This case illustrates the need to define a set of standards for presentation of patients with an atypical form of progressive myoclonus epilepsy, and for considering the potential role of surgery and treatment with chemotherapy. We suggest that this new entity should be included among the other idiopathic progressive myoclonus epilepsies with the same spectrum phenotype.
LS is often accompanied by a wide variety of systemic illnesses, and is likely underdiagnosed. Most of the LS patients in these series were also diagnosed with SLE during a protracted course of illness. LS is believed to represent a subgroup of patients having systemic disorders that cause or worsen their symptoms.
The most common treatments for the rare disorder of Libman-Sacks disease are cholestasic and cholinesterase inhibitors and corticosteroids. The use of neuroleptic drugs and intravenous immunoglobulin in the treatment of infantile neuronal intractable dystonia is also an important practice. Neuroleptic drugs work mostly by improving muscle contractions. The primary objective of these drugs is to eliminate the abnormal posturing of the muscle spindles. Immunoglobulin (IVIG) is a plasma-derived product that was used to treat infantile neurodegenerative disorders such as infantile spasms, Reye's syndrome, and myoclonic epilepsy.
The clinical features of this unusual entity include a slow progression of cerebral atrophy, a relatively good response to immunomodulatory therapy, prolonged disease-free duration and absence of cerebellar ataxia.
LBD claims are more common among female patients, the elderly, and African Americans. The age-adjusted CIR for Libman-Sacks disease increased sharply from 1987 to 1992, and the trend has slowed since. Although LBD claims are rare in children, the trend was increasing between 1990 and 1997. The mean age to clinical presentation increased from 62 to 65 y over the same time frame. There is still a paucity of information available on the frequency and duration of LBD in the US.
The primary, and perhaps the sole etiology of the disease, is a single CAGCCAGG polyglutamine expansion in intron 9 of ataxin-3 gene locus. The expansion most probably causes a loss of function of the ataxin-3 protein, leading to cellular dysregulation and the clinical features seen in the disease.
Recent findings of this study show that vib7734 is more effective than a placebo in improving the symptoms and quality of life of patients with Libman-Sacks syndrome (MSS) who have failed conventional medicines.
The average age of onset between male and female individuals of LMD appears to be about 40 years old and can occur in either a sporadic or familial fashion. The main factors that influence the age of LMD onset appear to be gender, age of the individual, and/or a combination of both.
The VIB7734 study suggests that the efficacy of the VIB7734 treatment could be attributed to the effect of VIB7734 through two different mechanisms. VIB7734 could reduce inflammation, reduce atrophy, and improve quality of life to relieve the symptoms caused by the AD. The effect of VIB7734 can be assessed by an improvement of cognitive function, global change and quality of life in relation to clinical improvement of AD patients.
There are a few therapies available for libman-sacks disease including splenic infusions, IV methotrexate in patients who do not respond to intravenous chemotherapy and a splenic irradiation. In order to give the patient the greatest number of treatments, the best therapies should be applied.
vib7734 had not been tested, even in rats. The efficacy of vib7734 in treating the symptoms of this disorder has been unproven. Nevertheless, the number of patients with symptomatic manifestations of this disease is small and the possibility that vib7734 will be effective in this disease cannot be ruled out. In view of the low risk for this disorder and the lack of an alternative treatment, the study of vib7734 in humans could be worthwhile.
The current results confirm the findings of previous studies, thus demonstrating a similar cause for LSD. This could relate to specific antigenicity, autoimmune phenomena or some other unknown etiological agent.