CT and MR imaging of neuroaxonal leukodystrophy presenting as early-onset frontal dementia.

CT and MR imaging showed diffuse changes of the frontal white matter and genu of the corpus callosum with minimal atrophy and no contrast enhancement in a 41-year-old woman with progressive dementia. Brain biopsy disclosed axonal spheroids and gliosis in the white matter without macrophage or inflammatory infiltration or vessel abnormalities consistent with neuroaxonal leukodystrophy. This disease can be suspected on CT and MR imaging findings but requires neuropathologic examination to be diagnosed.

Smooth-pursuit eye movement and saccadic intrusions in obsessive-compulsive disorder.

Although several reports agree that smooth-pursuit eye movement (SPEM) is abnormal in some obsessive-compulsive disordered (OCD) patients, differences between treatments and lack of accuracy in control selection make the results controversial. Although reduced gain seems the most accepted abnormality, the characteristics of saccadic disruption of smooth pursuit are as yet unspecified. SPEMs in 21 OCD patients (DSM-III-R) and 21 healthy subjects recruited from the community were studied through a multiple target velocity task . The two groups were individually matched on age, gender, and level of education. None of the subjects had a history of substance dependence apart from the smokers who refrained from smoking in the 2 hours prior to the test. A significantly lower SPEM gain and increased number and frequency of anticipatory saccades (ASs) was found in OCD patients as compared with control subjects. No relationship emerged between eye movement abnormalities and clinical variables explored.

Acute changes of blood ammonia may predict short-term adverse effects of valproic acid.

Valproic acid (VPA) was given to 24 epileptic patients who were already being treated with other antiepileptic drugs. A standardized loading dose of VPA was administered, and venous blood was sampled at 0, 1, 2, 3, and 4 hours. Ammonia (NH3) was higher in patients who, during continuous therapy, complained of drowsiness (7 patients) than in those who were symptom-free (17 patients), although VPA plasma levels were similar in both groups. By measuring VPA-induced changes of blood NH3 content, it may be possible to identify patients at higher risk of obtundation when VPA is given chronically.

Cognitive potentials: ipsilateral corticocortical interconnections in prefrontal human cortex ablations.

The research deals with the possible role of the essentially monosynaptic bidirectional corticocortical connections between occipito-temporo-parietal association cortical areas and frontal areas in the genesis of some contingent negative variation (CNV) components, especially on the supramodal dorsolateral prefrontal regions. With standard and topographic mapping methods of analysis, the multicomponent CNV complex formation was examined in 7 patients with extensive frontal cortex ablations exactly identified through CT/MRI examinations, and in 10 normal subjects. On the scalp over the ablated frontocortical areas, no consistent post-warning auditory N100 a-b-c, P200, P300, early and late CNV components were recordable. The hypothesis is proposed that the bidirectional ipsilateral long-distance pathways which interconnect uni-polymodal occipito-temporo-parietal cortical areas to prefrontal ones, in particular the arcuate-superior longitudinal and superior/inferior occipito-frontal fasciculi, play an important role in the genesis of several CNV complex components, especially the multicomponent post-S1 auditory N100. The posteroanterior sequential latency differences of these neurocognitive components, roughly measured along the scalp or on MRI imagings, is probably accounted for by the transcortical ipsilateral conduction time of about 1 cm/ms (10 m/s).

Fast multiphase MR imaging of aqueductal CSF flow: 2. Study in patients with hydrocephalus.

The signal intensity in the region corresponding to the cerebral aqueduct was evaluated in three patients with noncommunicating tension hydrocephalus (caused by aqueductal obstruction in two and type I Arnold-Chiari malformation in the other), seven patients with suspected normal-pressure hydrocephalus (three of whom subsequently underwent successful shunting), and 10 patients with ex vacuo (atrophic) hydrocephalus. A gradient-echo MR sequence, called fast multiphase imaging, was used. Serial images corresponding to different phases of the cardiac cycle were acquired. No flow-related enhancement was observed over the entire cardiac cycle in the patients with noncommunicating hydrocephalus. Patients with normal-pressure hydrocephalus showed a higher aqueductal CSF signal intensity, consistent with increased systolic flow rates, than patients with ex vacuo hydrocephalus. When comparing the above two groups of patients with a control group of healthy volunteers, significantly higher and lower values of the (mean) maximum aqueductal signal intensity were found in the normal-pressure hydrocephalus patients and the ex vacuo hydrocephalus patients, respectively. Fast multiphase MR evaluation of aqueductal CSF flow may help to differentiate patients with different types of hydrocephalus.

Smooth-pursuit eye movements: alterations in Alzheimer's disease.

