Publicación Oficial de la Sociedad Ecuatoriana de Neurología, de la Liga Ecuatoriana Contra la Epilepsia y de la Sociedad Iberoamericana de Enfermedad Cerebrovascular

Amyotrophic Lateral Sclerosis

 

Esclerosis Lateral Amiotrófica Secundaria a Infección por VIH.

Motor neuron diseases are not frequently associated to Human Immunodeficiency Virus (HIV) infection, although some reports have mentioned this relationship, suspecting a viral involvement in the pathogenesis of this disease. A 43-years-old male with HIV diagnosis is described, who started with progressive weakness of his legs, showing further worsening 6 months later, with upper extremity and bulbar involvement. Neurophysiological studies demonstrated upper and lower motor neuron compromise in all the extremities and bulbar muscles. Other causes were ruled out. Auto-antibodies against Human Herpes Virus type 8 were positive for IgG. There were no anomalies on image studies. A change in the antiviral scheme stopped temporarily the progression of the clinical features. However, posterior withdrawal of medications due to toxic hepatitis led to worsening of signs and symptoms. This case, among the previously reported worldwide, suggests that the association between motor neuron diseases and HIV is not coincidental, this must raise suspicion in every subject with risk factors for HIV and coexistent motor neuron disease.

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Open label study of riluzole for the treatment of amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) has a 5-years mortality of 80%. Several treatment modalities have been used to delay disease progression. The aim of the currrent study was to evaluate of riluzole on clinical progression as assessed by jablecki’s scale in Mexican patients with ALS. Fifty patients with a definitive diagnosis of ALS according to El Escorial criteria were selected. To measure the usefulness of riluzole therapy, disease progression was measured before and after treatment with jablecki’s scale. Patients received a daily oral dose of 100 mg of riluzole throughout the one-year study period. For the 50 patients initially enrolled, 31 (62%) completed the study. After the one-year, monthly progression decreased to 0.5682 points per month (p<0.05). In the 14 bulbar-onset patients with spinal-onset, initial progression was 0.6702 points per month, which decreased to 0.5551 (p<0.05). In 17 patients with spinal-onset, initial progression was 0.6702 points per month, which decreased to 0.5789 (p<0.05). There were no severe side effects related to therapy. Riluzole can delay disease progression and its use should be considered in ALS patients, after making it clear to them and their families that they will not be cured, and after taking into account cost-benefit issues.

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