Italian journal of neurological sciences最新文献

Symptomatic dissecting aneurysms of the extracranial internal carotid artery are not frequent. Medical treatment of these lesions can be ineffective and surgical options present high morbidity. We describe a case successfully treated with endovascular therapy, using a self-expanding stent. The advantages of the endovascular treatment and the rationale of the use of self-expanding stents are discussed.

Proton magnetic resonance spectroscopy ((1)H-MRS) was performed in patients with a clinical diagnosis of idiopathic Parkinson's disease (IPD), multiple system atrophy (MSA) or progressive supranuclear palsy (PSP) in order to assess metabolic differences between the three groups of patients. Single-volume (1)H-MRS, localized to the lentiform nucleus, was carried out in 19 IPD patients, 14 MSA patients, 11 PSP patients and 12 age-matched healthy subjects. The signals of N-acetylaspartate (NAA), choline-containing compounds (Cho) and creatine-phosphocreatine (Cr) were evaluated as peak area ratios. The NAA/Cho peak ratio was significantly reduced in MSA and in PSP patients compared to IPD patients and to controls. The NAA/Cr peak ratio was significantly reduced in MSA, in PSP and in IPD patients compared to controls, but only in MSA compared to IPD patients. The NAA reduction in the basal ganglia of MSA and PSP patients may reflect a neuronal loss or damage. Single-volume (1)H-MRS may be a useful tool in differentiating MSA and PSP from IPD patients.

Superficial siderosis of the central nervous system is a rare condition characterized by deposition of haemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. With the widespread use of magnetic resonance imaging, an increasing number of cases of superficial siderosis are being discovered, secondary forms being more frequent than idiopathic ones. We report a 78-year-old man in oral anticoagulant therapy, who presented neurosensory hearing loss, gait ataxia and spastic paraparesis. Magnetic resonance imaging suggested the diagnosis of superficial siderosis of the central nervous system, without an evident bleeding source.

Cytomegalovirus (CMV) encephalitis is particularly evident in immunodepressed patients. Often diagnosis is difficult and time-consuming because of the complex basic clinical picture of these patients. We describe the diagnostic steps taken in a case of cytomegalovirus encephalitis affecting an elderly patient, classified as immunodepressed and deceased on the thirty-fifth day of hospitalisation in the intensive care unit due to acute myocardial infarction. Treatment with ganciclovir, 30 mg/kg per day, begun at the time of diagnosis, appears to have had a positive effect on the neurologic symptoms.

We describe a right-handed female patient who, after a right-hemisphere stroke, showed a florid representational neglect, but no evidence of perceptual neglect. This finding provides further support for the argument that orienting attention to the representational and perceptual spatial domains is subserved by independent neural mechanisms.

The aim of our work was to identify clinical and electroencephalographic factors that are statistically correlated to 99mTc HMPAO hypofixation in cryptogenic temporal lobe epilepsy. We selected a homogeneous group of patients (28 males and 17 females) affected by temporal lobe epilepsy and having normal results on computed tomography and magnetic resonance imaging. Patients were classified according to semeiological characteristics of the seizures, presence or absence of spikes on electroencephalography (EEG), age at onset, illness duration, and drug resistance. The presence of focal interictal spikes on EEG was statistically correlated to 99mTc HMPAO temporal hypofixation (p = 0.04). The other electroclinical factors considered in this series did not play an important role in modifying radiopharmaceutical fixation in focal epilepsy in interictal phase. We speculate that a possible explanation for focal radiopharmaceutical hypofixation, in addition to hypoperfusion, is an increased permeability of the blood-brain barrier (due to perivascular inflammation or other causes) which can be responsible for 99mTc HMPAO escape with a resultant hypofixation.

Differential diagnosis between autosomal dominant cerebellar ataxia type I (ADCA I) and idiopathic cerebellar ataxia type P (IDCA-P) is very difficult given only clinical and neuroradiological data. The only certain distinctive characteristic is the presence or absence of family history. We observed 7 patients with late-onset cerebellar ataxia associated with other non-cerebellar signs and without a family history of the disease in which clinical signs were comparable to symptoms found in SCA2. The neuroradiological study showed olivopontocerebellar atrophy in all patients and the presence of hyperintensity of the transverse pontine fibers in 6 patients (85. 6%); molecular analysis showed SCA2 mutations in 2 patients. We also report the case of a patient who was initially considered as IDCA-P but who was later correctly identified as SCA2 with an atypical family history (false IDCA-P), after a genetic mutation was found and following an interview with the mother. Our data suggest that spinocerebellar ataxia syndrome should be defined as idiopathic not only after having excluded the possible symptomatic causes but also in the absence of family history, after having excluded the presence of genetic mutation. We believe that family history, in late-onset spinocerebellar ataxia, cannot be considered as the differential criterion among hereditary (ADCA-I) and non-hereditary (IDCA-P) forms; molecular analysis is required for a correct diagnosis.

Cerebral venous and sinus thrombosis (CVST) is still considered a severe clinical problem that is difficult to diagnose and manage and is linked to a poor prognosis. Nonetheless, conventional cerebral angiography and magnetic resonance imaging (MRI), or more recently, MR angiography allow a more rapid and precise diagnosis, and prognosis has improved with the use of anticoagulant treatment. We report 23 cases of CVST consecutively admitted to the Institute of Neurology of the University of Parma during the period 1990-1997. In all cases diagnosis was confirmed by means of MRI or conventional angiography of brain vessels. Among the patients, 22 were female and 1 was male. In all patients, plasma levels of protein C, protein S, antithrombin III (ATIII) and antiphospholipid antibodies (APA) were evaluated. In 15 of 23 patients, the presence of factor V Leiden mutation was also determined, and found positive in 3 patients (20%). Of the 22 female patients, 15 (68%) were on low-oestrogen (containing less than 50 microg oestrogen) oral contraceptive (OC) treatment. This percentage of OC use by patients with CVST is much higher than that of the rest of the female Italian population. OC use was associated with the presence of factor V Leiden mutation in two cases, with a deficiency of protein C in 1 case and a deficiency of protein S in another.Whether low-oestrogen Ocs may induce cerebral thromboembolic events is an open matter. According to our data, it may be argued that Ocs, even if at low oestrogen content, represent a major risk factor for CVST. The use of Ocs, as is the case for systemic venous thromboembolic events, may further increase the risk of CVST in women carrying the factor V Leiden mutation or other inherited hyperthrombotic conditions.

Transcranial Doppler detection of microembolic signals has been described in several clinical conditions such as carotid stenosis, prosthetic heart valves, acute stroke, and carotid surgery. The importance of these events for clinical decision-making is still a matter of debate. The authors review the literature and report their personal experience on the clinical significance of microemboli detection.