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[Two clinical cases diagnosed provisionally as Binswanger type of vascular dementia--on possibility to its clinical recognition from neuropsychiatric findings and computed tomography (author's transl)].

The diagnosis of subcortical arteriosclerotic encephalopathy of Binswanger should be confirmed neuropathologically by postmortem examination. But we expect that it may be possible to diagnose provisionally as Binswanger's disease on the basis of clinical course and findings including computed tomography. Two cases, 57 years old postman (case 1) and 60 years old housewife (case 2) were reported. They had hypertension for over 20 years in their past history and showed a progressive dementia since before one year and 9 years. Transient syncopal attack and epileptiform seizure revealed occasionally. Psychiatrically either of them were apathetic, aspontaneous and autistic. One of them (case 2) showed a striking paranoid-hallucinatory state. Spastic gait disturbance and dysarthria are found neurologically, and moreover in case 1 revealed the incontinence of urine and feces. Arteriosclerotic changes were seen in the fundi. The EEG showed a slow alpha rhythm with scattered theta- and delta-waves. Laboratory data of blood, urine and CSF were normal. On the basis of these clinical course and findings the diagnosis of Binswanger's type of cerebral arteriosclerosis were suspected. The CT-scan of 2 cases proved a symmetrical enlargement of the lateral ventricles and marginated areas of definite abnormal low density in the white matter of the occipital (case 1) and frontal (case 1 and 2) lobes. Conclusively we may provisionally diagnosed our two cases as Binswanger's disease, also supported by the findings of CT.

Dementia↗

[The life of Joseph Haydn from a medical point of view (author's transl)].

Joseph Haydn (1732 to 1809) had hardly been troubled by any illness of note, apart from a chronic nasal polyp until 1799. During the composition of the oratoria "The Seasons" (1799 to 1801), the signs of cerebral arteriosclerosis with cardiac insufficiency made their appearance. Haydn's creative strength subsequently declined rapidly and he was virtually unable to compose any more after he had finished "The Seasons" until his death in 1809. The plan to compose a third oratoria "The Last Judgement" ended abortively. Haydn himself suffered acutely from the inactivity imposed on him by the illness, as documented by various sources. Hadyn remained fully conscious right up to the time of his death, which presumably resulted from cardiac failure. Haydn's grave was ransacked only a few days after the funeral and his skull was snatched. It has been finally restored to his resting place in Eisenstadt only as recently as 1954 after a protracted and devious journey.

Austria↗

[Occurrence, causes and clinical characteristics of status epilepsy in adults].

