Neuropathic pain is notoriously difficult to manage properly, not only because of its varied nature and the absence of objective diagnostic tools but also because of extensive reciprocal neuronal interactive pathogenic mechanism from the molecular level to patient’s own psychophysical characteristics. This paper briefly reviews the pathophysiology of neuropathic pain to the level of clinicians’ interest and its potential in clinical practiceCurrent Concepts: Recent research progress now allows us to obtain a bird view of neuropathic pain pathophysiology: peripheral and central sensitization. For peripheral sensitization, a local inflammatory milieu of the injured nerve primarily drives sequential phenotypic changes, which are critical and shared by both neuropathic and inflammatory pain. Central sensitization is led either by the hyperexcitability of the second-order afferent neuron itself or loss of physiological inhibitory control of the transmission of pain signal to the higher nervous system. Peripheral and central sensitization work synergistically but can also introduce neuropathic pain alone.Discussion and Conclusion: The cause of neuropathic pain is diverse, and understanding of its pathophysiology is still insufficient to realize a mechanism-based approach to clinical phenotypes or therapeutic applications. In dealing with chronic neuropathic pain, it is highly desirable to assess key aspects of a patient’s pain based on a plausible mechanism and select the best management method accordingly.

Medicine places its ultimate purpose at prolonging people’s healthy lives. Health-related quality of life (HRQoL) has emerged as one of the key measures of medical practice. HRQoL, the most important patient-reported outcome, should include not only functional status and disability as result of a disease but also patient’s handicaps and restriction of social participation as result of the disease. Neuromuscular disorders as a whole comprise of wide constellation of various symptoms and signs, which in turn, affect negative influence on patients’ functional status, psychological well-being, and tend to restrict patients’ financial and social achievement. The most influential HRQoL measures, either generic or neuromuscular disease-specific, will be presented and discussed. Medical Outcomes Study Short Form 36-Item, EuroQoL-5 Dimensions, Individualized Neuromuscular QoL Questionnaire, Norfolk Quality of Life Questionnaire-Diabetic Neuropathyare among the lists.

Neuropathic pain is notoriously difficult to manage properly, not only because of its varied nature and the absence of objective diagnostic tools but also because of extensive reciprocal neuronal interactive pathogenic mechanism from the molecular level to patient’s own psychophysical characteristics. This paper briefly reviews the pathophysiology of neuropathic pain to the level of clinicians’ interest and its potential in clinical practiceCurrent Concepts: Recent research progress now allows us to obtain a bird view of neuropathic pain pathophysiology: peripheral and central sensitization. For peripheral sensitization, a local inflammatory milieu of the injured nerve primarily drives sequential phenotypic changes, which are critical and shared by both neuropathic and inflammatory pain. Central sensitization is led either by the hyperexcitability of the second-order afferent neuron itself or loss of physiological inhibitory control of the transmission of pain signal to the higher nervous system. Peripheral and central sensitization work synergistically but can also introduce neuropathic pain alone.Discussion and Conclusion: The cause of neuropathic pain is diverse, and understanding of its pathophysiology is still insufficient to realize a mechanism-based approach to clinical phenotypes or therapeutic applications. In dealing with chronic neuropathic pain, it is highly desirable to assess key aspects of a patient’s pain based on a plausible mechanism and select the best management method accordingly.

Medicine places its ultimate purpose at prolonging people’s healthy lives. Health-related quality of life (HRQoL) has emerged as one of the key measures of medical practice. HRQoL, the most important patient-reported outcome, should include not only functional status and disability as result of a disease but also patient’s handicaps and restriction of social participation as result of the disease. Neuromuscular disorders as a whole comprise of wide constellation of various symptoms and signs, which in turn, affect negative influence on patients’ functional status, psychological well-being, and tend to restrict patients’ financial and social achievement. The most influential HRQoL measures, either generic or neuromuscular disease-specific, will be presented and discussed. Medical Outcomes Study Short Form 36-Item, EuroQoL-5 Dimensions, Individualized Neuromuscular QoL Questionnaire, Norfolk Quality of Life Questionnaire-Diabetic Neuropathyare among the lists.

