Cerebellar developmental venous anomalies with associated vascular pathology.

Cerebellar developmental venous anomalies (CDVA) are benign conditions, although sometimes they are associated with haemorrhages and reported to be symptomatic. This is the largest follow-up study to investigate the symptomatology of CDVAs and their association with other malformations. Thirty-two patients were followed for 2 - 9 years. Twenty-eight had isolated asymptomatic CDVA without any neurological condition during follow-up, which might be linked to the CDVA. Four patients had CDVA and an associated vascular pathology: two pontine cavernomas, one asymptomatic arteriovenous malformation (this is the first published case in the literature) and one cerebellar infarct with a developmental variation of the posterior fossa venous circulation. One patient had two CDVAs, while another had a unique draining vein from the upper part of the brainstem too. In conclusion, CDVAs are benign, asymptomatic conditions, but they are sometimes associated with pathogenic malformations requiring detailed neuroradiological investigations.

Familial prion disease in a Hungarian family with a novel 144-base pair insertion in the prion protein gene.

About 15% of human prion diseases are inherited, and are associated with point or insertional mutations of the prion protein gene (PRNP). Four families with six octapeptide repeat insertions (OPRI) in the PRNP gene have been described in the literature so far. Here we report two cases in a Hungarian family with a new six OPRI (R1R2R2R3R2R3gR3R2R2R3R4) in the PRNP gene. The clinical features (progressive ataxia, dementia and anosmia), the age of onset and the duration of disease were almost identical. In addition to the cerebellar and parahippocampal pathological changes already described, we also found deposits of pathological prion protein in the olfactory system.

Superior sensitivity of motor over somatosensory evoked potentials in the diagnosis of cervical spondylotic myelopathy.

Myelopathy secondary to cervical spondylosis is often a difficult clinical diagnosis. Furthermore, with the introduction of magnetic resonance imaging (MRI) an increasing number of patients are identified with spondylotic cervical spinal cord compression. We analyzed the value of functional assessment of the spinal cord by motor and sensory evoked potentials (MEP and SEP) in the detection of myelopathy, with special emphasis on the correlation of clinical and electrophysiological findings. Fifty-one patients with at least some degree of spinal cord compression because of cervical spondylosis, as shown by MRI, were included in the study, grouped according to clinical symptoms. We found that patients who had no clinical symptoms whatsoever indicating myelopathy (they were referred to MRI examination mostly because of cervical radiculopathy), had in the large majority normal MEP and SEP findings. Patients with slight, unspecific and non-confirmative symptoms without pyramidal signs had mostly abnormal MEP but normal SEP findings. This points to the superior sensitivity of MEP over SEP in detecting myelopathy in its early stages. Patients with obvious clinical signs of myelopathy, including pyramidal signs had both abnormal MEP and SEP findings. Altogether these findings may help clinicians in interpreting MRI signs of cervical spinal cord compression.

MR findings in subacute combined degeneration of the spinal cord caused by nitrous oxide anaesthesia--two cases.

Vitamin B12 deficiency causes haematological and neurological diseases. Subacute combined degeneration (SCD) of the spinal cord is characterized by degeneration of the posterior and lateral columns. We report two cases of SCD induced by nitrous oxide (N2O) anaesthesia. In both cases magnetic resonance imaging (MRI) of the spinal cord showed symmetric, reversible changes in the posterior columns, correlating well with patients' symptoms.

Inherited prion disease with A117V mutation of the prion protein gene: a novel Hungarian family.

Three members of a family with inherited prion disease are reported. One additional family member had a progressive neurological disease without details. Two developed symptoms of ataxia, dementia, myoclonus, rigidity, and hemiparesis, and one had a different phenotype with the combination of lower motor neuron deficit, parkinsonism, intellectual decline, and ataxia. In this last patient cell loss of the anterior horn motor neurons and chronic neurogenic muscle atrophy was evident. Immunostaining for the prion protein disclosed unicentric and multicentric plaques, and coarse and fine granular positivity. Genetic analysis of the prion protein gene of the propositus showed a 117 codon alanine to valine mutation and homozygous 129 valine/valine genotype.

