A Case of Moyamoya Disease Associated with Systemic Lupus Erythematosus

Moyamoya disease is a rare clinical entity, diagnosed by cerebral angiography and characterized by occlusion of the bilateral internal carotid artery system and development of collateral arteries. The etiology of moyamoya disease is unknown but some cases were reported to be associated with many other diseases such as hereditary, infectious, autoimmune, and metabolic disorders. A 16-year-old female was admitted with right side motor weakness, visual dis-turbance, malar rash, and emotional lability. Physical examination showed hair loss on frontal and bitemporal areas. Neurologic examinations revealed right hemiparesis and left homonymous hemianopsia. Brain magnetic resonance image showed high signal lesions in the right occipito-temporal and left high frontal area in T2-weighted image. Cerebral angiography showed focal obstructions of supraclinoid portion of both internal carotid arteries and multiple collateral vessels consistent with Moyamoya disease. The clinical features and serologic test of anti-nuclear antibody, anti-double stranded deoxyribonucleic acid antibody and cryoglobulin confirmed the diagnosis of systemic lupus ery-thematosus. We report a case of Moyamoya disease associated with systemic lupus erythematosus which might con-tribute to the etiological variability of Moyamoya disease.

A Case of Chordoma Arising from Clivus

Chordoma is a rare tumor derived from notochord remnants, but can occur at any level of the craniovertebral axis, pri-marily in the sacrum, clivus, and cervical regions. Therefore, chordoma should be considered as a rare cause in the dif-ferential diagnosis of mass lesions around the cervicomedullary junction. A 62-year-old man suffered from a suboccipi-tal, nuchal pain and headache for 15 years and nasal obstruction, recently. In a neurological examination, dysarthria, uvula and tongue deviation, depressed soft palate, decreased gag reflex, and atrophic SCM were noted. The MRI find-ings included an isosignal, round, well- marginated, and nearly homogeneous tumor compressing the lower brainstem. A biopsy was done by the transoral approach with a permanent diagnosis of chordoma arising from the clivus. We report a rare case of chordoma arising from the inferior margin of the clivus in a patient with multiple lower cranial nerve palsies.

A Case of Chordoma Arising from Clivus

Chordoma is a rare tumor derived from notochord remnants, but can occur at any level of the craniovertebral axis, pri-marily in the sacrum, clivus, and cervical regions. Therefore, chordoma should be considered as a rare cause in the dif-ferential diagnosis of mass lesions around the cervicomedullary junction. A 62-year-old man suffered from a suboccipi-tal, nuchal pain and headache for 15 years and nasal obstruction, recently. In a neurological examination, dysarthria, uvula and tongue deviation, depressed soft palate, decreased gag reflex, and atrophic SCM were noted. The MRI find-ings included an isosignal, round, well- marginated, and nearly homogeneous tumor compressing the lower brainstem. A biopsy was done by the transoral approach with a permanent diagnosis of chordoma arising from the clivus. We report a rare case of chordoma arising from the inferior margin of the clivus in a patient with multiple lower cranial nerve palsies.

A Case with Cerebral Radiation Necrosis Mimicking Recurrent Brain Tumor

Regardless of an appropriate radiation therapy administered to a patient with a brain tumor, clinical and radiological evidence of progression may still develop because of a recurrence of the tumor and/or radiation necrosis, or even rarely, a radiation induced neoplasm. The evaluation of tumor recurrence and radiation-induced necrosis presents a diagnostic challenge. A thirty eight year old woman was treated for a focal motor seizure on the right side of her face.with a fractionated external beam radiotherapy under the impression of cystic astrocytoma in the left frontoparietal region. After 6 months, she developed a headache, nausea and vomiting, dysphagia, and secondary generalized seizure. A follow-up brain MRI showed a high signal mass lesion in a T2 weighted image and a fluorodeoxyglucose PET revealed hypometabolism in the left frontoparietal lobe, suggesting radionecrosis. It was confirmed as radionecrosis with an expanding edema by open biopsy. We report a cerebral radiation necrosis mimicking recurrent brain tumor.

A Case of Melkersson-Rosenthal syndrome

Melkersson-Rosenthal syndrome (MRS) is a rare form of hereditary angioedema characterized by a triad of orofacial swelling, relapsing facial paralysis, and a fissured tongue. However, the classic triad is not frequently seen in its complete form, and monosymptomatic or oligosymptomatic forms are more common. Case: A 20-year-old man presented with recurrent labial swelling 9 months ago and recently with a fissured tongue. The surface of the tongue showed deep furrows characteristic of lingua plicata, and an edematous enlargement was observed on the lower lips. Upon neurologic examination, a left sided facial palsy of a peripheral type was noted without abnormalities in the taste sense or lacrimation. An electromyography of the left frontal muscle showed positive sharp waves and fibrillation potentials. Biopsies performed on the lower lips revealed the congested vessels and perivascular inflammatory cells. We report a 20-year-old man with a classical triad of symptoms of Melkersson-Rosenthal syndrome.

