• Annals of Clinical Neurophysiology
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• v.19 no.1
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• pp.74-76
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• 2017

Muscle specific tyrosine kinase (MuSK) myasthenia gravis (MG) is a rare subtype of MG, which is immunologically distinct and differential therapeutic response. Though MG is often associated with other autoimmune disorders or malignancy, concurrence of other disease and MuSK MG has been infrequently reported. We present a patient of MuSK MG associated with multicentric Castelman disease.

• Annals of Clinical Neurophysiology
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• v.22 no.1
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• pp.8-12
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• 2020

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy with heterogeneous features. Appropriate treatment will produce a favorable outcome, but a poor treatment response and severe disability have also been reported. The roles of the clinical phenotypes and electrophysiological features of CIDP as well as of autoantibodies against nodal and paranodal proteins have been highlighted previously due to their association with the treatment response and long-term prognosis. This review addresses the diverse factors associated with the prognosis of CIDP.

• Annals of Clinical Neurophysiology
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• v.22 no.1
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• pp.1-7
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• 2020

Neuromuscular ultrasonography has emerged over the last decade as a useful tool for diagnosing peripheral nerve disorders. It has been studied extensively with a particular focus on the assessment of compression neuropathies. Neuromuscular ultrasonography complements electrodiagnostic studies well by visualizing both the nerve anatomy and surrounding structures, providing useful data that cannot be obtained using the latter methodology only. This review article summarizes and synthesizes the literature focusing on the diagnostic role of neuromuscular ultrasonography in common compression neuropathies of the upper limb.

• Annals of Clinical Neurophysiology
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• v.20 no.1
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• pp.3-11
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• 2018

Skin biopsy and staining the specimens with immuno-reactive markers has been proven to be a useful method to demonstrate the pathologic status of small nerve fibers. Quantification of intraepidermal nerve fiber density using anti-protein gene product 9.5 antibody is a standard method to diagnose small fiber neuropathy. Skin biopsy also makes it possible to differentiate the nerve fibers according to their function by using different markers. Quantification of dermal structures with different types of nerve fibers could reveal the pathophysiologic mechanism of the disease state.

• Annals of Clinical Neurophysiology
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• v.22 no.2
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• pp.67-74
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• 2020

Vestibular-evoked myogenic potentials (VEMPs) are useful for evaluating the vestibulocollic reflex arising mostly from the saccule and the vestibuloocular reflex originating from the utricle. VEMPs can vary with the characteristics of the applied stimuli and the effects of aging and diseases. VEMPs have been found to be useful for diagnosing superior canal dehiscence, but their usefulness for other clinical disorders remains unclear. This review discusses the principles of VEMP tests and summarizes the findings for VEMPs in common vestibular disorders.

• Annals of Clinical Neurophysiology
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• v.22 no.2
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• pp.109-111
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• 2020

Germ-cell tumors (GCTs) are common in the central nervous system. GCTs are highly sensitive to radiotherapy and chemotherapy and can be cured without radical surgery. However, this tumor produces nonspecific imaging findings, and its rarity can make diagnosis challenging. Here we report a case in which the first diagnosis was uncertain and follow-up imaging revealed intramedullary germinoma. The patient underwent chemotherapy and radiotherapy after the diagnosis.

• Annals of Clinical Neurophysiology
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• v.22 no.2
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• pp.125-128
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• 2020

Focal eosinophilic myositis (FEM) is the most limited form of eosinophilic myositis that commonly affects the muscles of the lower leg without systemic manifestations. We report a patient with FEM who was studied by magnetic resonance imaging and muscle biopsy with a review of the literature.

• Annals of Clinical Neurophysiology
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• v.21 no.1
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• pp.66-69
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• 2019

Orthostatic hypotension (OH) is commonly associated with autonomic failure in the peripheral nervous system. Less often it is related to central lesions in brainstem and cerebellum. We describe a patient with OH associated with tuberculosis meningoencephalitis involving the brainstem including rostral ventrolateral medulla. This is the first case of OH resulting from focal lesions in the dorsal medulla in a patient with meningoencephalitis.

• Annals of Clinical Neurophysiology
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• v.21 no.1
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• pp.44-47
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• 2019

Zoster-associated limb paresis is a relatively uncommon complication of herpes zoster that is characterized by focal motor weakness. Awareness of this disorder is important to avoid unnecessary invasive investigations and to ensure appropriate treatment. We report a case of a herpes zoster involving the femoral nerve.

• Annals of Clinical Neurophysiology
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• v.23 no.1
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• pp.29-34
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• 2021

New therapeutics in neurology are expanding at an unprecedented pace. In addition to the classic enzyme-replacement therapies, monoclonal antibodies are increasingly being used to modulate autoimmunity. RNA therapeutics are an emerging class, together with gene and cell therapies. The nomenclature of international nonproprietary names helps us to recognize these new drugs according to their class and function. Suffixes denote major categories of the drug, while infixes provide additional information such as the source and target.