Ictal headache and visual sensitivity.

Migrainous headache is reported by patients with photosensitive epilepsy, whereas their relatives complain more often about headache than the relatives of patients with other types of epilepsy. We therefore investigated whether headache itself could be an epileptic symptom related to photosensitivity. Four probands with headache and photosensitive epilepsy were selected. Their first-degree family members were studied using video-EEG with extensive intermittent photic stimulation and pattern stimulation. Nine of the 12 subjects (10 female and two male, mean age 30 years, range 14-46 years) proved to be photosensitive with either focal (n = 5) or generalized (n = 4) epileptiform discharges. In two subjects an ictal recording of headache occurred after visual stimulation. We found evidence that, in specific patients, headache could be an ictal sign of epilepsy. Photic stimulation during EEG recording can contribute to correct diagnosis and lead to the best care and management of the patient.

Emerging patterns of diffusion, perfusion-weighted MRI and spectroscopy in creutzfeldt-jacob disease.

Sporadic Creutzfeldt-Jacob disease (CJD) is a transmissible, progressive, fatal spongiform encephalopathy. Routine MR imaging sequences may show abnormalities in the basal ganglia and cerebral cortex. Recently, several reports claimed that diffusion weighted MRI (DWI) could demonstrate early brain lesions in CJD patients when conventional MR images are normal on T2-weighted sequences. We evaluated the usefulness of DWI, perfusion-weighted MRI (PWI) and spectroscopy to confirm the clinical diagnosis and assess lesion progression in two patients with suspected CJD. We noted a diffuse hypoperfusion in the basal ganglia where ADC values were reduced but spectroscopy values were normal. A strong hypoperfusion was observed in the right head of the caudate nucleus in patient n° 2 where spectroscopy values were abnormal. A typical distribution of hypoperfusion followed the posterior progression of disease. We suggest the hypoperfusion in the areas presenting restricted diffusion probably reflects spongiform degeneration and moderate mass effects from cytotoxic edema.

Altered circadian cortisol secretion in Alzheimer's disease: clinical and neuroradiological aspects.

We determined circadian salivary cortisol levels in 18 outpatients affected by probable Alzheimer's disease (AD) and looked for a possible correlation with both cognitive impairment and brain CT scan findings. The diagnosis of probable AD was made according to the NINCDS-ADRDA criteria. The severity of cognitive impairment was quantified using the Mini Mental State Examination (MMSE) and the Global Deterioration Scale (GDS). Cortisol levels were measured on saliva samples collected at 08:00 AM and 08:00 PM. For each sample, a duplicate cortisol measurement was performed on 50 microl of saliva by means of a modified commercial radioimmunoassay kit. At the same time, 11 of the 18 AD patients enrolled also underwent a brain CT scan to estimate cerebral atrophy by using linear indexes. The mean value of cortisol levels was significantly higher in AD patients than in controls at both the morning and the evening measurements, and the circadian fluctuation of cortisol was less marked in AD patients than in controls, although this difference did not reach statistical significance. Morning cortisol levels were significantly correlated to both the MMSE and the GDS scores. A significant correlation was also found between morning cortisol levels and all the cerebral atrophy indexes. By contrast, no correlation was observed between evening cortisol levels or cortisol circadian fluctuations and either cognitive impairment or cerebral atrophy. In conclusion, despite the potential biases deriving from the small sample and the limitations of the CT scan study, our results suggest that, in AD patients, hypercortisolemia is correlated with severity of the disease.

Movement-related electroencephalographic reactivity in Alzheimer disease.

Event-related desynchronization/synchronization (ERD/ERS) of alpha and beta electroencephalographic (EEG) rhythms was investigated in normal subjects and mild Alzheimer Disease patients (AD), performing unilateral right finger movements (about 10 s intermovement interval). Electroencephalographic data were sampled based on 10-20 system electrode montage. Surface Laplacian estimate of the potential reduced the head-volume conductor effects and annulled electrode reference variations. Results showed that EEG reactivity (i.e., ERD/ERS) of modeled contralateral rolandic cortex and motor performance were preserved in mild to moderate AD. In contrast, modeled activity (i.e., ERD/ERS) of frontolateral, centromedial, and ipsilateral rolandic areas was abnormal. Furthermore, interrelatedness of cortical response and movement timing was abnormal in AD patients. These results would support the working hypothesis that mild to moderate AD is a global brain network disease, including processing of sensorimotor information (despite no overt movement disorder). Further investigations will ascertain the clinical relevance of these results.

Preserved blood pressure and heart rate circadian rhythm in early stage Alzheimer's disease.

BACKGROUND: This study investigated the blood pressure (BP) values over the day-night period in 11 noninstitutionalized patients affected by probable Alzheimer's disease (AD) in its early stage. The scientific aim was to detect whether the BP circadian rhythm (CR) was preserved, given the fact that CR disruption was observed in advanced or institutionalized AD patients. METHODS: The BP within-day values were gathered via noninvasive ambulatory monitoring. The BP time series were analyzed according to the chronobiological procedure, called Cosinor method with three harmonic components. RESULTS: The biometric analysis was able to document that BP changes over the 24-h scale in AD patients as a function of a significant CR. Such a preserved circadian regulation is, however, compromised in the second and third harmonic component, suggesting that the BP within-day variability is desynchronized by the environmental clues that act as synchronizers during the diurnal part of the day. CONCLUSIONS: The preservation of the BP CR in the early stage of AD suggests using such a finding as a clinical tool for confirming the recent onset of the disease. As a matter of fact, it is presumed that the disease is not evolved enough to reach the suprachiasmatic nuclei, wherein is located the BP circadian pacemaker. The abolition of the ultradian components is another precocious sign that, in turn, indicates early-stage AD patients to be particularly compromised in their synchronization to diurnal cues, such as social routines, meal timing schedule, psycho-physical activity, and occupational schemes.