Vogt-Koyanagi-Harada disease presenting initially as aseptic meningoencephalitis.

Vogt-Koyanagi-Harada (VKH) disease in a Gypsy woman was diagnosed 4 months after her initial complaints. The delay is explained by the facts that: (1) the characteristic ophthalmological symptoms, which usually herald the disease and ensure early diagnosis, developed only late during the course; and (2) only retrospective analysis of the cerebrospinal fluid (CSF) cell preparation proved the presence of melanin-laden macrophages (MLMs), specific for the syndrome. We emphasize that VKH syndrome may initially present as aseptic meningitis, without specific ophthalmological symptoms. In suspected cases a very detailed CSF cell analysis is needed, because the presence of MLMs could confirm the diagnosis. However, VKH syndrome has a much higher incidence in Asia; cases in other races, including white people in Europe, also occur.

Primary progressive aphasia: a case report.

We report a 69-year-old male patient whose motor aphasia started at the age of 61. The language disability remained isolated and progressed over a period of eight years without any additional cognitive deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed moderate cortical atrophy with frontal dominance. Single photon emission tomography (SPECT) showed hypoperfusion in the frontotemporoparietal region, positron emission tomography (PET) demonstrated a global cortical reduction of glucose utilization with a lesser decrement in the occipital lobes. The clinical symptoms and the neuropsychological findings fit the diagnosis of primary progressive aphasia.

[Analysis of the activity of neurologic intensive care units]. / Neurológiai intenzív részlegek müködésének elemzése.

The authors analysed six-year activity of the intensive Care Unit of Department of Neurology, Medical University of Pécs (POTEI), and two-year activity of the Intensive Care Unit of Department of Neurology, Semmelweis University of Medicine, Budapest (SOTEI). Mortality at POTEI and SOTEI was 33.9% and 32.2%, respectively. Mean duration of stay of survivors at POTEI was 10 +/- 12.8 days, and 7 +/- 6.8 days at SOTEI; mean duration of care of the deceased patients at POTEI was 6.3 +/- 10.5 days, and 10 +/- 13.7 days at SOTEI. At POTEI 60.7%, at SOTEI 63% of the patients was admitted because of cerebrovascular insult. Mortality of patients with brain haemorrhage at POTEI and SOTEI was 53.4% and 57.7% respectively. Mortality of the ischaemic group was 40.6% (POTEI) and 35.3% (SOTEI). In the group of intracranial tumours 44.4% mortality was recorded at POTEI and 47.6% at SOTEI. At POTEI 240 patients (15.9%), while at SOTEI 94 patients (21%) were admitted to treat epileptic seizures. Among the 510 patients, who died within one month 284 patients (55.6%) were unconscious at admission. From those with coma due to severe structural lesion of the brain (brain ischaemia, bleeding, meningitis) only 15 patients survived. Among the 184 patients, who were comatose and survived, the most frequent diagnosis was suicidal attempt with hypnotics (n = 67), metabolic encephalopathy (n = 19) and epilepsy (n = 12). At SOTEI among the 144 deceased patients 102 (70.8%) were unconscious at admission. Coma at admission proved to be a strong predictor of mortality. Mortality of the ventilated patients was 83% at POTEI and of those having subclavian catheter (n = 592) was 47.1%. In the acute phase of brain ischaemia at POTEI 39%, at SOTEI 10.7% of the patients received heparin. At SOTEI the cost of medication of patients who died after two weeks of care was 65.2% higher than that of the survived patients.

A double-blind placebo-controlled study assessing the efficacy and tolerability of 50 mg sumatriptan tablets in the acute treatment of migraine. Sumatriptan Tablets S2CM07 Study Group.

