The limb girdle muscular dystrophy (LGMD) is a heterogeneous group of genetically determined disorders characterized by progressive weakness and atrophy predominantly impacting the shoulder and pelvic girdles. Their classification has been revised in recent years because of advances in understanding the molecular basis of the various subtypes of LGMD. The similarities of clinical features and muscle pathology between the diverse subtypes may cause confusion and difficulty relative to differential diagnosis by clinicians. The recognition of the characteristics of each of the subtypes and approaches to precise diagnosis based on available biochemical and molecular testing will allow directed management for each patient by predicting specific complications such as cardiac or respiratory systems, and in the future will be a beginning for specific gene and protein based therapies. Through the extensive review of literature, recent developments of LGMD regarding diagnosis and treatment are summarized.

Electroencephalogram (EEG) is a representative diagnostic tool in epilepsy. However, there are several points of debate on the role of EEG in diagnosis and management of epilepsy. We suggest that EEG has some limitations for differential diagnosis from nonepileptic episodic diseases, classification of epilepsy, prediction of recurrence, and evaluation of treatment response. Interictal EEG cannot diagnose or exclude epilepsy because interictal epileptic discharge (IED) is frequently absent in epilepsy and can appear in nonepileptic conditions. Although EEG is helpful in classification of epilepsy, focal spikes in generalized epilepsy and secondary bilateral synchrony in localization related epilepsy cause interrater disagreement. It is controversial whether EEG predicts recurrence after the first seizure in adults. The predictive value of EEG in antiepileptic drug (AED) withdrawal is not absolute. The prognosis after AED withdrawal depends on epilepsy syndrome. Many studies could not confirm the value of EEG in assessing the treatment response. After all, epilepsy is clinically diagnosed and assessed. Interictal EEG alone does not provide decisive information and routine follow-up of EEG is not recommended.

Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has beenused in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

Background
Testosterone is reported to have neuroprotective effect in various neurological diseases. Recently, the mechanism involved in nitric oxide (NO)-mediated motor neuron death is under extensive investigation. The Cu/Znsuperoxide dismutase (SOD1) mutations has been implicated in selective motor neuron death of amyotrophic lateral sclerosis (ALS) and it is said to play an important role in NO-mediated motor neuron death. However, neuroprotective effect of testosterone on motor neuron exposed to NO has rarely been studied.
Methods
Motor neuron-neuroblastoma hybrid cells expressing wild-type or mutant (G93A or A4V) SOD gene were treated with 200 μΜ S-nitrosoglutathione. After 24 hr, cell viability was measured by MTT assay. To see the neuroprotective effect of testosterone, pretreatment with 1 nM testosterone was done 1 hr before S-nitroglutathione treatment. To study the mechanism of protective effect, 20 μΜ flutamide (androgen receptor antagonist) was also pretreated with testosterone 1 hr before S-nitroglutathione treatment.
Results
S-nitrosoglutathione showed significant neurotoxic effect in all three cell lines. Percentage of cell death was somewhat different in each cell line. 1 nM testosterone showed neuroprotective effect in G93A and wild-type cell line. In A4V cell line, testosterone did not showed neuroprotective effect. The neuroprotective effect of testosterone was reversed by 20 μΜ flutamide.
Conclusions
These results indicate that testosterone induces neuroprotection in NOmediated motor neuron death directly through the androgen receptor. This neuroprotective effect of testosterone varies according to the types of SOD1 gene mutation. These data suggest that testosterone may be of therapeutic value against ALS.