Neurons

Guillain-Barré syndrome: A review

Arthit Udomkaewkanjana, Gan Tangsrisuk — Tue, 30 Jun 2020 00:00:00 -0500

Introduction

Guillain-Barré syndrome (GBS) is a genetic condition that represents the most common cause of the acute flaccid symmetrical weakness of the limbs and areflexia that typically occurs within a month. The etiology and pathogenesis remain largely mysterious and despite immunotherapy, the syndrome leads to death or serious disability in 9-17 percent of cases. Guillain-Barré syndrome is related to Campylobacter infection in terms of etiology but the disease occurs in less than 0.1 per cent of infections. Activated macrophages and T cells, as well as serum antibodies to gangliosides, are identified in terms of pathogenesis but their meaning is unclear. Antiganglioside antibodies are present in 25 percent of patients with standard acute demyelinating GBS; 95 percent of patients in Miller-Fisher syndrome have antibodies to GQ1b ganglioside. The Guillain-Barré syndrome is a heterogeneous disease with various subtypes and recent evidence pointing to the role of immunohistochemical methods in ganglioside epitopes. GBS derives from a permissive genetic background from which disease is caused by environmental factors, including pathogens, vaccination and the effect of aging. Over 10 white cells in cerebrospinal fluid are likely to raise questions about alternative diagnoses like HIV. There are several treatment options, including plasma exchange and intravenous administration of the immunoglobulin. Most cases can be resolved without sequelae, but those that don't will leave substantial residual debility behind.

Conclusion

Biological therapies currently underway, which includes monoclonal antibodies directed at components of the supplement pathway. Individuals are encouraged to communicate with their general practitioner and neurologist or to contact the GBS Support Group's information pages for general guidance on vaccine use after GBS.

Multiple myeloma as cerebral venous sinus thrombosis: An unusual case report

Arman Parastui, Jasper Hatami — Tue, 30 Jun 2020 00:00:00 -0500

Introduction

Cerebral venous sinus thrombosis is a common condition associated usually with hypercoagulable states such as puerperium and inherited as well as acquired coagulopathies.

Case presentation

A 67-year old male presented with acute onset, moderately severe headache of 2 weeks duration without vomiting, fever, neck pain or stiffness. He was admitted in a confused state following two episodes of left focal seizures with secondary generalization.

Conclusion

Cerebral venous sinus thrombosis is an unusual presenting manifestation of multiple myeloma and the latter should be considered in the differential diagnosis of cerebral venous sinus thrombosis.

Myasthenia Gravis: An unusual case report with rare presentation

Agafya Sotiropoulou — Tue, 30 Jun 2020 00:00:00 -0500

Introduction

Myasthenia gravis (MG) is part of a continuum of autoimmune diseases that damage neuromuscular junctions by anti-acetylcholine receptor antibodies. It is a relatively rare disease with a greater prevalence in women. The classical presentation is diplopia or ptosis fluctuating and, uncommonly, dysphagia or dysphonia. While this condition is rare, it can affect any muscle, including the muscles of the neck or of the proximal limb. No documented cases of MG exhibiting as isolated neck weakness.

Case presentation

A 72-year-old female patient had neck weakness associated with a slight pain that intensified gradually during the day. Examination indicates only reduced tension of the cervical muscle motor strength. Anti-acetylcholine receptor antibodies The anti-acetylcholine receptor binding antibody was 14.07 nmol / L, 57 per cent blocking antibody, and 78 per cent modulating antibody, which is consistent with a myasthenia gravis diagnosis. The patient was prescribed Regonol, which improved her neck weakness. The patient was followed up, she returned to her previous baseline lifestyle with no clinical complications.

Conclusion

MG usually occurs in middle-aged female populations but can rarely also occur in elderly people with atypical symptoms. Clinicians should have a high index of myasthenia suspicion showing fatigued muscle exhaustion to minimize investigation costs and morbidity.