INTRODUCTION: Status epilepticus, particularly grand mal, is one of the gravest and most dramatic conditions in neurology requiring immediate attention. Status epilepticus can occur in epileptic patients, often with higher mortality rates in symptomatic than idiopathic, but also as an initial symptom of a number of neurological and systemic diseases. No data are available on the exact incidence rates of status epilepticus. According to some assessments, 10% of patients have at least one status epilepticus in their lifetime (3,6). The prognosis mostly depends on the main cause, time in which seizures are stopped and age of patients. Latest data available in literature suggest the mortality rate of 2-8%. MATERIALS AND METHODS: We analyzed frequency of hospital admissions, causes and clinical characteristics of status epilepticus in adults. The study was retrospective, based on case histories of epileptic patients from the Intensive Care Unit of the Neurology Clinic in Novi Sad in 1990, 1993 and 1995. Special emphasis was placed on differences in studied parameters between cases confirmed earlier and those with status epilepticus occurring as an initial symptom of some other illness or condition. RESULTS: Number of hospital admissions rose slightly in the interval observed in comparison with total admissions (0.68% in 1990, 1.24% in 1993, and 1.73% in 1995) (Tabs 1 and 2). During 1993, status epilepticus was more frequent in cases confirmed earlier (69%) compared with the years 1990 (56%) and 1995 (43%) (Graf.1). Epileptic patients were younger on the average than nonepileptic ones (Tab. 3). Status epilepticus occurred more often in male patients (Tab. 4). Irregular treatment was the prevailing cause in epileptic patients (Tab 5). Symptomatic status epilepticus was reported higher in 1990 and 1995, and stroke was definitely the predominant cause (Tab 6). Convulsive grand mal status prevailed in all patients (Graf 2). Focal status was a more frequent finding in nonepileptic patients (Graf 3). Every third in 16 patients died in 1993 and every fifth in 23 in 1995 probably due to the acute destructive brain damage rather than the status itself. No deaths occurred in 1990. DISCUSSION: According to research carried out by other authors, half of grand mal status cases occurred in confirmed epileptics (4). In our study the grand mal status was reported in 70.4% cases of epilepsy. Primary cause was abrupt withdrawal of antiepileptic treatment, infections, alcohol abuse and use of convulsive drugs. This is compatible with our results which confirm that grand mal status either primary or with secondary generalization prevail in both groups of patients (7,8,9). In terms of causes of status epilepticus in nonepileptic patients, literature data mainly suggest cerebral trauma, frontal brain tumors, cerebral arteriosclerosis or other vascular disorders and anaphylaxis (4). Our results point to stroke as the major cause of status epilepticus in nonepileptic patients, similar with data presented by Towne (10). There is no data in literature concerning the relation between sex of patients and occurrence of status. In our study status epilepticus occurred more frequently in male patients. CONCLUSION: The grand mal status was the major clinical type of status in all patients and was primarily caused by discontinued or irregular antiepileptic treatment in patients with confirmed epilepsy, and by stroke in nonepileptic patients.

Adult↗

The event-related P300 potential approach to cognitive functions of nondemented patients with cerebral and peripheral arteriosclerosis.

OBJECTIVES: To detect subtle cognitive dysfunction in non-demented patients with either cerebral (cAD) or peripheral (pAD) arteriosclerotic disease, and to evaluate in these patients the effects on cognitive functions of carotid endarterectomy and bypass surgery, respectively. DESIGN: Case-control study. SETTING: Tertiary care referral center. PARTICIPANTS: Eighty consecutive patients with moderate to high-grade stenosis of the internal carotid artery (ICA) (mean age +/- SD, 62 +/- 8 years), 53 patients with stenoses of the peripheral arteries (60 +/- 10 years), and 80 healthy volunteers (58 +/- 15 years) enrolled in a study on healthy aging. Cerebral and peripheral arteriosclerotic disease was verified by digital subtraction angiography, and all patients were screened for confounding effects of concomitant diseases. MAIN OUTCOME MEASURES: Cognitive functions by event-related visual P300 potentials. RESULTS: Patients with cAD showed prolonged P300 latencies and reduced P300 amplitudes, whereas pAD patients had reduced P300 amplitudes only. On an individual scale, 25% of cAD patients, but only 6% of pAD patients, revealed P300 abnormalities. In the cAD, but not in the pAD patients, the P300 latencies were especially prolonged in the older patients, but other factors such as sex, cerebral symptoms, degree of ICA stenosis, and premorbid intelligence did not play any role in either group. Within 1 to 2 weeks of surgery, the P300 latencies shortened in both cAD and pAD patients with high initial values. CONCLUSIONS: As demonstrated by P300 potentials, even nondemented arteriosclerotic patients reveal signs of subtle cognitive dysfunction affecting especially the older cAD patient. In the short-term, carotid endarterectomy presumably improves cognitive functions unspecifically in nondemented patients with a higher initial degree of P300 abnormality.

Adult↗

[Therapeutic efficacy of pyridinolcarbamate (Anginin) in patients with combined cerebral and peripheral arteriosclerosis].

