An unusual cause of dementia: essential diagnostic elements of corticobasal degeneration-a case report and review of the literature.

Corticobasal degeneration (CBD) is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson's Disease. At our clinical examination, she presented left upper-arm-fixed-dystonia, spasticity in left lower limb and pyramidal signs (Babinski and Hoffmann). Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect. This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition. There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the difficulty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reflexes, and correlations between results on electroencephalography (EEG) and electromyography (EMG) provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease.

The Società Italiana di Neurologia: origins.

This paper describes the history of the Italian Society of Neurology (Società Italiana di Neurologia, SIN), originally an offshoot of the Società Freniatrica Italiana, whose origins go back to Aversa in 1861, when Biagio Gioacchino Miraglia founded the Società Frenopatica, the first neuropsychiatric organisation for Italian scientists. The "Società Freniatrica" was founded in Rome in 1873 and was to bring together neurologists and psychiatrists until 1907, when the SIN was founded with its own Articles of Association (subsequently modified in 1971) and the two specialisations of neurology and psychiatry were recognised as being distinct entities. The first SIN Congress was held in Naples in 1908, and an analysis of its themes and those of subsequent Congresses highlights the fact that, right from the start, the interests of neurologists covered the whole range of the neurosciences: neuroanatomy, histology, physiopathology and clinical neurology. Over the years, special Groups have been established to enable the more profound study of specialised aspects, and the Society of Hospital Neurologists founded.

Charcot and his drawings: images from "les leçons du mardi à la Salpêtrière 1887-1888".

In this paper, the authors pay tribute to Jean Martin Charcot in the centenary of his death (16th August 1893), concentrating on his artistic sense and drawing ability. The authors give some examples of the drawings Charcot used to illustrate his Tuesday Lessons at Salpêtrière, together with his highly precise clinical and semiological descriptions.

The care of epileptics: from the madhouse to the League against Epilepsy.

We trace the first steps in the organization of the International League against Epilepsy, the great initial impetus for which came from an Italian savant, Prof. Tamburini, among others. The League came into being because of the need to differentiate epilepsy from insanity and to channel into a single institution all research into the etiopathogenesis, new therapies and principles of care to ensure their rapid diffusion all over the world.

New concepts in preoperative imaging of anorectal malformation. New concepts in imaging of ARM.

In this study of 14 patients with anorectal anomalies CT and MRI were employed for preoperative assessment. The use of a pressure enhanced water soluble enema via the colostomy proved to be an extremely efficient method for showing a fistula. MRI studies were enhanced by the use of vaseline oil and in one case this technique was used prior to surgery to provide important information by injecting through a perineal fistula. CT and axial MRI proved to be more valuable than sagittal MRI which is only useful for the length of the atretic segment. The authors consider that a combined approach using pressure enhanced water soluble enema and MRI will provide the most valuable preoperative information to plan a successful operative approach and enable an accurate prognostic evaluation of continence in these difficult and complex patients.

[Computed tomography vs. magnetic resonance in the diagnosis of anorectal anomalies]. / Tomografia Computerizzata vs Risonanza Magnetica nella diagnostica delle anomalie ano-rettali.

Anorectal malformations include a variety of anatomical anomalies which are often difficult to assess. Thirty-four patients (age range: 1 month-19 years) were studied over a 4-year period, 14 of them preoperatively and 20 postoperatively. In addition to inverted lateral plain films, a barred Iopamiro enema was performed in 13 of 14 cases by a Foley's catheter via colostomy. Endocolic pressure could thus be increased, which allowed the detection of rectal fistulas in 11 of 13 cases. Preoperative CT was performed in 9 cases, and MRI in 5. Both CT and axial MR scans provided accurate images of sphincteric muscles: they were well developed in 7 patients and poorly developed in 6; in 1 case the muscles were not identified. Sagittal MR scans only demonstrated the distance between blind pouch and perineum. Sagittal MRI also provided images of the spine (1 patient exhibited tethered cord). In the postoperative management, except for severe sacral anomalies, miscentered puborectal muscle and hypoplastic musculature were observed as the most frequent causes of fecal incontinence in patients treated with abdomino-perineal pull-through of the colon. To select the cases who needed reoperation, most frequently secondary PSARP, 17 patients were studied by CT and 7 by MRI; both techniques were employed in 4 cases. In 6 patients good development of sphincteric musculature and neo-anorectum correctly positioned within were observed. In 4 cases the neo-anorectum was correctly positioned, but sphincteric muscles were too hypoplastic to allow good continence; in 10 patients with incontinence, the neo-anorectum was seen outside sphincteric musculature: in 2 cases the latter was well developed (1 patient had had severe surgical damage to levator ani), while in 8 it appeared to be poorly developed. In our experience, postoperative CT allowed a correct evaluation to be made, whereas MRI failed to yield additional information.

Cor triatriatum. Clinical presentation and operative results.

