M. Poloni

Monophosphate, the only phosphoric ester of thiamin in the cerebro-spinal fluid

With a specific and sensitive electrophoretic-fluorometric method, thiamin was found in the cerebro-spinal fluid of different mammals both in free and phosphorylated form, monophosphate being the only thiamin phosphoric ester. In humans, its amount was about 60% of total thiamin. Alcoholism greatly lowered total thiamin content, affecting both thiamin forms.

Intrathecal IgG synthesis in multiple sclerosis and other neurological diseases: A comparative evaluation by IgG-index and isoelectric focusing

SummaryIntrathecal IgG synthesis has been investigated by determining the IgG index and by isoelectric focusing in 30 cases of definite multiple sclerosis, in 15 cases of probable multiple sclerosis and in 128 patients affected by other neurological diseases. The blood-brain barrier function was evaluated at the same time by serum albumin/CSF albumin quotient and isoelectric focusing. The IgG index was found elevated in 73.3% of definite multiple sclerosis patients, while oligoclonal IgG bands occurred in 90%. In the other neurological diseases the IgG index was abnormally increased in 35.1% but IgG bands were present only in cerebrospinal fluid (CSF) in 1.5% and both in the CSF and serum in 7% of patients.The high capacity of isoelectric focusing to detect IgG oligoclonal bands in the CSF is pointed out as an extremely useful diagnostic tool in multiple sclerosis.ZusammenfassungDas Vorhandensein von IgG-Synthese im Nervensystem wurde mittels IgG-Index-Determination und mittels Isoelektrischer Fokalisation untersucht, und zwar bei 30 Fällen mit eindeutig diagnostizierter Multipler Sklerose, bei 15 Fällen, in denen Multiple Sklerose wahrscheinlich ist, und bei 128 Patienten, die andere neurologische Erkrankungen aufwiesen. Gleichzeitig wurde die Blut-Hirn-Schrankenfunktion untersucht mittels des Albuminserums des CSF-Albumin-Quotienten und Isoelektrischer Fokalisation. Es zeigte sich, daß der IgG-Index bei 73,3% der eindeutig diagnostizierten Multiple-Sklerose-Patienten erhöht ist, während bei 90% der Patienten das Vorhandensein von oligoclonalen Zonen nachweisbar war. Was die anderen neurologischen Erkrankungen betrifft, so war in 35,1% der Fälle der IgG-Index abnormal erhöht, während IgG-Bänder im Liquor nur bei 1,5% sowie beide bei 7% der Patienten im Liquor und im Serum vorhanden waren. Bei der Diagnose Multiple Sklerose wird der Isoelektrischen Fokalisation der höchste diagnostische Wert zugesprochen, um IgG-oligoclonale Bänder im Liquor nachzuweisen.

Is progressive multifocal leukoencephalopathy associated with intrathecal IgG synthesis

Examination of the cerebrospinal fluid by isoelectric focusing was done in 2 cases of progressive multifocal leukoencephalopathy (PML). The diagnosis was confirmed at autopsy in one case. IgG oligoclonal bands were found in both, suggesting that there was intrathecal immunoglobulin synthesis. Production may be due to a specific intrathecal response to papova virus or to IgG oligoclonal formation by lymphoma cells confined to the central nervous system or to a specific immune response to tumor cell antigens.

Neuromuscular diseases associated with benign monoclonal gammopathy: STUDY OF CSF AND SERUM PROTEINS BY ISOELECTRIC FOCUSING

In over 1200 cases examined by isoelectric focusing of CSF and serum proteins, 4 patients had benign monoclonal gammopathy. These patients were affected by amyotrophic lateral sclerosis of bulbar onset, sensitive neuropathy of Thevenard type, myasthenia gravis and limb‐girdle muscular dystrophy, respectively. The difficulty to relate the differences in the clinical features of these cases to a common physiopathological mechanism and the incidence of this finding (0.4% in 873 cases ranging from 21–60 years of age) do not seem to suggest any pathogenetic relationship between benign monoclonal gammopathy and the different neurological disorders encountered in these patients.

