• Annals of Clinical Neurophysiology
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• v.10 no.1
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• pp.29-32
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• 2008

Although various criteria on the diagnosis of diabetic neuropathy are applied from trial to trial, being tailored in concert with its purpose, the utmost evidences of the diagnosis are subjective symptoms and objective signs of neurologic deficit. The application and interpretation of auxiliary electrophysiological test including nerve conduction study (NCS) should be made on the context of clinical pictures. The evaluation of the functions of small, thinly myelinated or unmyelinated nerve fibers has been increasingly stressed recently with the advent of newer techniques, e.g., measurement of intraepidermal fiber density, quantitative sensory testing, and autonomic function test. And the studies with those techniques have shed light to the nature of the evolution of diabetic neuropathy. The practical application of these techniques to the diagnosis of diabetic neuropathy in the individual patients, however, should be made cautiously due to several shortcomings: limited accessibility, wide overlapping zone between norm and abnormality with resultant unsatisfactory sensitivity and specificity, difficulty in performing subsequent tests, unproven quantitative correlation with clinical deficit, and invasiveness of some technique. NCS, as an extension of clinical examination, is still the most reliable electrophysiological test in evaluating neuropathy and gives the invaluable information about the nature of neuropathy, whereas the newer techniques need more refinement of the procedure and interpretation, and the accumulation of large scaled data of application to be considered as established diagnostic tools of peripheral neuropathy.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.535-539
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• 2002

Incontinentia pigmenti is a rare neurocutaneous syndrome characterized by vesiculobullous skin disease in neonates and infants, a noninfectious disease that should be distinguished from infectious diseases with the neonatal seizure or encephalopathy. This disease is X-linked dominant with Xq28 region abnormalities and often associated with developmental defects of the ocular, skeletal, dental, and central nervous system. Central nervous system involvement in the neonatal period, or complicated by encephalopathy, may cause severe neurologic impairment, retardation or even death. We experienced a case of incontinentia pigmenti in a three-day-old female patient who had characteristic papulovesicular skin lesions and partial seizures with secondary generalization. Histopathological examination favored the diagnosis of incontinentia pigmenti and a brain MRI showed undifferentiated white matters with periventricular nodular lesions.

• Clinical and Experimental Pediatrics
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• v.59 no.11
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• pp.456-459
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• 2016

Triple-A syndrome, also known as Allgrove syndrome, is a rare autosomal recessive disorder. The 3 features of this syndrome are achalasia, adrenal insufficiency, and alacrima. Achalasia could be the first manifestation of the triple-A syndrome; however, its etiology is unclear. Alacrima is generally asymptomatic but can be detected by obtaining patient history. Although adrenal insufficiency could have manifestations such as asthenia, it might be wrongly diagnosed as muscle fatigue. Vitamin D and calcium supplements are usually prescribed for the prevention of osteoporosis. Neurologic manifestations could be present in adults. In some individuals with this disorder, genetic examination indicates mutations in both alleles of the AAAS gene, which encodes a special 546-amino-acid protein designated ALADIN, and in chromosome 12q13. The genetic cause of the triple A syndrome in some patients who do not have an identified mutation is unknown. While very few such cases have been reported till date, one such case was presented to us as an edentulous child.

• Journal of Korean Neurosurgical Society
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• v.43 no.4
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• pp.194-197
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• 2008

Primary cerebellar glioblastoma multiforme (GBM) is a rare tumor in adults that accounts for just 1% of all cases of GBM. Due to their rarity, cerebellar GBMs are not yet completely understood about the pathogenesis and the prognosis. Here, we present a case of GBM in a 69-year-old man. Neurologic examination revealed the presence of cerebellar signs. Magnetic resonance imaging (MRI) showed a 4.5${\times}$3.6 cm-sized, ill-defined, heterogeneously enhancing mass in the left cerebellum and two patchy hyperintense lesions in the right cerebellum with minimal enhancement. After operation, glioblastoma was histologically confrimed. Postoperative radiotherapy with concomittent and adjuvant temozolomide chemotherapy was subsequently followed. Here, a case of unusual GBM in the cerebellum is reported with review of literature regarding the pathogenesis, the differential diagnosis and prognosis. There was no evidence of recurrence during postoperative one year. This patient showed a good prognosis in spite of the multiple lesions.

• Journal of Korean Neurosurgical Society
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• v.49 no.5
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• pp.284-286
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• 2011

Glossopharyngeal neuralgia is a relatively rare condition characterized by severe, paroxysmal episodes of lancinating pain in the tongue, throat, ear, and tonsil. This disorder is assumed to be due to compression of the glossopharyngeal nerve by vascular structures. A 47-year-old woman complaining of sharp and lancinating pain in the right periauricular and submandibular areas visited our hospital. Swallowing, chewing, and lying on her right side triggered the pain. Her neurologic examination revealed no specific abnormalities. The results of routine hematologic and blood chemistry studies were all within normal limits. Carbamazepine and gabapentin were given, but her symptoms persisted. Her pain was temporarily relieved only by narcotic pain medication. MRI showed an arachnoid cyst located in the right cerebellomedullary cistern extending to the cerebellopontine cistern. Cyst removal was performed via a right retrosigmoid approach. Lateral suboccipital craniotomy was performed using the right park-bench position. After opening the dura and cerebellopontine angle, the arachnoid cyst was exposed. The arachnoid cyst was compressing the flattened lower cranial nerves at the right jugular fossa. Her symptoms resolved postoperatively. Two months after the operation, she was completely free from her previous symptoms.

