Malignancy and sensory neuropathy of unexplained cause: a prospective study of 51 patients.

OBJECTIVE: To investigate the frequency of cancer developing in patients with peripheral sensory neuropathy of unexplained cause. DESIGN: Prospective study. SETTING: A neurologic unit in a general hospital. METHODS: Following the diagnosis of neuropathy, we searched for occult malignancy. This search was repeated together with neurologic evaluations every 6 months thereafter. Patient recruitment began January 1, 1988, and ended December 31, 1995. The end point of the study was December 31, 1996. RESULTS: In the study period, we observed 363 patients with peripheral sensory neuropathy. Of these, 53 patients without any identified cause of neuropathy were invited to participate in the study. Of the 53, 2 patients refused. Thus, we examined and followed up 51 patients, 42 men and 9 women, with a mean age of 64.5 years (range, 19-80 years). The range between the onset of neurologic symptoms and the diagnosis of neuropathy was 2 to 72 months (mean, 13.9 months). The follow-up period ranged from 14 to 94 months (mean, 51.4 months). In 18 patients (35.3%) (16 men and 2 women) whose mean age at diagnosis of neuropathy was 66.5 years. malignant growths were found 3 to 72 months (mean, 27.4 months) after the onset of the neuropathy. The cancer was in the liver in 4 patients (all had a primary hepatoma), the bladder in 3, the lymph nodes in 3 (all with non-Hodgkin lymphoma), the prostate gland in 2, the lungs in 2 (small cell lung cancer in both), the breast in 1, the pancreas in 1, the sublingual gland in 1, and the bone in 1 (a metastatic sarcoma). CONCLUSIONS: More than one third of the patients with peripheral sensory neuropathy of unexplained cause developed cancer without any predominating type of malignancy.

Immediate anticoagulation with heparin for first-ever ischemic stroke in the carotid artery territories observed within 5 hours of onset.

OBJECTIVE: To evaluate the safety and possible efficacy of heparin sodium anticoagulation within 5 hours of the onset of first-ever nonlacunar ischemic strokes in the internal carotid artery territories. DESIGN: Pilot study, prospective and open. SETTING: Inpatient stroke unit. PATIENTS: Of 360 stroke patients observed during 13 months, 45 (12.5%) were included in the study. INTERVENTIONS: Heparin sodium was administered intravenously, starting with a bolus of 10,000 U, followed by continuous infusion over 4 days at a rate adjusted to maintain an activated partial thromboplastin time ratio between 2 and 2.5. The mean interval from stroke to treatment was 197 minutes. RESULTS: Two patients had cerebral hemorrhage, one of which was fatal. None had extracranial major bleeding, while six had minor bleeding. The conditions of 23 patients improved, 16 patients were stable, and six patients worsened by day 1, while 29 patients improved, eight patients were stable, and eight patients worsened by day 7. Six patients died by the first month and five more by the sixth month. Twenty-one patients were self-sufficient, both at 1 and 6 months. Hemorrhagic complications were unrelated to any investigated factor. Multivariate analysis indicated that short-term outcome was predicted only by infarct size (P < .0001) and long-term outcome by infarct size (P = .002) and large vessel status (P = .0235). CONCLUSIONS: Our study suggests that immediate heparin treatment for ischemic carotid stroke is feasible and generally safe and that patients whose conditions improve are those with smaller infarct size and no evidence of large vessel obstruction.

An analysis of the costs of ischemic stroke in an Italian stroke unit.

OBJECTIVE: To determine the direct costs of hospital care of acute ischemic stroke in a large Italian hospital, and to identify the main components of such costs. BACKGROUND: Cost containment in stroke care requires an up-to-date assessment of expenditures in the different areas of stroke management. However, costs may vary among countries because of different health system organizations. METHODS: All patients with ischemic stroke admitted during 1996 were considered. Total cost was the sum of a daily component, reflecting personnel wages and general care, and an ancillary component, reflecting mostly investigations and treatments. The real costs were used, not fixed charges. RESULTS: We included 245 patients, with a mean length of stay (LOS) of 13.1+/-7.0 days, and an in-hospital case fatality rate of 8.2%. The mean total cost per patient was 5,087,000+/-2,536,000 Italian Lira (LIT; $3,289+/-$1,640), with a mean cost per day of 388,000 LIT ($251). Approximately 80% of total costs were due to the daily component and 20% to the ancillary component. A multiple linear regression model of length of stay, which determines the daily cost, showed that the Rankin score at entry, the clinical syndrome type, and the destination at discharge independently contributed to LOS. A second linear regression model showed that younger age and longer LOS significantly increased ancillary costs. CONCLUSIONS: The containment of hospital costs of ischemic stroke may be achieved mostly through measures that reduce LOS, such as effective treatments and a quicker deployment.

