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Biomedical subjects

C Perla

Publications and source records attributed to C Perla.

12 recordsLinked to original sources

[Cerebellar degeneration associated with anti-Tr antibodies without Hodgkin's disease. Four years follow-up].

The presence of antineuronal anti-Tr antibodies is associated to paraneoplastic cerebellar degeneration due to Hodgkin's disease. The anti-Tr can become negative after successful and early treatment of the tumor, and there could even be remission of the cerebellar symptoms in some patients. There are few cases in which no tumor is found when there are anti-Tr. We report the case of a 66 year old man with a severe cerebellar syndrome and anti-Tr in serum detected by immunohistochemistry. After a 4 year follow-up, no underlying tumor has been found. In addition, anti-Tr spontaneously disappeared. The cerebellar degeneration persists and is incapacitating. This case suggests that in a few instances the origin of anti-Tr is not a tumor but another unknown cause. Alternatively the anti-Tr mediated immune response could have eradicated the underlying lymphoma.

Aged↗

[Alien hand syndrome in Creutzfeldt-Jakob disease].

Alien hand syndrome is defined by uncontrolable actions of the arm and hand that seem to have a purpose. It is usually associated with acute focal lesions after a stroke or surgery of the corpus callosum. It has been described in chronic dementiating diseases such as cortico-basal degeneration, Alzheimer's disease, orthochromatic leukodystrophy and Marchiafava-Bignami disease. We now report a patient with Creutzfeldt-Jakob disease and alien hand syndrome, which appeared after the cognitive alterations and had characteristics of the frontal type of alien hand affecting the dominant upper limb. In the three cases described previously in the literature the alien hand affected the non-dominant hand and the abnormal movement appeared before dementia was clinically obvious. The non specific nature and poorly localising sign of alien hand is remarked. Alien hand should be added the list of features of Creutzfeldt-Jakob disease.

Aged↗

Lithium-induced Creutzfeldt-Jakob syndrome.

a 67-year-old man with bipolar disorder developed a Creutzfeldt-Jakob like syndrome during lithium carbonate treatment. Lithium serum level was within the therapeutic range. Complete clinical-electroencephalographic recovery was achieved after lithium therapy was discontinued. Several cases of lithium-induced Creutzfeldt-Jakob syndrome have been reported to date; all of them were elderly patients and a half had "therapeutic" lithium serum levels. Patients in this age group receiving antimanic maintenance treatment should keep lithium serum levels as low as possible. Lithium neurotoxicity should be considered in Creutzfeldt-Jakob disease differential diagnosis, serial electroencephalograms being the most valuable.

Aged↗

[Celiac disease with occipital calcifications: 2 late cases].

The association of celiac disease, epilepsy and occipital calcifications with initial clinical manifestations of epilepsy during the first two decades of life with an often progressive and variable course and clinical expression of malabsorption has recently been described. Two cases of celiac disease with occipital calcifications and a presentation with neurologic symptoms in adulthood are reported. The first case is that of a 40-year-old male who presented recurrent and alternating pure brachial monoparesis and later acute abdominal pain following which celiac sprue was diagnosed. The second case is that of a 53-year-old woman diagnosed with celiac sprue 20 years before, presenting permanent myoclonus in the lower limbs which were progressive in severity, ataxic march and generalized tonoclonic seizures. Both patients had bilateral occipital calcifications on CT and celiac disease was demonstrated on biopsy. The first case also showed marked signal alteration in the white matter on MRI. Celiac disease with cerebral calcifications presents also in adulthood with atypical clinical manifestations. Suspicion of celiac disease may be confirmed by non-invasive methods such as antigliadin and antiendomysium antibody determination. CT imaging is characteristic.

Adult↗

Spinal cord compression by amyloid deposits.

STUDY DESIGN: Dorsal spinal cord compression caused by amyloid tissue deposits in the posterior vertebral arch and epidural space of the T6-T7 region in a 29-year-old woman is reported. RESULTS: Studies did not show any primary cause of amyloidosis. CONCLUSIONS: This case can be classified as primary localized tissue because other causes and amyloid deposits were not discovered. This patient was the youngest person reported to have dorsal spinal cord compression caused by amyloid tissue deposits, and the spinal cord compression was localized in the thoracic region, which occurred in six of the seven cases reported.

Adult↗

[Subdural hemorrhage of aneurysmal origin].