A group of 20 patients (average age 55.5 yrs) with advanced arterioscleorosis obliterans and cerebral thrombosis were treated with pyridinolcarbamate. Anginin was given for 6 months, 1,5--2 g. per day. Observation period before, during and after the treatment lasted 2 yrs. Medical and psychological examinations were performed; laboratory test included: sphigmooscylography, photoplethysmography, EKG, EEG, cholesterol, triglyceridis and other routine biochemical blood tests. All data are statistically evaluated. Results. During Anginin treatment clinical improvement was noted in 8 cases, in 9 there was no change in neurological examination; 3 cases--deteriorated. Control examination 6 to 12 months after discontinuation of the treatment revealed no changes in 13 patients; 1 patient deteriorated and 6--died. There was improvement in blood circulation as shown by plethysmography and sphigmooscylography in patients with less advanced vascular changes and no change in cases with more advanced disease. In 75% of the patients there was statistically significant (p less than 0.001) decrease of cholesterol and in 50% of triglicerids (not statistically significant). In patients with advanced disease there was no clinical and EEG improvement, on the contrary 1/3 of them deteriorated clinically and in EEG. In cases with less advanced disease clinical improvement was recorded. Anginin seems to be a good drug for patients with moderately advanced combined peripheral and central arteriosclerosis.

Adult↗

Selective determination of cholesterol in high density lipoprotein subfractions (HDL2 and HDL3) in patients with cerebral and peripheral arteriosclerosis.

Cholesterol levels in high density lipoprotein subfractions (HDL2 and HDL3) were evaluated in 69 patients (55 males, average age +/- SD 58.3 +/- 8.8, and 14 females, average age +/- SD 63.1 +/- 10.3) with extra-coronary arteriosclerosis (lower limbs, supraaortic trunks and both sites), and in 79 healthy age-matched control subjects. HDL cholesterol was significantly reduced in male and female patients. The HDL cholesterol decrease was due to a fall in both HDL2 and HDL3 cholesterols; nonetheless, an analysis of the HDL2-cholesterol/HDL3-cholesterol ratio disclosed that HDL2 cholesterol was the most reduced. Slightly higher plasma cholesterol and triglyceride levels were found in the patients as well as a higher plasma cholesterol/HDL-cholesterol ratio. On the contrary, the HDL2-cholesterol/HDL3-cholesterol ratio was significantly reduced in the patients. These preliminary findings suggest that, as in ischemic heart disease, the HDL cholesterol reduction in cerebral and peripheral arteriosclerosis is also mainly due to a reduction in the HDL2 subfraction. These results also lend further support to the proposal that determination of the HDL subfractions is useful for a better assessment of the risk profile for arteriosclerosis.

Aged↗

[Young-adult-onset hereditary subcortical vascular dementia: cerebral autosomal recessive arteriosclerosis with subcortical infarcts and leukoencephalopathy (CARASIL)].

The clinical features of a probably autosomal recessive syndrome ("CARASIL"), yet to be confined in Japan and characterized by prematurity of vascular dementia, alopecia and spondylosis deformans are reviewed through comparison with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), which has been reported in Europe and North America, and recently in Japan. These two syndromes have many common features, such as familiality, encephalopathy of Binswanger type, and absence of vascular risk factors. There exists, however, a number of differences as follows: (1) Onset of encephalopathy is 32 years of age in "CARASIL" vs. 45 in CADASIL. (2) Male to female ratio is 3.2: 1 vs. 2:1.(3) Two thirds of "CARASIL" patients show stroke and/or stepwise deterioration, while almost all CADASIL patients have stroke. (4) Associated psychiatric features are euphoria, emotional lability and loss of spontaneity vs. severe mood disorders. (5) Migraine is a cardinal feature of CADASIL and vasospasm may occur during cerebral angiography. (6) White matter lesions on MRI are diffuse and homogeneous vs. punctuated and nodular. The latter four differences may mirror the difference in the pathology of arteriopathies. "CARASIL" is clearly different from CADASIL and reflect a second genetic condition with a seemingly direct effect upon the cerebral vasculature.

Age of Onset↗