BACKGROUND: Oral sumatriptan 50 mg has been found to have good efficacy and tolerability in the acute treatment of migraine but has been less well studied than the 100 mg dose. METHODS: This was a double-blind, parallel-group study (Glaxo Wellcome protocol number S2CM07) comparing the efficacy and safety of sumatriptan 50 mg tablets with placebo in the acute treatment of migraine. Patients treated three migraine attacks with study medication; a second, optional dose was available for treating recurrent headache. Of the 560 patients randomized, 485 treated at least one attack, 411 at least two attacks, and 362 three attacks. The primary efficacy measure was the proportion of patients who had obtained complete or almost complete headache relief at 4 h after dosing. RESULTS: For all attacks, a significantly greater proportion of patients experienced headache relief at 4 h with sumatriptan 50 mg tablets than with placebo (59% to 62% versus 32% to 42%; P = 0.005). The same was true at 3 h across all attacks, and at 2 h for attacks 1 and 2 (49% versus 23% and 45% versus 29%, respectively). Although sumatriptan and placebo were associated with similar incidences of recurrence, sumatriptan was associated with a longer time to recurrence. The incidence of adverse events with sumatriptan was similar to that with placebo, and there was no increase in adverse events associated with use of a second dose to treat recurrence. CONCLUSIONS: Sumatriptan 50 mg tablets are well tolerated and efficacious in relieving migraine headache.

Reversible focal MRI abnormalities due to status epilepticus. An EEG, single photon emission computed tomography, transcranial Doppler follow-up study.

We demonstrate clinical data and findings of MRI, transcranial Doppler (TCD), single photon emission computed tomography (SPECT) and electroencephalography (EEG) in an 8 month follow-up study of a 15 year old girl who developed focal status epilepticus with sensory and visual illusions. EEG showed right temporal and occipital seizure activity and attenuation of the alpha activity with right predominance. MRI showed a right temporo-parietal hyper signal on the T2 weighted images involving the cortex with sulcal effacement. MRI-angiography suggested insufficient flow in the right transverse sinus. TCD detected an elevated flow velocity in the ipsilateral middle cerebral artery during status epilepticus, corresponding to an increased perfusion of the epileptic area revealed by SPECT. After normalization of the TCD finding, the MRI detected persistent cortical abnormality beyond the 70th day after admission. MRI normalized on the 103rd day of follow-up. Serial EEG frequency analysis demonstrated the recovery of alpha peak frequency on the left side, but the attenuation of rhythmic signals remained persistent on the right. In our case, the restitution of postictal EEG lag behind the consolidation of MRI signal abnormality.

Spectral EEG analysis following hemispheric stroke: evidences of transhemispheric diaschisis.

Quantitative EEG frequency analysis was performed within the acute stage and after the recovery in 40 patients with hemispheric stroke in order to analyze ipsi- and contralateral alpha peak frequency (APF) and band power changes. Localization of hemispheric lesion was determined by computer tomography. Changes of clinical scores were compared with the alpha asymmetries. In the cases of small subcortical infarcts good improvement of alpha activity was observed over the affected hemisphere; contralateral APF was relatively preserved. Bilateral symmetric reduction of APF was found in territorial middle cerebral artery infarcts, with poor tendency of recovery of alpha power and neurologic status. These findings suggest transitory derangement of alpha generators in the contralateral hemisphere evidenced by APF and power asymmetries. EEG signs of contralateral alpha reduction may be due to the remote effect of primary ischemic lesion indicating an electrical diaschisis phenomenon in the acute phase of stroke. EEG signs of diaschisis may anticipate a poor recovery of alpha activity and clinical status in the post-stroke period.

Failure of dopamine metabolism: borderlines of parkinsonism and dementia.