We present a Korean case of Hirayama disease with its typical neuroradiological findings of forward displacement of cervical dural sac and compression of the lower cervical cord during neck flexion. A 15-yr-old boy was presented with a one-year history of progressive weakness and atrophy affecting bilateral hands and forearms. The electrodiagnostic findings were compatible with the lesion of the anterior horn cells at the C7, C8, and T1 spinal segments. With neck flexion, cervical magnetic resonance imaging (MRI) showed the anterior shifting of the lower cervical dural sac resulting in the cord compression of those segments. Presumably, this disease might have been prevalent in Korea frequently under the diagnosis of "benign focal amyotrophy". In this regard, we discuss the clinical importance of cervical MRI with neck flexion and anticipate the increasing reports of the case substantiated by its characteristic radiological features.
Adolescent ; Cervical Vertebrae ; Humans ; *Magnetic Resonance Imaging ; Male ; Motor Neuron Disease/complications/pathology ; Research Support, Non-U.S. Gov't ; Spinal Cord Compression/*etiology/*pathology ; Spinal Muscular Atrophies of Childhood/*complications/*pathology

Pseudohypoparathyroidism (PHP) is a rare clinical syndrome characterized by hypocalcemia, hyperphosphatemia and increase of serum parathyroid hormone in association with unique clinical features. We recently experienced a typical PHP type Ia patient who presented with recurrent seizure and muscle spasms and electroencephalogram (EEG) showed generalized spike-and-wave discharges. With the correction of hypocalcemia, seizures did not recur and epileptiform discharges disappeared. We suggest that the possibility of PHP should be considered in patients with seizures showing hypocalcemia and hyperphosphatemia.
Electroencephalography ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Parathyroid Hormone ; Pseudohypoparathyroidism ; Seizures ; Spasm

Non-ketotic hyperglycemia (NKH) is often related to various types of epileptic seizures. However, aphasic seizures associated with NKH have been rarely reported. A 60-year-old diabetic woman was admitted with language disturbance. She presented recurrent motor aphasia and EEG demonstrated ictal rhythmic discharges initiated from left frontal lobe. The seizures disappeared after introduction of carbamazepine and successful control of serum glucose. She remained seizure-free for three months after discharge. We report a case of NKH, manifested by aphasic seizures.
Aphasia, Broca ; Carbamazepine ; Electroencephalography ; Epilepsy ; Female ; Frontal Lobe ; Glucose ; Humans ; Hyperglycemia ; Middle Aged ; Seizures

Nonconvulsive status epilepticus usually presents with altered mentation without distinct manifestations of seizures. It may be related with various medical disorders. Hashimoto's encephalopathy is characterized by various neurological manifestations accompanied by high titers of anti-thyroid antibodies. Here, we report a patient with nonconvulsive status epilepticus caused by Hashimoto's encephalopathy who showed a dramatic response to steroids.
Antibodies ; Humans ; Neurologic Manifestations ; Seizures ; Status Epilepticus* ; Steroids

Paraproteinemia potentially causes peripheral neuropathy via an unknown underlying pathogenetic mechanism. We report a case of pathologically proven amyloid neuropathy with AL amyloidosis with an IgA kappa light chain, which was initially diagnosed as neuropathy associated with monoclonal gammopathy of undetermined significance. This case indicates that in cases of neuropathy with paraproteinemia, the other potential causes should be excluded by appropriate means, especially pathological evaluations.
Amyloid Neuropathies ; Amyloidosis* ; Immunoglobulin A* ; Monoclonal Gammopathy of Undetermined Significance ; Paraproteinemias* ; Peripheral Nervous System Diseases ; Polyneuropathies*

Fascicular involvement of the median nerve trunk in the upper arm is uncommon in cases of peripheral neuropathy, and its symptoms are consistent with those of anterior interosseous nerve (AIN) syndrome. We report three cases of focal anterior interosseous fascicular involvement in the median nerve trunk presenting as AIN palsy. Our report emphasizes the unique ultrasonographic and magnetic resonance imaging (MRI) features of swelling, hourglass-like constriction and torsion, and entwinement of the nerve fascicle of the dorsal region of the median nerve, which were confirmed surgically. On MRI, all patients showed denervation changes in the AIN territory, as well as in the median nerve territory, without compressing structures.