Neuropsychological profile in myotonic dystrophy.

Twenty patients with myotonic dystrophy underwent neuropsychological evaluation. Performances were analysed with respect to general cognitive profile, family patterns of cognitive impairment, relation with sex, age, extent of muscular involvement, and sex of affected parent. Results showed severe intellectual deficit in 50% of patients and selective impairment of visuospatial and constructional functions. Female patients showed significantly worse global intellectual status than males. No difference in intellectual level was observed in patients with respect to age, extent of muscular involvement and sex of affected parent. No family pattern of cognitive impairment could be identified. Our results show that an extensive neuropsychological battery can reveal the existence of selective mental impairment. It may provide further data on cognitive impairment onset, progression and relation to muscular involvement.

Evolution of neuropsychological changes after partial callosotomy in intractable epilepsy.

Early neuropsychological changes following partial anterior callosotomy were evaluated in 15 patients with epilepsy (10 secondary generalized and 5 Lennox-Gastaut syndromes) by comparing their performances 1 month before surgery and then 15-20 and 90-100 days postoperatively without modifying the anticonvulsant treatment. The following neuropsychological and motor functions were tested: memory, attention, visuo-motor ability, posture, motor dexterity, language, praxis and gnosis. Social behaviour was also investigated. The main results are as follows: (i) most cognitive functions showed no significant variation; (ii) motor organization was still slightly impaired at the second check-up after the surgery; (iii) improvements in social behaviour and posture were frequently observed at the final evaluation. The analysis of individual cases highlights the influence of the extent of commissurotomy, lesions related to the surgical procedure and preoperative brain damage in determining the post-surgical profile. On this basis, the short-term neuropsychological cost of partial callosotomy appears to be low and seems to depend mostly on surgical parameters and brain conditions before the operation.

Comparative study of terfenadine and cetirizine in hay fever: assessment of efficacy and central nervous system effects.

A daily dose of either terfenadine 120 mg or cetirizine 10 mg was compared in two parallel groups of patients suffering from hay fever. According to a double-blind, double-dummy, randomized design, 28 patients were treated with one of the two drugs once daily in the morning for 2 weeks during the 1990 grass pollen season. The severity of nasal congestion, rhinorrhea, sneezing, nasopharyngeal itching and itchy, watery, red eyes was evaluated by the investigator after a 1-week run-in period and at the end of the treatment. The patients made a daily record of the severity of symptoms on a diary card. In addition, drug-related central nervous system (CNS) effects were assessed at baseline and at the end of the treatment by neuropsychological tests aimed at investigating selective and sustained attention, visuomotor abilities and anxiety, and by quantitative, bit-mapped EEG. Both terfenadine and cetirizine produced a significant improvement in symptoms at endpoint without any significant difference between the two drugs. Drowsiness was referred by one patient in each treatment group. No significant impairment of psychomotor performance occurred with either drug. Quantitative EEG showed a significant power increase in the relative (%) delta band in both groups of treated patients. Although the difference was not statistically significant, a tendency towards greater involvement of the CNS was observed with the use of cetirizine. In conclusion, the results of this study confirm that terfenadine and cetirizine are equally effective in the management of hay fever. Some differentiated untoward EEG changes were also observed in relation to the drugs used, without any variation in neuropsychological performance.

Peripheral neuropathy in myotonic dystrophy: electrophysiological and clinical features.

Peripheral neuropathy was investigated in thirty-one patients with myotonic dystrophy (MyD) and sixteen relatives. Using standard electrophysiological criteria, a sensorimotor axonal peripheral neuropathy was found in 14 MyD cases (45%) and not in unaffected first-degree relatives. The whole group of the MyD patients showed significant impairment of mean motor and sensory conduction values, compared with controls. The presence of polyneuropathy was correlated with the patients' age and the severity and duration of the clinical manifestations of MyD.

[Remarks on callosotomy in the treatment of drug-resistant epilepsy]. / Remarques sur la callosotomie dans le traitement de l'épilepsie pharmaco-résistante.