Dementia in parkinsonian patients is estimated to 10-40%. Several factors may take part in the development of cognitive impairment: i/ defective function of subcortico-cortical pathways including the mesocorticolimbic dopamine deficiency with cell loss in medial substantia nigra, the degeneration of noradrenergic, serotonergic and cholinergic systems ii/ cortical and limbic Alzheimer pathology iii/ cortical Lewy bodies iv/ vascular alterations. Using HMPAO SPECT we distinguished three main types of hypoperfusion in Parkinson patients with dementia: i/ predominantly frontal lobe type ii/ posterior ("Alzheimer-like") type iii/ multiple small vascular defects. Neuropsychological investigation was based on the use of MMSE, word pair, digit span, verbal fluency, Lurija's tapping tests. In addition CURS and Hachinski's score were also applied. Selegiline (JumexR) therapy was introduced and the patients were followed for at least 60 days. Selegiline improved significantly the MMSE values and verbal fluency in the "frontal lobe" type further the memory functions in "Alzheimer-like" patients. The mechanism is still not clarified, the increased dopaminergic tone should be taken into account.

Relation of laboratory and clinical variables to the grade of carotid atherosclerosis.

BACKGROUND AND PURPOSE: To clarify diagnostic entities in ischemic stroke we analyzed the relation between the severity of carotid atherosclerosis, coagulation parameters, lipoproteins, neurological status, and risk factors in 232 patients. METHODS: Duplex ultrasonography, computed tomography scan, and laboratory investigations were performed between the third and tenth days after stroke. Based on carotid ultrasound scores, we categorized the patients into four groups (A, B, C, and D) according to severity of atherosclerosis. Corresponding laboratory variables and clinical data were statistically analyzed. RESULTS: Ultrasound scores were significantly (P < .05) higher in the male (n = 126) versus female (n = 106) patients. The hematocrit was significantly higher and thrombin time was significantly shorter in the male group compared with the female group. Severe atherosclerosis (group C) and occlusion (group D) of the internal carotid artery was associated with smoking (C = 56%; D = 78%), hypertension (C = 43%; D = 35%), claudication (C = 13%; D = 5%), and antecedent myocardial infarction (C = 9%; D = 13%). There was no statistical correlation between ultrasound scores and the patients' neurological condition. Cholesterol and plasma fibrinogen levels were significantly higher and high-density lipoprotein cholesterol was significantly lower in groups with severe atherosclerosis compared with patients with slight intimal damage. The presence of multiple plaques or thrombosis of the internal carotid artery was concordant with the prevalence of single cerebral infarcts. CONCLUSIONS: Severity of carotid atherosclerosis corresponded well with the following factors: age, smoking, and low concentration of high-density lipoprotein cholesterol. Elevation of plasma fibrinogen combined with a loss of high-density lipoprotein cholesterol is strongly associated with severe atherosclerosis and results in brain infarction.

Topographic EEG analysis in two patients with basilar thrombosis.

The EEG activity of two patients with occlusion of the vertebral and basilar arteries was analyzed. Power maps, peak power frequency, and field power differences during both photic drive and verbal command were evaluated. Clinical findings of one patient fit the criteria of locked-in syndrome. Photic stimulation and event related desynchronization paradigm was used for testing the reactivity of EEG. Averaged EEG epochs during intended movement after verbal command showed significant alpha and sub-alpha power reduction. Regional differences of EEG reactivity were assumed secondary to the underlying hemispheric infarcts. Awareness of the patient in a locked-in state was documented by EEG analysis. In the second case permanent vegetative state was associated with a nonreactive rhythmic alpha pattern. Time sequence analysis of power ratios showed spontaneous alternating activity of alpha and sub-alpha generators. The authors conclude that pseudo-periodic fluctuation of alpha activity reflects partial preservation of thalamo-cortical connections.

Spectral EEG parameters in patients with tick-borne encephalitis: a follow-up study.

