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Biomedical subjects

C Crisci

Publications and source records attributed to C Crisci.

At least 19 recordsLinked to original sources

Italian neurologists and euthanasia: a poll.

We have tried to sound out Italian neurologists regarding their attitude to euthanasia, a very controversial issue today. We gave a short multiple-choice questionnaire to neurologists attending a national conference, asking their opinion on some issues related to euthanasia. 75 (25%) of the 300 doctors polled completed the questionnaire. Answers were often contradictory. A trend in favor of passive euthanasia among the respondents and a strong demand for guidelines are evident. Emotional rather than professional or rational factors seem to play a major role in the approach to this dilemma.

Attitude of Health Personnel

Is early onset cerebellar ataxia with retained tendon reflexes identifiable by electrophysiologic and histologic profile? A comparison with Friedreich's ataxia.

An electrophysiologic and histologic study was performed on 18 patients affected by early onset cerebellar ataxia with retained tendon reflexes (EOCA). Sensory and motor conduction velocity (SCV, MCV) was measured along peripheral nerves in all patients, somatosensory (SSEP) and brainstem auditory evoked potentials (BAEP) were recorded in 13; cortical stimulation (CS) in 12, and sural nerve biopsy in 4 patients were also performed. The results as a whole allow a division of EOCA patients into 2 groups: with (7 patients) and without (11 patients) peripheral neuropathy. Among EOCA patients with neuropathy a differential diagnosis with Friedreich's disease patients was not possible according to BAEPs and CS, while SSEPs could differentiate 2 out 5 patients in whom they were performed.

Adolescent

POEMS syndrome: follow-up study of a case.

We report here the case of a 20-year-old man with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M proteins, skin changes). This rare syndrome followed a 3-year history of a syndrome that mimics a chronic inflammatory demyelinating polyneuropathy (CIDP). Treatment with cyclophosphamide induced regression of the syndrome and improved peripheral nerve conduction.

Adult

The relationship between electrophysiological findings, upper limb growth and histological features of median and ulnar nerves in man.

In 19 median and 10 ulnar nerves of 28 healthy young volunteers, the maximum orthodromic sensory conduction velocity was studied along one or two fingers (the third and/or the fifth), the palm, and the forearm. In five 20 to 32-yr-old males and in a newborn male, post-mortem samples of either the median or the ulnar nerve were obtained from the finger (either the third or the fifth), the palm, the wrist and the forearm in order to study the size distribution of external fibre and axon diameters, and the fibre internodal length. In 2374 infants, children and adolescents, the height, length of both the third and the fifth finger, length of the palm, and length of the forearm were measured. The results showed (i) a significantly slower conduction velocity along the fingers; (ii) a significantly shorter internodal length without remarkable fibre diameter changes in the same nerve segments; (iii) a significantly smaller elongation of these body parts; (iv) a significant correlation between these data. In conclusion, internodal length seems to play an important role in governing conduction velocity of myelinated peripheral nerve fibres.

Adolescent

Screening for HIV.

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AIDS Serodiagnosis

Electrophysiological and histological follow-up study in 15 Friedreich's ataxia patients.

A clinical and electrophysiological follow-up was carried out for 3 to 7 years on 15 patients with Friedreich's ataxia (FA). Sural nerve biopsy was performed once in all patients, and a second time 6-7 years later in three of them. Clinical worsening and progression of disturbance were evaluated according to IAP and IACR scales. Sensory orthodromic conduction along median and tibial nerves was typical of FA and did not change between first and last examinations, nor were there morphological changes between the first and the second sural nerve biopsies. Peripheral nerve involvement is thought to be a result of defective development of the largest neurons and to remain stable from a very early stage of the disease; the clinical worsening may then be due to a progressive involvement of the pyramidal tracts and the cerebellar pathways.

Adolescent

Excision of the aortic wall in the surgical treatment of acute type-A aortic dissection.

Between March 1986 and September 1988, 38 patients underwent extended aortic resection (aortic valve, ascending aorta, and arch) for acute type-A aortic dissection with aortic valve insufficiency; deep hypothermia and circulatory arrest were used. All patients were operated on within 17 hours of the onset of symptoms. In the first 24 patients, operation was performed by the "inclusion technique." In the last 14 patients, the "excision technique" was used: the ascending aorta and arch was excised, and the aorta was transected at the beginning of the descending thoracic tract. Excision and transection were considered essential to prevent back flow from the false lumen, which is the main source of bleeding, and to allow all anastomoses to be constructed beyond the limits of dissection. The only anastomosis to the dissected aorta was at the distal end of the graft. One of the 14 patients died (7.1%). One patient was reopened for bleeding: blood was issuing from the attachment of the carotid trunks, and the defect was repaired by interposing a bifurcated Dacron graft between the arch graft and the carotid arteries. Extended aortic excision meets the principle of either eliminating as far as possible the diseased aorta or controlling intraoperative and postoperative bleeding. An operation of great magnitude can be considered a life-saving procedure when compared with the high risk of acute type-A aortic dissection.