Clinical and CSF Findings in Multiple Sclerosis Patients with or without IgG Oligoclonal Bands at Isoelectric Focusing Examination of CSF and Serum Proteins

The IgG oligoclonal bands in CSF can be found in high percentage of MS patients but the existence of a limited number of cases with CSF normal IgG profile is known as well. In the present study 63 out of 70 patients with definite MS and 24 out of 35 with probable MS had oligoclonal bands in the CSF at the isoelectric focusing examination. The 18 patients with normal CSF IgG pattern did not show any statistically significant difference as concerns the age at onset and the duration of the disease, the functional disability, the course of the disease and the quantitative CSF parameters (IgG index, IgG synthesis and serum/CSF albumin quotient). The oligoclonal pattern does not seem of value to discriminate different groups of MS patients but it remains essentially of great diagnostic importance in this disease.

Identification of the κ and λ light chains within the CSF immunoglobulin region in multiple sclerosis and subacute sclerosing panencephalitis by immunofixation after isoelectric focusing

SummaryThe distribution of the κ and λ light chains within the cerebrospinal fluid (CSF) IgG oligoclonal bands of 15 multiple sclerosis (MS) and 3 subacute sclerosing panencephalitis (SSPE) patients has been studied by isoelectric focusing (IF) followed by direct immunofixation. The oligoclonal bands demonstrated by IF were both homogeneous and heterogeneous with regard to the light chain type; in the MS patients there was a prevalence of heterogeneous bands and of homogeneous bands of λ type, while in SSPE a slight prevalence of κ homogeneous bands was found. The κ homogeneous bands were preferentially disposed in a more cathodal position in respect to λ IgG bands; in some cases bands probably constituted only of free light chains of λ type were found. Finally, in some cases, the oligoclonal IgG pattern was more distinct after immunofixation than after basal IF.ZusammenfassungDie Verteilung von leichten κ- und λ-Ketten in den oligoklonalen IgG-Fraktionen des CSF ist bei 15 MS- und 3 SSPE-Patienten durch isoelektrische Fokussierung (IF) und anschließende direkte Immunofixierung untersucht worden.Die durch IF hervorgehobenen oligoklonalen Fraktionen waren sowohl homogen als auch heterogen hinsichtlich des Typs der leichten Ketten; bei den MS-Patienten waren die heterogenen und homogenen IgG-Fraktionen des Typs λ vorherrschend, während bei den SSPE-Patienten die homogenen IgG-Fraktionen des Typs κ leicht vorherrschend waren.Die homogenen IgG-Fraktionen des Typs κ waren im Vergleich zu den IgG-Fraktionen des Typs λ vorzugsweise in einer kathodennaheren Position angetroffen worden; in einigen Fällen wurden IgG-Fraktionen gefunden, die wahrscheinlich nur aus freien leichten Ketten des Typs λ bestehen.Schließlich waren in einigen Fällen die oligoklonalen IgG-Fraktionen nach der Immunofixierung deutlicher als nach der IF.

Comparative Doppler Directional Sonographic and Ophthalmodynamographic Study in the Occlusion of the Internal Carotid Artery

In the occlusion of the internal carotid artery, the change occurring in the internal-external carotid pressure gradient may cause flow reversal within the ophthalmic artery and its branches. Ophthalmodynamographic, Doppler directional sonographic and angiographic investigations were performed in 21 cases (11 total occlusions, 6 severe and 4 moderate stenoses of the internal carotid artery). The authors compare the results of these investigations and discuss the practical importance of Doppler directional sonography and the significance of flow reversal in the ophthalmic artery.

A Case of Late Onset Lipid Storage Myopathy

A 65-year-old woman showed the clinical picture of a myogenic myopathy, confirmed electromyographically. Detailed histopathological, histochemical and ultrastructural studies were performed on deltoid and quadriceps femoris biopsy specimens. Many muscle fibres were found to contain vacuolar spaces with lipid droplets: perivascular inflammatory changes were also observed. The affected fibres were consistently type I. Under the electron microscope the fibres contained large numbers of lipid droplets, usually next to normal mitochondria. A slight improvement occurred after prednisone therapy.