Multifocal motor neuropathy with Anti-GM1 antibody: A Filipino case report

@#As far as we are aware of, we report the fi rst documented case of a 51-year-old Filipino female with multifocal motor neuropathy who presented with asymmetric weakness, unusually in the lower extremity, and confi rmed with anti-GM1 antibody. The treatment of intravenous immunoglobulin with a total dose of 2 g/kg was initiated and repeated every two months with noticeable improvement based on electromyography and nerve conduction studies. Apart from the unreported Filipino case of multifocal motor neuropathy substantiated by features in clinical, electrophysiologic, antibody testing and response to immunotherapy, the unique occurrence in a female and involving the lower extremity in this rare disorder deserve this present documentation. Multifocal motor neuropathy is seen more in males with a ratio of 2.7:1. It is described as a pure motor disease without sensory defi cits and predominantly affects the upper extremities. The diagnosis for the disorder is supported by determination of ganglioside GM1 antibodies.

Chinese guidelines for diagnosis and treatment of multifocal motor neuropathy 2019 / 中华神经科杂志

Multifocal motor neuropathy is a multiple mononeuropathy characterized by asymmetric weakness of extremities with preserved sensation and conduction blocks selectively affecting the motor fibers. Based on the consensus of experts in neuromuscular and neurophysiological area, the guideline summarizes the clinical, neurophysiological, radiological, immunological features and treatment progress in multifocal motor neuropathy for clinical reference.

Analysis of the clinical and electrophysiological characteristics of multifocal motor neuropathy / 中华内科杂志

Ten patients diagnosed with multifocal motor neuropathy ( MMN) were recruited in the Department of Neurology at Chinese PLA General Hospital from January 1, 2009 to August 31, 2015.The clinical and electrophysiological features were analyzed retrospectively .All patients complained of progressive asymmetric limb weakness , which was more severe in distal than in proximal . Five presented muscle atrophy.None had sensory disturbances .All suffered diminished or disappeared tendon reflex , whereas Babinski signs were negative .Multi-focal conduction block ( CB) was confirmed by nerve conduction studies ( NCS) in all patients and 7 showed spontaneous potentials in needle electrode electromyography .Abnormal sensory nerve conduction was seen in 3 patients.Laboratory test revealed anti-ganglioside GM1 antibody in cerebrospinal fluid (CSF) in 6 cases and elevated CSF protein in 7 cases.Limb weakness alleviated greatly in 9 cases after intravenous immunoglobulin ( IVIg) treatment.But the other one reported poor response , who had long course of disease , serious limb weakness and obvious muscle atrophy .Motor nerve damage is the most important manifestation of MMN and sensory nerve damage may also appear .NCS is essential to the diagnosis of this disease , with CB as the characteristic electrophysiological feature .IVIg is an effective treatment.

Multifocal Motor Neuropathy with Conduction Blocks During TNF-alpha Antagonist Therapy in a Patient with Spondyloarthropathy

Tumor necrosis factor (TNF)-alpha antagonist has been proven to have benefit for rheumatologic diseases. Because TNF-alpha is not only an important mediator of inflammation in human body, but plays many physiologic roles, it can cause unique adverse effects or complications related to these functions. Adverse effects involving neurological systems, such as Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy with conduction blocks (MMNCB), distal symmetric polyneuropathy, and small fibers neuropathy have been previously reported. However, only several cases of infliximab-associated MMNCB are reported. We report a case of MMNCB which developed while treating spondyloarthropathy with infliximab.

Concurrence of Multifocal Motor Neuropathy and Hashimoto's Thyroiditis

BACKGROUND: Multifocal motor neuropathy (MMN) is an immune-mediated disorder that is characterized by slowly progressive and asymmetrical weakness, but its pathophysiological mechanism is uncertain. The hypothesis that MMN is an immunological disease has been supported by the proven therapeutic effects of intravenous immunoglobulin and the detection of antiganglioside antibodies in MMN patients. The coexistence of MMN with other immune diseases has been rarely reported. CASE REPORT: A 37-year-old woman visited our hospital complaining of weakness in both hands. The clinical manifestations coincided well with MMN: predominantly distal upper-limb weakness, asymmetric involvement, a progressive course, absence of sensory symptoms, absence of pyramidal signs, and sparing of the cranial muscles. The electrophysiological findings also supported a diagnosis of MMN, with motor nerve conduction block in the median, ulnar, and radial nerves, without sensory nerve involvement. The patient was simultaneously diagnosed as having Hashimoto's thyroiditis, which is a well-known immune-mediated disease. CONCLUSIONS: The concurrence of MMN and Hashimoto's thyroiditis in our patient is significant for understanding the immunological characteristics of the two diseases.

A Case of Multifocal Motor Neuropathy with Conduction Block

Multifocal motor neuropathy with conduction block (MMN) is characterized by a slowly progressive, asymmetrical weakness of the limbs without sensory loss. A differential diagnosis from other motor neuropathies has become increasingly important as MMN has proven to be a treatable disorder. We present a 45-year-old man with longstanding weakness and atrophy of the hand muscles. Electrophysiological studies revealed a typical conduction block in the bilateral median and ulnar nerves. A cycle of intravenous immunoglobulin treatment apparently did not affect the symptoms.

Clinical and electrophysiological features of multifocal motor neuropathy / 临床神经病学杂志

Objective To study clinical and electrophysiological features of multifocal motor neuropathy (MMN). Methods The clinical features and data of electrophysiological examination from 16 patients with MMN were analyzed retrospectively.Results In the present study, the onset age was 14 to 47 years with the mean age of (28.2?2.4) years. All the 16 patients presented with unsymmetrical weakness of extremities (Ⅱ～Ⅳ grade of muscle strength) and no hypoesthesia. More serious weakness was found in the upper limbs and in the right extremities. 4 cases were accompanied with mild amyotrophy. Electromyography showed segment conduct block (CB) and slow down of conduct velocity of motor nerves in all 16 patients. Slow conduct velocity of sensory nerve was found only in one case.Conclusions MMN is an unsymmetrical peripheral neuropathy, which affects mainly on motor nerve. Nerve electrophysiology plays an important role in diagnosis of MMN and CB is the characteristic manifestation of nerve electrophysiological change in MMN.