Autonomic nervous activity during sleep in middle cerebral artery infarction.

No data are available on the autonomic system during sleep in patients with stroke. The purpose of this study was to determine the influence of acute ischemic stroke on the autonomic cardiovascular system during sleep, and to correlate autonomic activity with the clinical status of patients. Ten patients with ischemic stroke in the middle cerebral artery were studied by means of an all-night polysomnographic recording within the 1st week of the onset of symptoms and at the 3-week follow-up examination. Power spectrum analysis of the heart rate variability was performed using an autoregressive algorithm in 180 consecutive electrocardiographic RR intervals. Spectral power was calculated in 3 main frequency bands: high frequency (HF), 0.15-0.4 Hz; low frequency (LF), 0.04-0.15 Hz; very low frequency (VLF), < 0.04 Hz. The data were compared with those of 10 age-matched controls. A significant increase in VLF (p < 0.0005) and a decrease in HF (p < 0.0002) components were found in ischemic stroke patients. The sympathetic-parasympathetic balance (VLF + LF/HF) was higher in patients than controls (p < 0.005). However, these components changed significantly during sleep, revealing a physiological pattern. These power spectral data were still present at the 3-week follow-up. The 4 patients who developed cardiac arrhythmias showed higher sympathetic-parasympathetic balance than patients without arrhythmias (p < 0.05). These data suggest a sympathetic predominance in patients with acute ischemic stroke during sleep. However, the flexible and dynamic properties of the autonomic nervous system are preserved. Cardiac arrhythmias following stroke may be related to the degree of sympathetic predominance.

Cardiac autonomic dysfunction in patients with Alzheimer disease: possible pathogenetic mechanisms.

We studied a possible correlation between autonomic cardiac activity and the level of the red blood cell acetylcholinesterase (AChE) in patients with probable Alzheimer disease (AD). The influence of cholinesterase inhibitor treatment on this autonomic activity was evaluated. Twelve patients satisfying the NINCDS-ADRDA criteria of probable AD and 10 healthy controls were studied. Autonomic cardiac activity was evaluated by means of power spectral analysis (PSA) of heart rate variability (HRV) using an autoregressive algorithm on 250 consecutive electrocardiographic R-R intervals. All patients received oral eptastigmine, a new cholinesterase inhibitor, for 1 month. Before treatment, a simultaneous recording of the electrocardiographic and respiratory activities was performed at rest and subsequently during head-up tilt test at 700. Recording was repeated on the last day of treatment. The level of AChE activity during each recording was also evaluated. Spectrum power was calculated in three main frequency bands: high frequency (HF), 0.15-0.4 Hz; low frequency (LF), 0.04-0.15 Hz; very low frequency (VLF), <0.04 Hz. In addition, we calculated the total spectrum power (TSP) and the LF/HF ratio. The TSP and the absolute value of each spectral component were significantly lower in AD patients than in controls. In contrast with controls, AD patients did not show any significant change before treatment in either the LF and HF components or in the LF/HF ratio during the tilt test. However, the modification in the LF component, induced by tilting, showed a significant correlation with the level of AChE activity (p < 0.03). During the tilt test, the treatment caused changes in LF and HF components and in the LF/HF ratio similar to those observed in controls. These results suggest that the presence of autonomic cardiac dysfunction in AD patients might be due to a cholinergic deficit in the peripheral autonomic nervous system. Some aspects of this autonomic dysfunction can be normalized by cholinesterase inhibitor treatment.

Four-year follow-up of a case of acute multiple sclerosis of the Marburg type.

We describe the clinical course, treatment and magnetic resonance findings during a four-year follow-up of a patient with acute multiple sclerosis of the Marburg type, treated with steroid and mannitol therapy, who survived the first bout. Although the course of the disease was clinically indistinguishable from relapsing-remitting multiple sclerosis, our case was characterized by peculiar features. These included the need for protracted steroid therapy and the remarkable tendency of MRI lesions to resolve completely.

Quantitative magnetic resonance analysis in vascular dementia.

The potential role of magnetic resonance imaging (MRI) in differentiating between specific causes of cognitive decline in patients with vascular dementia (VD) has not yet been fully established. We therefore decided to assess the supratentorial cerebral contents in 24 patients with a diagnosis of probable VD and in 24 normal subjects, matched for age and education level, using MRI volumetric parameters obtained by means of a quantitative method. The volumes of subarachnoid and ventricular spaces, cerebral tissue, and hyperintense areas on T2-weighted images were calculated. In order to reduce interindividual variability caused by differences in intracranial size, each absolute measurement was normalized to the relative size of the intracranial volume. In addition, we calculated the ratio between the areas of the corpus callosum (CC) and supratentorial brain at the same level on the T1-weighted image midsagittal plane. The MRI data were correlated with the deterioration of cognitive functions. Patients with VD showed significantly lower cerebral tissue volume and CC area, and higher ventricular space volume than normal subjects. Furthermore, the total volume of the T2 signal alterations was higher in VD patients than in normal subjects. In VD patients, this volume was found to be proportional to the increase in the volume of the ventricular space. On the other hand, no correlation was found between the volume of the T2 signal alterations and the area of the CC. The degree of global cognitive dysfunction and the score of each neuropsychological test did not show any correlation with the MRI data. Our results suggest that ventricular enlargement in VD patients is correlated with the increase in volume of the T2 signal abnormalities, but that the degree of global cognitive dysfunction is not influenced by the volume of these T2 signal abnormalities. Furthermore, the CC atrophy does not influence the score of any neuropsychological test or the degree of global cognitive dysfunction.