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

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Neuropatía autoinmune por anticuerpos antigangliósidos GD-1b asociada a Síndrome de Sjögren primario. Autoimmune neuropathy by anti-ganglioside GD-1b antibodies associated with primary Sjögren’s Syndrome.

The terms axonal or demyelinating, traditionally disseminated in the classification of polyneuropathies, are electrophysiological interpretations that guide us to determine the pathogenic locus where the neuropathic lesion originates; this is an objective contribution to the knowledge of the etiology of the corresponding neuropathy. However, in some circumstances, a bimodal interpretation initially does not allow determining the possibility of involvement at the axolemma level, as can be identified in some immune-mediated polyneuropathies that compromise the Ranvier node or its different domains; These neuropathies are of autoimmune origin and are characterized by the presence of anti-ganglioside antibodies directed against antigenic epitopes at this anatomical locus.

This is a case report of a 66-year-old male patient with progressive weakness in all four limbs leading to prostration over a few weeks; no prior systemic infections were reported. Initially diagnosed with Guillain-Barré Syndrome (GBS), he was evaluated with electrophysiological studies that confirmed the existence of a sensorimotor demyelinating polyneuropathy and treated with intravenous immunoglobulin (IVIg), with partial clinical improvement. Two weeks later, he was readmitted with a new episode of global muscle weakness, due to a fluctuating response of GBS to immunomodulatory treatment; therefore, he received two cycles of IVIg with little response. Complementary immunological studies confirmed a diagnosis of Sjögren’s syndrome and showed positive IgM anti-GD1b antibodies.

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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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