Polyradiculopathy secondary to severe hypertriglyceridemia.

A 74-year-old man presented with a subacute severe thoracic polyradiculopathy affecting the T4-T8 dermatomes bilaterally. Extensive investigation demonstrated markedly raised triglyceride levels of 44 mmol/L (<1.7). The patient's unique presentation is discussed alongside a review of triglyceride-induced neurotoxicity and therapeutic management.

Encephalomyeloradiculopathy associated with Epstein-Barr virus: primary infection or reactivation?

INTRODUCTION: Encephalomyeloradiculopathy (EMR) is a new syndrome, characterized by extensive involvement of the nervous system at different levels, including brain, medulla and spinal roots. We describe a patient presenting with prodromal febrile illness, followed by a wide infection of the nervous system with transverse myelitis and less severe meningitis, encephalitis and polyradiculopathy. The patient was treated with high-dose corticosteroids, antibiotics and acyclovir; in spite of therapy his condition improved very slowly, with severe neurological sequelae. MATERIAL AND METHODS: Antiviral antibodies were searched for in serum and cerebrospinal fluid (CSF) by commercially available ELISA kits. Viral investigations were performed by cell culture isolation and search for viral antigens, and genomic nucleic acids were investigated by polymerase chain reaction (PCR). RESULTS: Virological and serological studies evidenced a primary infection by cytomegalovirus (CMV), possibly responsible for the prodromal illness, persisting in the course of the disease. PCR performed in the peripheral blood mononuclear cells (PBMCs), DNA collected early and in the CSF drawn 30 days after the onset of the disease showed Epstein-Barr virus (EBV) DNA. The serum panel of EBV antibodies was typical of an intercurrent virus reactivation, more than of a primary infection. CONCLUSION: EBV is known to be highly infectious for the nervous system, in this case of EMR the presence of DNA sequences in the PBMCs and CSF suggests that EBV plays a role in the development of this newly described syndrome.

Encephalitogenic peptide and platelet aggregation in multiple sclerosis.

Platelet aggregation (PA) stimulated by encephalitogenic peptide (EP) and PA induced by ADP were measured in 83 multiple sclerosis (MS) patients and 70 control subjects with other neurological diseases (OND). EP-stimulated PA was significantly increased in MS patients as compared with the controls. There was no significant difference in ADP-induced PA between patients with MS and OND. The results are discussed in terms of EP-stimulated platelets playing a role in the pathogenesis of MS by affecting the venular permeability of the brain.

[Serum lipids in various polyneuropathies]. / Lipidy surowicy krwi w niektórych polineuropatiach.

Serum lipids were determined in 40 cases of polyneuropathy including 17 cases of Charcot-Marie-Tooth peroneal muscular atrophy, 7 cases of Dejerine-Sot-tas polyneuropathy, 7 cases of Guillain-Barré syndrome, 2 cases of diabetic polyneuropathy, 2 cases of toxic polyneuropathy and 5 cases of polyneuropathy of unclear aetiology. In the studied material shifts were observed in the composition of serum lipids, in particular, sphingomyelin and cholesterol esters. No correlation was found beteen the type of lipid pattern changes and the clinical syndrome, type of histological changes in the sural nerve, the age at onset, as well as the duration of the disease. The probable mechanism of development of changes is discussed.

Evaluation of the blood-CSF barrier by protein gradients and the humoral immune response within the central nervous system.

A linear correlation was found between the serum/cerebrospinal fluid (CSF) concentration ratios of albumin, caeruloplasmin and alpha2-macroglobulin and their hydrodynamic radii in a semilogarithmic plot. This protein gradient is used as a parameter to evaluate the blood-CSF barrier under normal and pathological conditions. Irrespective of vastly different transfer rates, the ratio/size permeation curves of proteins at the blood-CSF barrier and the blood-lymph barrier have comparable characteristics. Therefore the protein gradients found in various disease states are interpreted by means of Renkin's general law of lymph formation. Declined gradients are caused either by an increased permeability of the barrier sites or by a decreased turnover rate of the CSF within the compartment punctured. The concentration ratios of immunoglobulins are related to the gradient that is constructed with the ratios of the barrier-indicative marker proteins. As judged by comparative disc electrophoresis of serum and CSF, those disease states that are dominated by barrier impairment are used to establish the range of concentration ratios, compatible with a passive immunoglobulin transfer in any condition in which the barrier is disordered. A mathematical approach is described, which allows the quantitative evaluation of the minimal immunoglobulin portion that is synthesized within the central nervous system.

Antibodies to Epstein-Barr virus and cytomegalovirus in Guillain-Barre syndrome.

Twenty-three patients with Guillain-Barré syndrome (GBS) were investigated for serum antibody titres against Epstein-Barr virus capsid antigen (EBV-VCA), cytomegalovirus (CMV) and herpes simplex virus type 1 (HSV1). The geometric mean EBV-VCA titre was 45 in GBS, compared with 15-22 in healthy adults and 60 in sarcoidosis. Nine of the 15 GBS patients (60%) from whom multiple serum specimens were available, displayed a four-fold variation in antibody titre to EBV-VCA during the course of their disease. The geometric mean titres against CMV and HSV were also slightly higher in GBS compared with controls, but no titre variations were observed. Serial samples of both serum and cerebrospinal fluid (CSF) were available from 2 patients. Serum antibody titres against EBV were significantly higher in both during the initial phase of the disease. Serum antibody titres against CMV were also significantly elevated in 1 patient, but not until the second month after onset. The CSF antibody titres against EBV were higher in the beginning of the disease compared with the values during convalescence, but the titres observed were low. No measurable CSF antibody titres against CMV were found. The observation of varying serum antibody titres against EBV during the course of GBS can be interpreted as primary infections or as manifestations of reactivated latent virus infections. Further studies of the pathogenetic significance of this for the aetiology of the disease are warranted.