In this report 17 patients with long-standing non-focal epilepsy underwent callosotomy (this was total in two patients and performed in two stages, and anterior-subtotal in the remaining patients). In all patients the atonic-hypertonic seizures with sudden falls were the most disabling epileptic fits. Callosotomy proved efficient in controlling atonic fits in 10 out of 15 patients in whom surgical results are evaluated. In 3 additional patients the frequency of atonic fits was reduced by more than 50%. In the remaining two patients, no therapeutic effect was observed. Callosotomy was less effective on seizures which were not atonic. Therefore, this procedure appears to be indicated in patients in whom atonic fits are predominant. The main effect of callosotomy is to transform drug-resistant seizures into drug-sensitive ones. Neuropsychological sequels are insignificant unless the splenium is severed. However, considerable psychic and behavioral improvement was nearly always observed after surgery. Despite the fact that on a therapeutic level results were often satisfactory, a number of practical problems still remain. These concern the full spectrum of indications for callosotomy, the extent of corpus callosum section, choice of methods in severely mentally retarded patients and, finally, the age at which the operation should be carried out.

Plasma homocysteine and severe white matter disease.

The objective of this study was to assess if high total plasma homocysteine (tHcy) levels are a risk factor for severe leukoaraiosis (LA). This case-control study was done in a primary care neurology ward and included 178 consecutive patients. Patients with severe LA at CT scan were compared with patients without any LA regarding age, cerebrovascular risk factors, tHcy, vitamin B12, folate, creatinine levels and dementia. Multivariate logistic regression was used to find variables independently associated with severe LA. Age (odds ratio [OR], 1.10 per year; p<0.0001), tHcy (OR, 1.07/micromol/l increase; p=0.045) and hypertension (OR, 2.97; p=0.007) were significantly associated with severe LA. Total homocysteine levels are associated with severe LA independently of other risk factors for cerebrovascular disease. This may suggest that decreasing tHcy may help preserve the integrity of the brain white matter.

Unusual findings in Creutzfeldt-Jakob disease. Case report.

We present a definite case of Creutzfeldt-Jakob disease (CJD) with visual evoked response (VER) and CSF oligoclonal band abnormalities and discuss the neurobiological significance and diagnostic value of these findings.

Reversible multifocal encephalopathy following tuberculous infection.

Tuberculous encephalic infection is commonly reported as confined to Asians or Africans or people living in poor hygienic conditions; very often it follows meningitis in patients with lung TB infection. We describe three western patients coming from good social environment and suffering from multifocal tuberculous encephalopathy. Two of them showed neither meningitis or lung TB when CNS involvement appeared. Complete recovery after therapy is described, together with the evolution of brain CT and, in 1 case, MRI features. The instrumental findings accompanying the complete recovery suggest that the lesions described in these cases are a localized form of encephalitis responsive to medical treatment, unlike tuberculomas, which often need surgical treatment. The occurrence of tuberculous encephalic infection in western, middle-class patients with or without meningitis emphasizes that tuberculous encephalopathy must be considered in the differential diagnosis of multifocal brain lesions.

May preclinical markers of brain infection by HIV be detected? A cognitive, CSF and neuroradiological investigation.

A group of patients with HIV infection in various stages of the disease was studied with regard to CSF, neuroradiological and neuropsychological aspects. A considerable number of them showed signs of CNS involvement, as revealed by abnormalities in all the three fields investigated, despite a frequently unremarkable neurological examination. The findings of CSF alterations, neuroradiological abnormalities and selective cognitive impairment in the absence of opportunistic infections of the CNS support the hypothesis of an early and direct action of HIV on the nervous system.

Length of hospitalization of patients with transient ischemic attacks.

OBJECTIVE: To investigate factors that influence length of stay (LOS) in patients hospitalized for transient ischemic attacks (TIAs). This may help to reduce unnecessary days of hospitalization. DESIGN: Retrospective study. SETTING: A large, non-academic general hospital. PATIENTS: All patients hospitalized for TIAs during the years 1996-1998 were retrospectively studied. We analyzed the relationship between demographic, clinical and management features and LOS. RESULTS: We studied 157 patients. Median LOS was 8 days (range, 1 to 21 days). Statistical analysis showed that female sex, being hospitalized in 1996, hospitalization from Wednesday through Saturday, and second-level cardiological, neuroradiological and miscellaneous investigations significantly increased LOS. CONCLUSIONS: The LOS of patients with TIAs may be reduced through measures that accelerate the diagnostic work-up and by scheduling the admission to the first days of the week, when this is possibile.

Spontaneous dissection of carotid and coronary arteries.

The authors report a case of spontaneous dissection of both carotid arteries, followed by spontaneous dissection of the left anterior descending coronary artery after a few days. No major abnormalities of collagen production were found. This case may be underlain by a dysplasia of common precursors of the carotid and coronary walls.

Commissurotomy in intractable epilepsy: clinical and surgical comment.

