function that are typical of patients with alzheimer disease. The course of cognitive decline may also differ between those dementia subtypes, with many, but not all, patients with vascular dementia exhibiting a stepwise course of decline caused by recurrent stroke and most patients with alzheimer disease exhibiting a gradually progressive course of decline. The findings of prior studies of the cognitive syndrome of vascular dementia must be interpreted with caution, however, because of (1) possible inaccuracies in the determination of the dementia subtype and the loss of precision that might result from pooling heterogeneous subgroups of patients with vascular dementia, (2) difficulties inherent in identifying a pattern of strengths and weaknesses in patients who are required to have memory impairment and other deficits to meet operationalized criteria for dementia, and (3) the use of limited test batteries whose psychometric properties are incompletely understood. Specific questions that should be addressed by future studies are discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/172. Reversible tetraplegia due to polyneuropathy in a diabetic patient with hyperosmolar non-ketotic coma.critical illness polyneuromypathy has not previously been reported as a complication of diabetic coma. We describe a patient with hyperosmolar non-ketotic coma (HONK) complicating gram-negative sepsis in whom persistent coma and profound tetraplegia caused considerable concern. Although, initially, it was feared that the patient had suffered a central neurological complication such as stroke or cerebral oedema, a diagnosis of critical illness motor syndrome (CIMS) was subsequently confirmed neurophysiologically. Profound limb weakness associated with HONK is not necessarily due to a catastrophic cerebral event, rather it may be a result of CIMS, which has an excellent prognosis for full neurological recovery.- - - - - - - - - - ranking = 16.965907572655keywords = diabetic (Clic here for more details about this article) |
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