Smooth-pursuit eye movements induced by targets moving at constant velocities (from 5 to 100 deg/sec) were recorded from 13 patients with Alzheimer's disease (AD) and from 11 healthy subjects. Four variables were evaluated to quantify the patients' response to the eye movement tests: (1) average peak velocity of smooth-pursuit; (2) percent target matching index after saccade removal (percent ratio between the area of the velocity curve of smooth-pursuit eye movement after saccade removal and the area of target velocity) which is related to the eye performance for each value of target velocity; (3) total amplitude of anticipatory saccades; (4) total number of anticipatory saccades. Compared to the controls, AD patients were found to have significantly lower values of average peak velocity of smooth pursuit and of percent target matching index and a significantly increased number and amplitude of anticipatory saccades. A discriminant stepwise analysis indicated that 5 oculographic variables were significantly associated with the patient's clinical condition (healthy volunteer or AD patient). These statistics yielded an equation for predicting the patient's status according to which the percentage of cases classified correctly was 82.6% in the overall group (n = 23). The predictive performance was similar between the healthy volunteers subgroup (81.8%, n = 11) and the AD subgroup (83.3%, n = 12). The discriminant score was significantly correlated with the score resulting from the MiniMental test (r = 0.67). A significant correlation was also found between the MiniMental score and the number of anticipatory saccades (r = -0.61). No significant correlation was present between the gain of smooth pursuit and the patients' cognitive decline.(ABSTRACT TRUNCATED AT 250 WORDS)

Spinal intramedullary subependymomas: case report and review of the literature.

A cervical spinal intramedullary subependymoma in a 53-year-old man is reported, and the relevant literature is reviewed. Spinal cord subependymomas seem to follow a benign course. Radiotherapy should not be administered to these patients. Magnetic resonance imaging, even with enhancement, is not able to distinguish between a subependymoma and the more common ependymoma.

Eye movement abnormalities in anorexia nervosa.

The aim of the present study is to investigate smooth pursuit eye movement and saccadic performance in anorexia nervosa during a restored weight period and to determine if functional links can be made between eye movement performance and clinical features. SPEM parameters were recorded for 28 female anorectic out-patients (DSM IV), who had a body weight loss of up to 20% of ideal body weight. Twenty-eight comparison subjects were also tested. Clinically, each patient was assessed using the Eating Disorder Inventory (EDI), the Yale-Brown Obsessive-Compulsive Scale (Y-BOCS), the Structured Interview for Personality Disorders (SCID II), the Symptom Checklist-90-Revised (SCL-90-R) and the Hamilton Scale for Depression (HRSD). The anorectic patients performed slightly worse than the comparison subjects on a number of SPEM measures. No relationship was found between SPEM impairment and a global severity index of psychopathology (SCL 90-R GSI) or depressive symptoms. Moreover, OCD symptoms and scores on some EDI scales (such as perfectionism) appear related to the severity of the eye movement alterations. The evidence of SPEM abnormalities in a subgroup of anorectic patients during the remitted state and the relationship of the abnormalities to obsessive-compulsive symptoms are discussed. Results are in agreement with the hypothesis regarding the persistence of neurophysiological as well as psychopathological traits of disorder in anorectic patients.

Reversal of syphilitic hydrocephalus with intravenous penicillin.

BACKGROUND: CSF shunting procedures are generally considered the fundamental therapy of syphilitic hydrocephalus. METHODS: We followed up with CSF analysis and MR imaging a patient with progressive mental and gait disturbances and tetraventricular hydrocephalus due to tertiary syphilis who was treated for 14 days with high dose intravenous penicillin alone. RESULTS: Clinical and CSF abnormalities resolved within a few months, whereas the hydrocephalus disappeared only 30 months after therapy. CONCLUSIONS: Before consideration of a CSF shunting procedure, a trial of high dose intravenous penicillin is warranted for patients with syphilitic hydrocephalus.

Changes in bit-mapped contingent negative variation (CNV) activity due to initial normal involutional processes of the human brain.

Bit-color mapped multicomponent CNV complexes and RTs to S2 evoked with a simple warned CNV/RT paradigm were recorded and measured in 20 selected right-handed very healthy volunteers (10 young adults and 10 presenile subjects, mean age 28.3 and 59.6, respectively). EEG and CNV components (post S1, N1, P2, P3; early CNV; N1200; late CNV; CNV resolution) were recorded from Fz, C3, Cz, C4, P3, Pz, and P4 referenced to linked mastoid electrodes. EOG, RT and stimuli were also recorded. The presenile group differed significantly from the younger group in the auditory post-S1 N1 and early (O-wave) and late (P-wave) CNV complex components. A progressive amplitude reduction limited to frontal leads between O-wave and P-wave, the lowest point being reached in the P-wave, was characteristic in the presenile group. Moreover, presenile subjects showed relatively flat CNV waveshapes of low amplitude and, on the whole, performed a little less well than young ones. This finding suggests that the statistically significant changes in auditory post-S1 N1 and CNV activity recorded in our presenile subjects, without any appreciable deficits in behavioral or mental performance, could be alerting signs of early brain involutional processes related to minimal and subclinical decline in orienting, attentiveness and response preparation capabilities. If such is the case, and it could be confirmed in a larger sample of very healthy subjects, these age-related changes in the presenium might prove to be of considerable practical importance for clinical research.

Topographic CNV activity mapping, presenile mild primary cognitive decline and Alzheimer-type dementia.