Although most subdural hematomas are considered to be venous in origin, they may also be of arterial origin. When subdural bleeding is due to the rupture of an intracranial aneurysm, most commonly at the middle cerebral or internal carotid arteries, the amount of subdural blood is usually small and of no clinical importance. We describe two patients with subdural hematomas secondary to rupture of an intracranial aneurysm, who needed prompt surgical treatment. The first patient had a left internal carotid artery aneurysm at the origin of the ophthalmic artery. In the second patient the aneurysm was at the anterior communicating artery and rebled into the subdural space directly through a right intraparenchymatous frontobasal hematoma. The most probable mechanism of subdural bleeding in our two patients was the existence of adhesions between the aneurysm and the arachnoid due to previous minor hemorrhages. The indication of cerebral angiography in a patient with subdural hematoma is based mainly upon the existence of meningeal signs, the presence of blood in more than one intracranial compartment or the rapid progression of bleeding.

Aged↗

[Striatocapsular infarct in a young patient with heterozygous familial hypercholesterolemia and Klinefelter's syndrome].

Familial hyperlipidemia has received little attention as a possible cause of stroke in young patients. Some recent studies have demonstrated that lipoprotein (a) is a key factor for atherogenesis in familial hypercholesterolemia. Hypogonadism may also contribute to the elevation of serum lipids, but their influence as a risk factor for stroke is still less understood. A 34-year-old patient with heterozygous familial hypercholesterolemia presented with a left pure motor hemiparesis secondary to a right striatocapsular infarction. Arteriography showed atherosclerotic lesions in both internal carotid arteries. High levels of cholesterol, cLDL, apo B, and lipoprotein (a) were found. Clinical signs of hypogonadism were present and the karyotype led to the diagnosis of Klinefelter's syndrome (47,XXY). The early clinical course was excellent, and the levels of serum lipids were normalized with diet, lipid-lowering drugs and androgens. The importance of hyperlipidemia as a risk factor for stroke in the young, specially when it occurs in the context of familial hypercholesterolemia with elevated lipoprotein (a) levels, as well as the possible contribution of hypogonadism to the development of accelerated atherosclerosis in young patients, are discussed upon.

Adult↗

Effects of chronic dobutamine on cardiac mechanics and biochemistry after myocardial infarction in rats.

After myocardial infarction in rats, muscle performance in the remaining hypertrophied myocardium deteriorates and is associated with a decrease in myosin adenosinetriphosphatase (ATPase) activity and a shift to the V3 myosin heavy-chain isoform. We have previously shown in another model of hypertrophy, secondary to renovascular hypertension, that chronic intermittent adrenergic stimulation with dobutamine (Db) can prevent this biochemical adaptation. The present study was undertaken to assess the effects of chronic Db treatment on cardiac mass, function, metabolism, and myosin biochemistry in animals subjected to chronic myocardial infarction. Four groups of rats were studied: controls, animals treated with Db (2 mg/kg 2X daily for 4 wk), animals subjected to myocardial infarction and killed after 4 wk (MI), and MI animals concurrently treated with Db for 4 wk (MI-Db). The two MI groups were subdivided into those with and without congestive heart failure (CHF). Heart weight was increased by 13% with Db, unchanged in the infarct groups without CHF, and increased by 9 and 22% in the infarct groups with CHF. Db did not have any additional effect on heart weight in these later groups. Infarct weight was greatest in the animals with CHF, and viable myocardium was equivalent in all infarct groups suggesting that CHF was associated with a greater degree of hypertrophy. Ventricular performance, as assessed in an isovolumic heart apparatus, was markedly depressed in both infarct groups with CHF and was not affected by Db. Db increased myosin ATPase activity in control and infarcted animals both with and without congestive heart failure. Myosin oxygen consumption and lactate production were not adversely affected by Db.

Animals↗

[Radioactive phosphorus (32P) in the diagnosis of ocular tumors. Results and therapeutic doses].

Because of phosphorus involvement in metabolic processes, 32P is a suitable radioisotope for the investigation of the function of normal and malignant cells. The basic principles of the 32P test are analysed. This paper presents the results on a series of patients who underwent the 32P uptake test at the Ospedale Oftalmico of Turin. An evaluation of the dose to the patient is also presented.

Dose-Response Relationship, Radiation↗