Aortic Dissection

A family with tomaculous neuropathy mimicking Charcot-Marie-Tooth disease.

The appearance of Guillain-Barré syndrome in a 9-year-old girl led to the detection of a hereditary neuropathy in her family. This neuropathy showed clinical and electrophysiological characteristics of Charcot-Marie-Tooth disease. Only nerve biopsy performed in a sister of the proband allowed diagnosis of tomaculous neuropathy which presented unusual clinical, electrophysiological and bioptic aspects.

Biopsy

Somatosensory evoked potentials and motor cortex stimulation in myelopathies.

Short latency somatosensory evoked potentials (SSEPs) to stimulation of the tibial nerve at the ankle and at the knee and motor action potentials (MAPs) recorded from the tibial muscle during transcranial stimulation of the motor cortex were recorded in 21 patients with myelopathy. The electrophysiological results were compared with the clinical evolution over 12 months. Both scalp SSEPs and MAPs were absent in 10 patients with clinically 'complete cord transection.' The clinical and electrophysiological data remained unchanged in these patients. Scalp SSEPs were present at the first examination in all 11 patients with clinically 'incomplete cord lesion.' All these patients improved in the following year. The clinical recovery was almost complete in 3 patients who had normal scalp SSEPs but varied markedly in 8 patients with abnormal SSEPs. Normal MAPs were obtained in 1 of the 3 patients who showed the best clinical recovery. In the other subjects, MAP findings did not show a clear correlation with either the clinical signs or the course.

Action Potentials

The idiopathic hypereosinophilic syndrome. Clinical, electrophysiological and histological study of a case.

A case of idiopathic hypereosinophilic syndrome (HES) is reported. The disease started at the age of 31, with polyneuropathic-like symptoms and disorders of the gastrointestinal tract. Hypereosinophilia and leukocytosis were observed. Instrumental investigation of the digestive tract showed esophagitis, a peptic ulceration, signs of chronic rectocolitis. Charcot-Leyden crystals were present in feces. Peripheral nervous system changes were seen with EMG and nerve conduction studies. Muscle biopsy findings were in agreement with the electrophysiological data and showed inflammatory abnormalities. Qualitative histology and teased nerve fiber studies of sural nerve indicated axonal degeneration. Protein substances derived from eosinophils degranulation may account for the disturbances observed in various systems and organs.

Adult

Trochanteric sciatic neuropathy.

A patient with severe weakness, atrophy, and sensory loss of the right leg had a focal right sciatic neuropathy. The sciatic nerve was enlarged at the level of the lesser trochanter, excessively firm, and multistranded; its stimulation threshold was focally increased. Biopsied fascicles had reduplicated perineurial leaflets, many Renaut bodies, and an abnormal unimodal spectrum of small-diameter fibers. We postulate that the lesion was induced by the combination of an underlying prominent lesser trochanter and sitting on hard benches.

Atrophy

A case of Dejerine-Sottas disease with prominent ataxia and brain stem involvement. A clinical, electrophysiological, otoneurologic, and ultrastructural study.

A case is presented of Dejerine-Sottas disease in a 12-year-old boy in which clinical signs made diagnosis of Friedreich's ataxia seem plausible. Based on marked slowing of motor conduction velocity, the sural nerve biopsy findings of a hypertrophic neuropathy with hypo- and demyelination of the nerve fibres, as well as the clinical history, the diagnosis of Dejerine-Sottas disease was made. ABR examination suggested involvement of brain stem at the roots and/or nuclei of the eighth cranial nerve, without involvement of higher structures.

Adolescent

Is the sensory neuropathy in ataxia-telangiectasia distinguishable from that in Friedreich's ataxia? Morphometric and ultrastructural study of the sural nerve in a case of Louis Bar syndrome.

The bioptical morphometric and ultrastructural study of sural nerve in a 17-year-old boy with ataxia-telangiectasia (AT) is reported. Our findings include a loss of fibers, particularly of large ones, axonal degenerative changes, Schwann cell inclusions of various type, and rare signs of primary demyelination. Teased-fiber study showed paranodal myelin enlargements, segmental demyelination, shortening, and/or variability of internodal length. This picture is similar to that in Friedreich's ataxia (FA), although they differ in degree and time of onset. A correct neuropathologic diagnosis of AT cannot be made on the basis of sural nerve biopsy alone.

Adolescent

Intercellular glycosaminoglycans in human cancer.

Intercellular glycosaminoglycans (GAGs) from various tissues were analyzed by cellulose acetate electrophoresis and enzymatic treatment with specific mucopolysaccharidases. Each tissue exhibits a particular composition of sulfate and unsulfated molecular species. Invariably, malignant human neoplasias and their metastases show striking variations in the electrophoretic pattern typical of the corresponding normal tissue. An absolute or relative increase in surface ChS A/C and HA seems to be a consistent feature of neoplastic transformation. On the other hand, the GAGs composition of benign noninfiltrative tumors does not vary greatly with respect to the original normal tissue.

Animals