A Case of Neuromyelitis Optica with Paraneoplastic Syndrome in Hepatocellular Carcinoma

PURPOSE: We report a case of neuromyelitis optica (Devic's syndrome) with hepatocellular carcinoma. CASE SUMMARY: A 70-year-old male with hepatocellular carcinoma presented with bilateral visual loss. A relative afferent pupillary defect was not observed in either eye due to bilateral mydriasis. On brain MRI, there was no specific finding, however, on spine MRI, multiple and severe myelopathies were observed. After high-dose methylprednisolone pulse therapy, the visual acuity was 0.03 in the right eye and counting fingers at 30 cm in the left eye. CONCLUSIONS: We encountered a case of neuromyelitis optica involving bilateral optic neuropathy in hepatocelluar carcinoma. Therefore, in patients with an optic neuropathy of uncertain etiology, clinicians should consider performing a systemic evaluation.

Simultaneously Measured CO2 Reactivity in the Basilar and Middle Cerebral Artery: The Utilization of Power M-mode Doppler and Anterior-posterior Probes Fixating Device

BACKGROUND: Studies using a transcranial Doppler (TCD) to establish cerebral vasoreactivity (CVR) have mostly focused on the anterior circulation. The purpose of this study is not only to evaluate the feasibility of the power motion mode Doppler (PMD) with a probes fixating device, but also to simultaneously measure the CVR between the middle cerebral artery (MCA) and the basilar artery (BA) during hypercapnea simulated by the rebreathing technique. METHODS: Twenty eight healthy volunteers were enrolled. Baseline hemodynamic values (heart rate, blood pressure) were measured while volunteers sat in a comfortable position for 5 minutes. The TCD was performed in two steps. First, velocities and spectra of the MCAs were simultaneously monitored. Then, the velocities and spectra of the MCA and BA were simultaneously monitored by a headset that included an anterior-posterior probes fixating device. The equation for CVR was ([maximum mean velocity baseline mean velocity] x 100/baseline mean velocity). RESULTS: Baseline mean velocities were revealed as follows: (64.0+/-13.7, 65.0+/-11.9 cm/s in right and left MCA; p>0.05; 67.3+/-12.2, -45.3+/-7.6 cm/s in dominant MCA and BA). CVR did not differ between the dominant MCA and the BA (46.1+/-12.1, 46.0+/-15.1%; p>0.05), nor between the right and left MCAs (46.9+/-15.2, 46.4+/-14.8%; p>0.05). There was a positive linear correlation between the CVR of the dominant MCA and that of the BA (r=0.856; p<0.001). CONCLUSIONS: PMD with a probes fixating device for accurate insonation is a useful tool for evaluating the relative CVR between the MCA and BA. Our study suggests that CVR values of the BA are similar to those of the MCA.

Lethal Catatonia in the Right Middle Cerebral Artery Territory Infarction

Lethal catatonia is a rare and fatal clinical syndrome. It has diverse etiologies, both functional and organic. We experienced a man with left hemiplegia and striking catatonia. Brain imaging showed a large right hemispheric infarction and hypoperfusion. Symptoms and signs of catatonia were markedly improved after treatment of dopa-agonist and benzodiazepine. This case suggests that right hemispheric infarction could be a possible cause of lethal catatonia, and supports earlier clinical literature relating catatonia and the parietal lobe.

A Case of Gliomatosis Cerebri; MRI and MR Spectroscopy Findings

No abstract available.

The Clue for Early Diagnosis and Prediction of Intracranial Involvement in Rhinocerebral Mucormycosis

BACKGROUND: Rhinocerebral mucormycosis (RCM) is an uncommon and fatal clinical syndrome resulting from an opportunistic infection caused by a fungus of the order Mucorales in immunocompromized patients. The mortality and morbidity in the patients with intracranial involvement is invariably high, and it was reported that most survivors had early diagnosis and received aggressive treatment. Therefore, we retrospectively reviewed four patients of pathologically confirmed mucormycosis to find out the clues for early diagnosis of RCM and for prediction of fatal intracranial involvement. METHODS: The clinical, radiological features and histo-pathological involvement sites in the patients with intracranial involvement were compared to those without intracranial involvement. RESULTS: All the patients had uncontrolled diabetes and were admitted with orbital involvement. On the precise physical examination, mucosal involvement was found in all patients; three of them in only nasal mucosa and one in oral palatal mucosa. Despite the vigorous antifungal therapy, two patients had intracranial involvement and expired. MRI finding of extensive sphenoid sinusitis adjacent cavernous sinus preceded the intracranial involvement of RCM, which was not found in the patients without intracranial involvement. CONCLUSIONS: Our findings suggest that early observation of oral or nasal mucosal changes might be an important clinical clue for differentiation of RCM from other causes of rhino-oculo-cerebral symptoms in uncontrolled diabetic patients, and that extensive sphenoid sinusitis might be an important radiological feature for predicting the fatal intracranial involvement of RCM.

A Case of Polymyositis associated with Hepatitis B infection

Polymyositis(PM) is one of idiopathic inflammatory myopathy, characterized by proximal muscle weakness, myalgia and muscle enzyme elevation. Currently the main pathogenesis is well documented, the cell-mediated immunity. We experienced a case of polymyositis associated with hepatitis, developed after hepatitis B virus(HBV) infection. This virus-induced autoimmunity seems to result from the cross-reactivity between muscle protein and B-viral antigen, so called antigenic mimicry. This relation of PM and HBV is more significant in Korea because of the epidemicity of HBV infection.