Jean Demonty

Pathogenic Tracks in Fatigue Syndromes

This review analyses the recent literature devoted to two related fatigue syndromes: chronic fatigue syndrome (CFS) and acute onset postviral fatigue syndrome (PVFS). The articles are grouped into five pathogenic tracks: infectious agents, immune system, skeletic muscle, hypothalamo-pituitary-adrenal (HPA) axis and psychiatric factors. Although a particular infectious agent is unlikely to be responsible for all CFS cases, evidence is shown that host-parasite relationships are modified in a large proportion of patients with chronic fatigue. Antibody titres against infectious agents are often elevated and replication of several viruses could be increased. Chronic activation of the immune system is also observed and could be due to the reactivation of persistent or latent infectious agents such as herpes viruses (i.e. HHV-6) or enteroviruses. It could also be favorised by an impaired negative feedback of the HPA axis on the immune system. A model is proposed where the abnormalities of the HPA axis are primary events and are mainly responsible for a chronic activation of the immune system which in turn induces an increased replication of several viruses under the control of cellular transcription factors. These replicating viruses together with cytokines such as TNF-alpha would secondarily induce functional disorders of muscle and several aspects of asthenia itself.

CHOREA ASSOCIATED WITH ANTI-PHOSPHOLIPID ANTIBODIES: CASE REPORT

Abstract A seventeen year-old boy developed left sided chorea in a few days, subsequently involving the four limbs. Although he presented a marfanoid phenotype, genetic analysis of the Fibrillin 1 was normal. The genes for familial chorea and Huntington’s disease were also negative. Biological tests showed normal serum homocystein, but revealed very high levels of anti-β2-GP1 IgG, anticardiolipin and lupus anticoagulant, which remained at similar values for a period of over three months. Electroencephalogram and cerebral magnetic resonance imaging (MRI) showed no abnormalities. Brain PET-scan disclosed bilateral striatal hypermetabolism. The patient was treated with methylprednisolone and low dose of acetylsalicylic acid. He improved markedly after six weeks of treatment, and choreic movements disappeared completely after two months. A control PET-scan performed at this time showed reversion of striated hypermetabolism to a normal pattern. The pathogenic aspects of this relatively rare case of chorea are discussed.

Lesions cerebrales demyelinisantes decouvertes chez un patient immunocompetent VIH-1+.

We report the case of a 32-year-old immunocompetent HIV patient, presenting with acute demyelinating leukoencephalopathy. The patient displayed clinical and radiological features similar to multiple sclerosis. Histology revealed inflammation with necrosis, demyelination and destruction of axons. Serum tests were negative for various infectious agents as well as specific cultures and PCR. Corticotherapy and many antibiotic treatments failed. Resorption of the lesions occurred after partial excision and highly-active antiretroviral therapy (HAART). No recurrence was noted. This demyelinating cerebral disease was considered as the primary manifestation of HIV infection. HIV implication in the genesis of the process and its perpetuating this condition was suspected, but the mechanism is unclear. Dysimmune consequences related to the early course of HIV infection could be prevented by antiretroviral treatment. This study was the first description of tritherapy effectiveness on HIV related multiple sclerosis (MS)-like illness.