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CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY, AN ATYPICAL CASE
Abstract
Abstract Body
Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an imuno-mediated acquired, neuropathy that targets the peripheral nerves and nervous roots. It is characterized by a progressive or relapse-remitting course, electrodiagnostic or pathologic features of demyelination and glucocorticoid responsiveness. It has multiple variants who share the same pathophysiology of inflammatory demyelination.
Case: This case refers to an 83 year old male who progressively develops symptoms of lower limbs parestesia and hyposthesia with associated balance deficits. His neurological examination revealed mild proprioceptive hyposthesia and hipopalesthesia aswell as sensitive ataxia of the lower limbs. He also showed a positive romberg test and at a later stage diminished reflexes of the lower limbs. Cerebrospinal fluid analysis revealed elevated protein levels, normal white cell count and one band imunofixation as well as slightly elevated Imunoglobulin levels. The head CT scan did not had any remarkable findings and the outpatient electromiography was compatible with demyelinating motor-sensitive peripheral neuropathy. A later hospital electromiography revealed acentuated CIDP.
Discussion: Although it is still necessary to rule out the presence of other immune-mediated neuropathy such as IgM gammopathy Neuropathy, we hereby present an interesting case of a possible rare type of CIDP, the distal and sensory-predominant variant. This variant has a more indolent course characterized by sensory disfunction of the lower extremities, absent reflexes and may even present with ataxia in addition to autonomic symptoms.
Conclusion: Despite being a rare entity, the diagnosis of CIDP is important and must be taken into account in the diagnosis of a polyneuropathy specially due to its responsiveness to immunomodulatory therapies.