Two patients suffering from seizures unresponsive to medical treatment underwent two-stage "central" commissurotomy. In one patient anterior callostomy was carried out first; in the other the surgical procedure was performed in the reverse order. The clinical, EEG, and neuropsychological features of the two patients are reported before the operation, between stage 1 and stage 2 of the procedure, and after completion of the commissurotomy. The surgical aspects of the two different procedures and the related clinical pictures are described in detail.

Recognition impairment correlated with short bisynchronous epileptic discharges.

The occurrence of transitory cognitive impairment during diffuse subclinical electroencephalographic (EEG) discharges has been widely documented but the role of the parameters influencing the cognitive performance and the involvement of motor or verbal response in the tasks used is still under debate. Fifteen patients suffering from primary generalized epilepsy with frequent bisynchronous EEG epileptic bursts underwent a shape recognition task during EEG monitoring. The test sequence was as follows: memorandum, pause, and multiple choice set. After pressing the response button, the patient was asked to confirm the choice verbally. The following parameters were considered: geometrical complexity of the shape, chronological position of the burst occurring during the single test, and the duration of discharge ranging from 1 to 3 s. Results showed a significant increase in incorrect responses during the test when discharges occurred, with more errors occurring for difficult than for easy shapes. Neither the discharge position nor the duration of the epileptic burst influenced the performance. Diffuse epileptic activity of short duration produced selective effects on the cognitive process regardless of the motor component of the response.

Large demyelinating brain lesion mimicking a herniating tumor.

We describe the case of a large brain lesion whose computed tomography appearance and clinical evolution mimicked a herniating tumor. The patient progressed to coma within 6 days of hospitalization despite high-dose steroid treatment. Emergency excision of the lesion was carried out. Histological analysis showed massive demyelination, axon preservation and no tumor cells. No lesion recurrence was seen during a 55-month follow-up. Recognition of such lesions through magnetic resonance imaging or spectroscopy may spare unnecessary surgery or biopsy. However, our case shows that such lesions may still require resection in the face of a rapid clinical progression and poor response to medical treatment.

Functions of the corpus callosum: observations from callosotomy performed for intractable epilepsy.

The effects of complete and partial corpus callosotomy in 6 patients are reported. Only the 2 cases undergoing total callosotomy showed evidence of impaired interhemispheric sensory transfer, related to sectioning of the splenium. Only mild long-lasting neuropsychological deficits were detected. Post-commissurotomy mutism and akinesia appeared in 4 cases, 2 with total, and 2 with partial anterior callosotomy. The short-and long-term effects of corpus callosotomy appear to be related to the extent of the section the creation of lesions during the surgical procedure, and a peculiar organization of cognitive functions in chronic epileptic patients.

Prognostic use of ultrasonography in acute non-hemorrhagic carotid stroke.

In order to evaluate whether the results of ultrasound examination may be associated with 30-day outcome, 76 consecutive patients (43 men and 33 women; mean age 68.1 +/- 8.9 years) underwent duplex scanning of the carotid bifurcations and transcranial doppler investigation of the basal skull arteries within the first few hours of the onset of an acute carotid stroke. Forty-three patients (56.6%) had appropriate arterial occlusion at ultrasounds examination. On day 30, 22 patients (28.9%) were self-sufficient, 41 (53.9%) were disabled and 13 (17.1%) were dead. The chi-squared test showed that the ultrasound results were significantly related to 30-day outcome (p = .0003). After logistic regression analysis, the ultrasound results remained independent predictors of 30-days outcome (p = .0129), together with neurological impairment 24 hours after stroke onset and lesion size at control computed tomography. Our study suggests that the results of ultrasound examination may be useful in the management of acute carotid stroke as an early indicator of patients with a worse prognosis.

Carotid stents are not a source of microemboli late after deployment.

OBJECTIVES: To evaluate the presence of microembolic signals (MES) in the middle cerebral artery distal to a stented carotid artery late after stent deployment. MES may be a signal of thrombus formation at the stent level. PATIENTS AND METHODS: Two patient groups were studied with transcranial Doppler at different intervals after deployment of Palmaz stents or Wallstents for stenosis of the internal or common carotid artery. Group 1: from 6 to 12 months after stent deployment; Group 2: > 12 months after stenting. RESULTS: One out of 19 patients in Group 1 showed MES (5.0%); however, this patient also had two mechanical heart valves. None out of 17 patients in Group 2 had MES. CONCLUSIONS: Carotid stents seem to have a very low emboligenic potential after the early post-deployment period. This is a reassuring message for present and future patients harboring such stents.