Seizure-related injuries in epileptic patients

BACKGROUND: The epileptic patient are more easily exposed to danger because of consciousness change or convulsions but injuries resulting from epileptic seizure have received little attention in the literature. We evaluated the seizure-related injuries( SRI ) and the various factors which would often cause injuries. METHODS: We performed a prospective study of 238 epileptic patients of Hanyang University and Gachon Medical Center. Clinical profiles( seizure type, frequency, etiology, absence or presence of aura, etc ) of each patient and SRI excluding tongue bites were obtained from medical records and interviews. For the statistical analysis, chi-square test was used. RESULTS: 4584 seizure attacks during a period of one year resulted in 48 SRI and the incidence of SRI was approximately 1.0 %. SRI was noted as follows ; head contusion & laceration ( 22 ), other contusions & lacerations( 13 ), burns( 6 ), fractures & dislocations ( 5 ), and death( 2 ; one was during sleep, the other during a shower ). Type of seizure most often causing injury was generalized seizure. Presence of aura did not affect the incidence of SRI, but seizure frequency had a positive relation with SRI. CONCLUSION: The SRI was minimal but the patient with more frequent seizure tend to have chances of higher incidence of injuries. And the incidence of SRI was higest in young age group ( 20s, 30s ), probably due to their more active life. Even though strict restriction of daily activities would not be necessary, there should be an effort to improve seizure control and supervise hazardous circumstances.

A Case of Relapsing Myelopathy Caused by Spinal Epidural AV Malformation

Spinal arteriovenous malformation ( AVM ) can be a rare cause of relapsing myelopathy. We recently have the opportunity to study a 20-year-old male patient having spinal epidural AVM confirmed by surgical biopsy. To our knowledge, a case of relapsing and remitting course of spinal epidural AVM has not been reported in Korea. A 20-year-old male was admitted because of sudden paraparesis and disturbance of all sensory modalities below the T5 dermatome, 5 times during 2 years. Cerebrospinal fluid profiles did not reveal any pathologic findings. Until the 4th attack, MR findings were non diagnostic. At the 5th attack, thoracic spine MR revealed epidural hematoma compressing the spinal cord. Diagnostic and therapeutic decompressive surgical procedure was done. On surgical field, solitary and tortuous feeding vessels with hematoma were seen in epidural space. Microscopically, the tissue was compatible with arteriovenous malformation.

Neuro-Behcet diseases showing pseudotumoral presentation

BACKGROUND AND SIGNIFICANCE: Because of the diverse clinical and radiologic manifestations of Neuro-Behcet disease, it is sometimes difficult to differentiate with multiple sclerosis or tumorous condition. We report two cases of Neuro-Behcet disease underwent by stereotaxic biopsy showing vasculitis without any evidence of malignancy, initially misdiagnosed as solitary tumor and metastatic tumor, respectively. CASE: A 35-year-old male admitted due to right hemiparesis. Brain CT showed low density mass shadow in left thalamus. Under the impression of low grade astrocytoma, stereotaxic biopsy was performed. Dense perivascular lymphocytic infiltration with petechial microhemorrhage mixed with hemosiderin pigment was noted in thalamic region. The other patient (male, 44yrs old) admitted due to seizure. On MRI, there are multiple inhomogenous signals on right occipital and left frontal lobe and left thalamus. Under the impression of metastatic tumor, work-up about the primary tumor was done without any positive findings. To determine the primary focus, stereotaxic biopsy was performed. Only reactive gliosis and mild perivascular lymphocytic infiltration was noted in pathologic specimen. With the retrospective careful history taking and follow-up image study, two patients were confirmed as having a Behcet's disease.

Carotid body paraganglioma showing multiple spinal metastases

Paragangliomas are rare and slowly growing pulsating hypervascular tumors arising from neural crest cell derivatives throughout the body. In the head and neck, they originate from the branchiometric family of paraganglioma, neuroendocrine tissue that lies along the carotid artery or aorta. Despite their propensity for locally aggressive behavior, metastases of the tumors are quite rare. We report an unusual case of carotid body paraganglioma showing multiple spinal metastases and demonstrated by spinal angiography.