Thiamine uptake in human intestinal biopsy specimens, including observations from a patient with acute thiamine deficiency.

Mucosal biopsy specimens obtained by routine endoscopy from 108 human subjects, including one patient with thiamine deficiency, were incubated at 37 degrees C in oxygenated calcium-free Krebs-Ringer solution (pH 7.5) containing tritiated thiamine and [14C]dextran as a marker of adherent mucosal water. The amount of labeled thiamine taken up was measured radiometrically. In subjects with no clinical evidence of thiamine deficiency, 1) thiamine uptake by duodenal mucosa had a hyperbolic time course, reaching equilibrium at 10 min; 2) thiamine concentrations < 2.5 mumol/L were taken up predominantly by a saturable mechanism displaying Michaelis-Menten kinetics (K(m) 4.4 mumol/L and Jmax 2.3 pmol.mg wet tissue-1.6 min-1), whereas higher concentrations were taken up by passive diffusion; 3) thiamine transport had different capacities along the gastrointestinal tract (duodenum >> colon > stomach); and 4) thiamine uptake was competitively inhibited in the duodenum by thiamine analogs, albeit with a different order of potency compared with rats, and was blocked by 2,4-dinitrophenol. In the thiamine-deficient patient, the duodenal saturable uptake was increased, with higher K(m) and Jmax values. In conclusion, physiologic concentrations of thiamine were transported in human small intestine by a specific mechanism dependent on cellular metabolism, whose transporters appear to be down-regulated.

Clusterlike headache. A case of sphenoidal aspergilloma.

Clusterlike headaches secondary to structural disease have been described. Nevertheless, most of the reports antedate the International Headache Society classification and, indeed, only a few appear to fit its criteria for a diagnosis of cluster headache. We report here a case of sphenoidal sinus aspergilloma that fully complies with the criteria for cluster headache. This kind of lesion has never been described previously as a cause of a clusterlike headache.

Alzheimer's disease and senile brains: an immunofluorescence study.

The results obtained with direct immunofluorescence techniques on normal, senile and Alzheimer's disease brains are reported. The antisera used were directed against C3c, IgG (gamma chain), IgG Fc., kappa and lambda light chains and anti-Ig (total). The brain samples were prepared in different ways: formalin-fixed and paraffin embedded; fresh frozen samples and alcohol fixed, paraffin-embedded samples. The images obtained with the different processing methods did not coincide. In order to obtain a more complete picture, we suggest the use of different methods on the same samples. In Alzheimer's disease brains, abundant but probably not monoclonal Ig antigenic determinants were found. Ig were found diffusely in the tissue, connected to amyloid (both in senile plaques and blood vessel walls), in glial cells and neurons. The significance of such data in relation to the genesis of brain amyloid are discussed.

[Anatomo-clinical study of 4 cases of Jakob-Creutzfeldt disease (spongiform encephalopathy)]. / Studio anatomo clinico su quattro casi di malattia di Jakob-Creutzfeldt (encefalopatia spongiforme).

Four anatomo-clinical cases of Creutzfeldt-Jakob disease (spongiform encephalopathy) are described. In 3 cases the correct diagnosis was made while the patients were living. The most important diagnostic elements are the following: rapid evolution of a presenile or senile dementia, with neurological symptoms unusual in the Alzheimer's disease. The importance of EEG for the diagnosis of Creutzfeldt-Jakob disease is confirmed: however the onset of the typical EEG abnormalities was very late in its manifestation, the CT alterations are in general absent or very limited. The pathological alterations are aspecific. The spongiform process was sometimes scarce or limited to specific cerebral areas.

Lead polyneuropathy of nonindustrial origin.

A case of a subacute, distal, symmetrical polyneuropathy in a housewife is reported. The fact that the polyneuropathy affected almost only the motor fibers led us to suspect an exogenous intoxication. Blood lead level and the urinary excretion of lead after intravenous EDTA very high. The blood lead levels were normal in the husband and children oif the patient. A glazed cup used only by the patient to keep tea with lemon juice was found to release toxic amounts of lead with acidic solutions. The manufacturer of the cup was not identified by the enquiring authorities and therefore almost certainly other lead-releasing cups are still in use. The importance of suspecting lead intoxication in individuals not professionally exposed is stressed.

Electroencephalographic and histopathological correlations on Jakob-Creutzfeldt disease.

Three patients with Jakob-Creutzfeldt disease were studied. The clinical evolution was followed and several EEG controls were made. In all patients characteristic EEG abnormalities were present. Histopathological features in the cortical, subcortical grey matter and in the white matter were examined. Subcortical and cortical grey matter were involved while the white matter appeared intact. The results were compared with previous studies of other Authors and the correlation between the characteristic EEG alterations and the histopathological features was studied in order to explain the appearance of the paroxysmal EEG findings. In agreement with other studies, the Authors emphasized the cortical and subcortical grey matter involvement in the genesis of EEGraphic paroxysmal aspects. The intervention of other factors, possibly of histochemical nature, was postulated.

[Posterior fossa angiomata with slow evolution: clinical aspects (author's transl)]. / Angiomi in fossa cranica posteriore a decorso cronico aspetti clinic

The AA. report 9 cases of arteriovenous angiomata in the posterior cranial fossa with chronic evolution: in 8 of these the diagnosis was made before a subarachnoid haemorrhagic episode precipitated the symptomatology. Angiomata in the posterior cranial fossa are detected less often than sopratentorial angiomata, and the possible reasons of this are considered. During the last 11 years in the Neurosurgical Institute of the University of Padua the incidence was 23%, one of the highest reported in literature. The clinical diagnostic criteria of angioma in the posterior cranial fossa with chronic evolution are also discussed. The importance of a fluctuating development of the symptomatology is emphasised with an almost constant association of signs of pyramidal and cerebellar involvement, as well as the differential diagnostic problems with multiple sclerosis. In six of the cases the malformation was so extensive to make it impossible to determine afferences and drainages, so that a surgical operation could not be carried out. This finding seemed rather peculiar, owing to the relatively poor simptomatology, mainly if compared with smaller supratentorial malformations. It appears from the literature that angiomata in the posterior cranial fossa including those found at autopsy are as common as the supratentorial ones; this would suggest that many of these lesions are not diagnosed in life.

Motor neurone disease: an immunological study.

The state of the immune system, both humoral and cell-mediated, was evaluated in motor neurone disease (MND) patients. The data obtained for the MND patients were confronted with normal controls and a group of patients affected by nervous diseases not involving the immune system. Some differences were observed between MND patients and normal subjects, namely: increase of WBC and gamma-globulin in MND patients. However, such differences were not observed between MND patients and pathological controls, and therefore are probably due to a higher frequency of infectious complications in MND patients in respect to normal controls. The capacity of the immune system to respond to an adequate stimulus was normal, and no precipitating anti-CNS antibodies were detected in MND sera. Furthermore, no sex-linked differences were observed and the CSF abnormalities observed in 2 out of 16 MND patients were probably reflecting only destruction of CNS cells. The data are discussed in view of the possible pathogenetic mechanisms of MND.