• Journal of Korean Neurosurgical Society
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• v.40 no.2
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• pp.110-113
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• 2006

Surgery and radiotherapy are mainly used for plasma cell neoplasm which constitutes about $1{\sim}2%$ of human malignancy. The authors carried out Fractionated Stereotactic Radiotherapy[FSRT] on the residual tumor after the subtotal removal of Intracranial plasmacytoma. A huge mass lesion was observed on MRI [magnetic resonance image] in the left anterior and middle cranial fossa of a 63-year-old man with left exophthalmus which lasted for a month, and was suspected as a meningioma with strong contrast enhancement. Extramedullary plasmacytoma was diagnosed on histopathological examination. After the surgery, FSRT was also carried out on the residual tumor which invaded the skull base. One-year follow up after FSRT showed contrast enhancement only in the left sphenoid bone on MRI, which indicated significant decrease in the size of the tumor without any abnormal neurologic deficits. We treated intracranial plasmacytoma which invaded left anterior and middle cranial fossa and surrounded cavernous sinus without cranial nerve deficit through subtotal tumor removal and FSRT.

• Journal of Veterinary Clinics
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• v.34 no.5
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• pp.377-380
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• 2017

An 8-year-old, shih-tzu female dog was referred due to neurological signs including paraparesis and back pain. On the complete blood count, hematologic analysis showed elevated leukocytosis. Serum biochemical analysis revealed elevated serum alkaline phosphatase concentration and C-reactive protein concentration. On the neurologic exam, the dog was suspected to have thoracolumbar myelopathy. On magnetic resonance imaging, there were masses within the spinal canal at L1-3 intervertebral disc space that were located dorsal to spinal cord. It was hyperintense on T1-, T2-weighted magnetic resonance images, Fluid-attenuated inversion recovery, and fat suppression images. The contrast-enhanced T1-weighted images showed no enhancement. The lesions were well circumscribed. The spinal cord was compressed and displaced ventrally by the mass. After removal of the masses via L1-L3 dorsal laminectomy, pyogranulomatous inflammation was confirmed by histopathological examination. Six months after surgery, the dog recovered uneventfully and remained fully ambulatory with no neurological deficits. This case demonstrates the utility of magnetic resonance imaging for the diagnosis of spinal canal pyogranulomatous inflammation.

• Korean Journal of Clinical Laboratory Science
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• v.46 no.1
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• pp.38-45
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• 2014

Intraoperative Neurophysiological Monitoring (INM) inspection has a very important role. While preserving the patient's neurological function be sure to safe surgery, neurological examination should thank. Cerebello pontine angle tumor surgery, especially in the nervous system is more important to the meaning of INM. In cochlear nerve, facial nerve, trigeminal nerve, which are intricate brain surgery, doctors are only human eye and brain to the brain that it is virtually impossible to distinguish the nervous system. They receives a lot of help from INM. In this paper, we examined six kinds broadly. First, the methods of spontaneous EMG and Free-running EMG, which can instantly detect a damage inflicted on a nerve during surgery. Second, methods of triggered EMG and direct nerve electrical stimulation, which directly stimulate a nerve using electricity to distinguish between nerves and brain tumors. Third, the method of knowing a more accurate neurologic status by informing neurological surgeons about Free-running EMG wave forms that are segmetalized into four. Fourth, three ways of knowing when a patient will be awaken from intraoperative anesthesia, which happens due to a weak anesthetic. Fifth, a method of understanding the structures of a brain tumor and a facial nerve as five dividend segments. Sixth, comparisons between cases normal facial nerve recovery and occurrence of a facial nerve paralysis during the postoperative course.

• Annals of Clinical Neurophysiology
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• v.4 no.1
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• pp.74-77
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• 2002

Collet-Sicard Syndrome is one of the variant of the jugular foramen syndromes in which the last four cranial nerves are involved whereas the sympathetic plexus is spared. The possible causes of these multiple lower cranial nerve palsy are variable, including metastasis of systemic malignancy to the base of skull, primary tumor of head and neck, vascular complication, trauma and so on. We experienced two men visited to our clinic with symptoms of headache, hoarsness, swallowing difficulty and showed the evidence of cranial nerve palsy on neurologic examination. Magnetic resonance imaging and computed tomography demonstrated oropharyngeal and hypopharyngeal tumor and electrodiagnostic study supported the diagnosis.

• Journal of Korean Neurosurgical Society
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• v.30 no.10
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• pp.1245-1249
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• 2001

Germinomas of the central nervous system are rare embryonal tumors(accounting for less than 1% of intracranial neoplasms) that may be located in the pineal region, in the floor of the third ventricle, or in the suprasellar area. We report a case of germinoma developed in periventricular deep white matter without pineal region tumors or suprasellar masses. The 19-year-old male patient presented with slowly progressing headache, dizziness, photophobia, and dysarthria. Initial brain MRI revealed a irregular and dense enhancement from lateral ventricles to 4th ventricle. The stereotactic biopsy of tumor and histologic examination revealed the germinoma. Craniospinal axis radiation therapy was performed. After radiation therapy patient was improved and no neurologic sequelae was seen at discharge. Periventricular germinomas without pineal or suprasellar lesion are very rare. The radiation therapy, as in our case, is beneficial as with other intracranial germinomas. Stereotactic biopsy of periventricular germinoma provides precise pathologic diagnosis and thus allows more specific management.