Critical Illness Polyneuropathy (CIP) represents an acute axonal neuropathy that develops during treatment of severely ill patients and remits spontaneously once the critical condition is under control. Except for differences in the predisposing causes, it is difficult to distinguish CIP from axonal Guillain-Barré Syndrome (GBS) on purely clinical grounds. We describe a 70 years lady who develo...

Mutations of FIG4 are responsible for Yunis-Varón syndrome, familial epilepsy with polymicrogyria, and Charcot-Marie-Tooth type 4J neuropathy (CMT4J). Although loss of the FIG4 phospholipid phosphatase consistently causes decreased PtdIns(3,5)P₂ levels, cell-specific sensitivity to partial loss of FIG4 function may differentiate FIG4-associated disorders. CMT4J is an autosomal recessive neuropa...

Background: Guillain-Barré Syndrome (GBS) is the most common cause of acute, usually post-infectious, peripheral neuropathy resulting in a symmetrical, ascending paralysis. We evaluated clinical and neurophysiological features, treatment, outcomes patients with GBS our center. Methods: A retrospective chart review on admitted to King Abdulaziz Medical City, Riyadh, Saudi Arabia, from January 20...

BACKGROUND Multifocal acquired demyelinating sensory and motor neuropathies are considered autoimmune and responsive to immunotherapy. In the absence of demyelination, however, they are considered idiopathic if no other cause is found. OBJECTIVE To determine whether patients with multifocal acquired sensory and motor neuropathy of an otherwise unknown cause have antiganglioside antibodies, re...

Recent studies revealed an association between Zika virus infection and a variety of neurological disorders, including microcephaly, meningoencephalitis, myelitis, and Guillain-Barré syndrome (GBS). Following the first report of a patient diagnosed with GBS after Zika virus infection in December 2013, the number of GBS patients significantly increased in endemic countries, such as French-Polyne...

Objective To highlight treatment resistance of concurrent Guillain-Barre syndrome (GBS) and active systemic lupus erythematosus (SLE). Background Coincidence SLE GBS is an uncommon complicated course autoimmune disease. Treatment further by pregnancy exacerbation SLE. Design/Methods We report a case acute motor axonal neuropathy (AMAN) subtype in young pregnant woman her early 20s presenting wi...

Mutations in the SPG3A gene (atlastin protein) cause approximately 10% of autosomal-dominant hereditary spastic paraplegia. Most patients with an SPG3A mutation present with a pure phenotype and early-onset disease, although complicated forms with peripheral neuropathy are also reported. We report a new heterozygous S398F mutation in exon 12 of the SPG3A gene causing a very early-onset spastic ...

Multifocal motor neuropathy is a unique disease that not only challenges the electromyographer to make differential diagnosis from a fatal disease but also provides an opportunity to reconsider the mechanism of conduction block in demyelinating neuropathies. Conduction block or slowing is not always a consequence of demyelination, but can be due to membrane hyperpolarisation or depolarisation, ...

Three siblings are reported with childhood onset hereditary motor and sensory neuropathy (HMSN) and adult onset optic atrophy. Electrophysiological studies showed an axonal neuropathy and dysfunction of the retinal ganglion cells or optic nerve. The presumed mode of inheritance is autosomal recessive. This is the second family in which autosomal recessive inheritance of HMSN and optic atrophy (...

Patients with anti-myelin-associated glycoprotein (MAG)/sulfated glucuronyl paragloboside (SGPG) neuropathy associated with Waldenström macroglobulinemia show demyelinating neuropathy, but the temporal dispersion of distal compound muscle action potential (CMAP) in motor nerve conduction studies (NCS), which represents heterogeneous demyelination at the motor nerve terminal, is rare. We report ...