From November 1973 to January 1988, 15 patients with cor triatriatum underwent surgical correction at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. Their ages ranged from 15 days to 48 years. Eight patients (53%) were younger than age 1 year. Evidence of congestive heart failure was present in five infants, whereas three infants, three children, and two adults initially had signs of pulmonary venous obstruction, and two children had a heart murmur only. Five patients had cor triatriatum alone; in ten cases there also was an atrial septal defect. Associated anomalies in four patients included left superior vena cava, ventricular septal defect and left superior vena cava, partial anomalous pulmonary venous connection, and bilateral partial anomalous pulmonary venous connection. Cor triatriatum was repaired with the aid of cardiopulmonary bypass in all patients. Excision of the membrane was accomplished with a right atrial approach in 13 patients and a left atriotomy in one patient. Both atria were opened in one case. All associated anomalies were simultaneously corrected. Three patients (20%) died early after operation. Among the 12 survivors, no late events have occurred, and all of them are presently in New York Heart Association functional class I. A recent echocardiogram shows absence of residual obstruction or shunt and good development of the left cardiac chambers.

[Apple peel small bowel: surgical correction and post-operative treatment]. / Apple peel small bowel: correzione chirurgica e trattamento post-operatorio.

The authors describe 6 cases of Apple Peel Small Bowel observed in the last ten year experience in Institute G. Gaslini of Genoa, Italy. They make a precision on early diagnosis and on surgical treatment, which is original, and also on post-operatory medical treatment. The importance of making an optimal nutritional support with total and partial parenteral nutrition for long time is of great significance to have a better outcome and psycho-physical development in such patients.

[Importance of computerized tomography in the early diagnosis of Bourneville's tuberous sclerosis]. / Importanza della tomografia computerizzata nella diagnosi precoce della sclerosi tuberosa di Bourneville.

CT findings in a series of 23 cases of tuberous sclerosis are presented. The great importance of CT screening for the early diagnosis of this disease and the opportunity of CT follow-up of subependymal nodules, for their high malignant potential are underlined.

Aortopulmonary window in the first year of life: report on 11 surgical cases.

From June, 1975, through April, 1986, 11 consecutive infants with aortopulmonary window were operated on at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. The classic type of aortopulmonary window was present in 6 patients, whereas 4 patients had a ductus type of aortopulmonary window and 1 patient had a double aortopulmonary window, associated transposition of the great arteries, and ventricular septal defect. Operative technique included patch closure of the defect by means of cardiopulmonary bypass using various approaches: transaortic in 3, transpulmonary in 2, and transwindow in 2. The aortopulmonary window was ligated in 3 patients, in 2 of whom an incorrect diagnosis of patent ductus arteriosus was made at cardiac catheterization. Division and suture of the defect through a median sternotomy was performed in the remaining patient, who was moribund on admission. There was a single operative death. All survivors are in New York Heart Association Functional Class I at a mean interval of 6 1/2 years postoperatively.

[The hemophilic child. Post-traumatic cerebrospinal complications. Diagnostic protocol]. / Il bambino emofilico. Complicanze cerebro-spinali post-traumatiche. Protocollo diagnostico.

The authors report their experience on 30 hemophilic children controlled at the Pediatric Institute G. Gaslini, Genoa, Italy; among the 30 patients, there were 4 cases of hemophilia A, and 1 of Von Willebrand disease. Various degrees of trauma in different sites had caused central neurological complications. In all cases CT, both in the immediate post-traumatic phase and later on, allowed both an early diagnosis and the follow-up of hemorrhagic lesions and late complications, even in absence of significant neurologic symptomatology; medical replacement treatment and neurosurgery, when needed, allowed a positive resolution of all cases. The authors believe this early-phase and follow-up diagnostic protocol, together with strict clinical and laboratory controls, to allow a prompt interdisciplinary therapeutic approach, which has preventive aims as well.

[Congenital coronary fistulae. Comments on 9 surgical cases]. / Fistole coronariche congenite. Considerazioni su 9 casi operati.

The authors relate their experience concerning the surgical correction of congenital coronary fistula. Between May 1971 and June 1986, 9 patients (4 boys and 5 girls) aged from 17 days to 49 years were operated upon at the Division of Cardiac Surgery of Bergamo (Ospedali Riuniti). All the patients, except three who were asymptomatic, showed early cardiac failure or dyspnoea on effort and angina in the elderly. At the physical examination a continuous murmur was heard in 8 cases; the chest x-ray showed significant cardiomegaly and on the electrocardiogram a right/left ventricular hypertrophy pattern was detected in 5 patients. All the patients underwent cardiac catheterization and a coronary angiography. The left-to-right shunt ranged from 60% to 250% of the cardiac output. The anomalous communication affected the right coronary artery in 7 cases and the left in 2, opening into the right atrium in 4 patients, into the right ventricle in 3 and into the pulmonary artery in 2. All patients but one, in whom division and suture were the only necessary procedures, underwent correction by means conventional cardiopulmonary by-pass with moderate hypothermia. In 3 cases closure through the coronary artery was preferred, in 1 through the right ventricle and in 2 transpulmonary. There was only 1 late death which occurred in a 3 year-old patient due to renal failure. After a mean follow-up of 6 years, 7 patients are to be asymptomatic while 1 patient had to be reoperated for a significant residual shunt.