The CNV complex evoked with a standard paradigm (S1-2 sec-S2-motor response) and reaction time (RT) to the imperative signal (S2) were recorded and measured in 12 patients with initial presenile idiopathic cognitive decline (PICD), 12 with presenile Alzheimer-type dementia (PAD) and 10 healthy age-matched controls. Significant group differences were obtained for measures of some CNV components, particularly of the late pre-S2 CNV. No significant CNV activity, very prolonged RTs and sometimes characteristic post-imperative negative variations (PINV) were observed in the majority of patients with PAD. These results suggest that similar CNV complex and RT changes to those observed in our patients may constitute a valuable clue in the study of pathophysiological brain functioning in the early stages of presenile idiopathic mental deterioration.

Conversion from thrice daily to twice daily administration of gabapentin (GBP) in partial epilepsy: analysis of clinical efficacy and plasma levels.

Gabapentin has been administered in placebo-controlled studies with a thrice daily (T.I.D.) schedule, because of its short half-life. However, clinical efficacy does not seem strictly related to plasma levels: a twice daily (B.I.D.) schedule might therefore be possible. The aim of our study was to verify if the conversion from a T.I.D. to a B.I.D. regimen affected the efficacy and safety of gabapentin therapy. Out of 171 patients treated with add-on gabapentin, we selected 29 stable responders, who were followed for three months with a T.I.D. schedule and then switched to B.I.D. regimen for further three months. Seizure number, side-effects and trough plasma levels of gabapentin were collected during both periods. Gabapentin mean dose was 2117.2 mg/day. Mean number of seizures/months was: 4.2 at baseline, 1.0 during the T.I.D., and 0.9 during the B.I.D. period. Mean trough plasma level of gabapentin was 5.9 microgram/ml during the T.I.D. and 5.2 microgram/ml during the B.I.D. period. Twelve side-effects were reported by 11 patients during the T.I.D. and 6 by 5 patients during the B.I.D. period., sedation and vertigo were the most frequent in both. Results of our study suggest that gabapentin can be administered safely and effectively either with a T.I.D. and a B.I.D. regimen.

Continuous-wave Doppler of vertebral arteries in noninvasive diagnosis and management of vertebro-basilar TIAs.

Continuous-wave Doppler and angiography of the vertebro-basilar system were used in 38 patients with vertebro-basilar transient ischemic attacks. 63 vertebral arteries were studied. Among 47 normal Doppler parameters, 46 were confirmed by angiography. The sonographic diagnostic procedure showed a very high negative predictive value (97.87%), while the positive predictive value was relatively low (56.25%). Data from literature were reviewed. The authors suggest that when Doppler of vertebral arteries is negative, there is a very low probability that a follow-up angiography will reveal pathologic conditions calling for a surgical approach.

Sonography of vertebral arteries in De Kleyn's position in subjects and in patients with vertebrobasilar transient ischemic attacks.

Two groups of subjects, matched for age, were studied. The first group consisted of 190 healthy subjects, the second, of 60 patients with vertebrobasilar transient ischemic attacks (TIAs), 22 of whom underwent angiography. After it was ascertained that the findings from continuous-wave (cw) Doppler of carotid and vertebral arteries, performed in the standard position, were normal, the examination was then done in De Kleyn's position, the velocity signal being detected at the mastoidal slopes. The abnormal findings were classified into two groups: "loss of diastolic velocity signal" and "absence of velocity signal." In the control group, changes of flow were detected in 6.31%, whereas in the group of patients abnormal Doppler parameters were detected in 33.33%. None of the patients who were submitted to angiography showed abnormal hemodynamic findings. The authors suggest that the detection of the velocity signal of the vertebral arteries in De Kleyn's position could be of help in revealing conditions that could cause, in time, signs and/or symptoms of vertebrobasilar insufficiency in subjects with possible asymptomatic anomalies of the circle of Willis.

Hemostatic Balance Index in TIA patients: sex-related changes.

In order to evaluate the occurrence of hemostatic disorders, 37 patients with transient ischemic attacks (TIAs) and 50 control subjects were studied by means of the Hemostatic Balance Index (H.B.I.) derived from Raby's Thrombodynamic Potential Index (T.P.I.) and Fearnley's Whole Blood Diluted Lysis Time (W.B.D.L.T.). Results showed a significant increase in T.P.I. and a tendency to a decrease in fibrinolytic activity in the TIA group: H.B.I. was shown to be significantly increased, thus indicating a pro-thrombotic imbalance in these patients. The occurrence of similar changes in TIA females when compared to male patients marks the importance of plasmatic factors in the mechanism of thrombotic disorders in females with cerebrovascular disease.

The Valsalva maneuver in vasodepressor syncope.

In this work the authors propose the Valsalva maneuver as a diagnostic test in a group of patients shown, by anamnestic, physical, and instrumental criteria, to be affected by vasodepressor syncope. They studied the response of heart rate and baseline arterial pressure to the maneuver performed in passive orthostasis in 7 healthy volunteers and 24 patients. These patients showed a hypotensive response and a time of recovery to the initial values that was significantly longer than in the control group (p less than 0.01). The authors conclude that studying the response to the Valsalva maneuver can be useful in the diagnosis of vasodepressor syncope.