Follow-up of spectral EEG parameters were reported for five patients with serologically verified tick-borne encephalitis. Comparisons were made for clinical data and findings of EEG analysis (power probability mapping, peak-power frequencies, and alpha/theta power ratios) were compared. A prominent feature of the EEG abnormality was the marked attenuation of background alpha activity. The peak-power frequency and absolute power analysis of three patients showed significant hemispheric asymmetry in the alpha and theta bands respectively. EEG topograms demonstrated persistent theta power fields in one patient. Alpha/theta power ratio was a reliable indicator for the recovery of rhythmic EEG activity during the convalescence. Reorganization of alpha activity appeared to lag behind the clinical improvement, its course was different from patient to patient. Because of the discrepancy of bioelectric and clinical findings the authors recommend long-term EEG monitoring in patients with tick-borne encephalitis.

[Value of the Medicor CT 0100 equipment in a county hospital in the diagnosis of cerebrovascular diseases]. / Megyei kórházban müködö Medicor CT 0100 diagnosztikai jelentösége cerebrovascularis kórképekben.

Data of patients with CVD in the ward of the authors in the last quarters of 1986 and 1987 has been compared, in the second period they used a MEDICOR CT 0100 computer tomograph. The subsequent results are stressed: The rate of the patients with CVD in the Department of Neurology was approximately 40% in the studied period. 88-90% of all lethal cases has been covered by this patients's group. Half of the patients with CVD was between 50-70% years, however younger age was more common among men. The majority of CVD was ischaemic. In vivo diagnosis of intracerebral bleeding has been markedly increased after introduction of CT investigation. CT scan on the first three days is helpful to localise the source of the subarachnoideal bleeding, however can not replace the angiography in the cases indicated for operation. Investigations by angiography have decreased to the one third in the department without neurosurgery after introducing CT however their indication has been changed. In traumatic cases CT is more informative and more sparing. Diagnoses suggested by neurologists have been confirmed by the CT in 69%.

[Value of the carotid compression test for the diagnosis of cerebrovascular disorders]. / Der Wert des Karotis-Kompressions-Tests für die Diagnose zerebraler Durchblutungsstörungen.

The carotid compression tests have been performed in 1940 outpatients and 233 hospitalized patients with clinical signs and symptoms of cerebrovascular disturbances. The frequency of EEG slowing, positivity of the compression tests and carotis sinus hypersensitivity was in patients with local neurological signs: 36%, 22.5%, and 24.8%, while the corresponding numbers for patients without neurological signs were: 24.6%, 21.8%, 14.7%. On the basis of the carotis angiography the following groups of the patients could be formed: Thrombosis of the internal carotid artery (A), thrombosis of the anterior or medial cerebral artery (B), carotis stenosis (C), other vascular pathological signs of the arteries (D), normal angiography (E). They were compared with the control group of patients with vertebrobasilar insufficiency (F). The frequency of EEG slowing (A = 92%, B = 72%, C = 58.9%, D = 57%, E = 40%, F = 22.5%). The percentage of the positivity of carotid compression tests (A = 54%, B = 32%, C = 46%, D = 26%, E = 12%) and occurrence of carotis sinus hypersensitivity (A = 26.8%, B = 16%, C = 17.7%, D = 10.8%, E = 11.3%, F = 6.9%) were proportionate to the pathological morphological signs. The results of the compression tests may give indication for detailed clinical investigation, and are helpful in the estimation of the functional capacity of the carotid arteries in the cerebral circulation.

Correlation between blood pressure and the level of monoamines in the cerebrospinal fluid of patients with cerebral haemorrhage. Case reports.

The correlation between the noradrenalin (NA), dopamine (DA) and serotonin (5-HT) contents of cerebrospinal fluid (CSF) samples obtained from the cerebro-ventricular space and the systolic and diastolic blood pressure (BP) of two patients with cerebral haemorrhage were investigated. Significant correlation was found between CSF NA, CSF DA level and systolic BP in case I. There was no correlation between CSF catecholamine levels and diastolic BP and between serotonin level and BP. Significant correlation was observed in case II between CSF DA level and diastolic BP. There was no correlation between CSF serotonin level and BP. These results suggest that CSF catecholamines might play some role in the regulation of BP.