[Legionnaires' disease asssociated with meningitis and cerebellar ataxia].
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Biomedical subjects
Publications and source records attributed to Fujio Fujiki.
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We report a case with recurrent orbital pain and unilateral cranial nerve paresis mimicking Tolosa-Hunt syndrome. However, these features were most likely caused by bacterial infection because of beneficial response to antibiotics therapy. A 32-year-old man developed severe right orbital pain and diplopia. Neurological examination revealed right oculomotor paresis and 1st division of the right trigeminal nerve dysfunction. MR imaging revealed thickness of right cavernous sinus region with marked gadolinium enhancement. Cerebrospinal fluid (CSF) examination was initially normal. Treatment with steroid showed marked improvement. However soon after tapering of steroid dosage, his symptoms recurred and deteriorated. He was referred to our hospital because of second opinion. Neurological examination still showed right oculomotor paresis and 1st division of the right trigeminal nerve dysfunction with orbital and retro-orbital pain. Re-examination of CSF showed pleocytosis with neutrocytes dominancy and elevated protein concentration. Intravenous treatment with penicillin was initiated with marked improvement. There have been reported cases with bacterial infection resulting pseudo Tolosa-Hunt syndrome, which have good response to antibiotics treatment with excellent prognosis. It is speculated that bacterial infection might cause clinical features mimicking Tolosa-Hunt syndrome in our case.
Garcin syndrome is characterized by an unilateral cranial nerves involvement without sensory or motor long-tract disturbances. It is usually caused by tumor infiltrating in the skull base with osteolytic changes on radiological study. We report a case of 64-year-old man with history of alcohol overintake, who admitted local hospital, because of right periorbital edema and facial swelling. He noted right ptosis 2 weeks prior to admission. Neurological examination revealed right multiple cranial nerves involvement including II, III, IV, V, and VI cranial nerves. MR imaging of the brain showed marked paranasal sinusitis and abnormal infiltration of right orbital fat. Orbital apex syndrome related to paranasal sinusitis was diagnosed, and antibiotics was administered. But a few days after admission, he developed a right VII, IX, X cranial nerve palsy. He was transferred to our hospital because of acute development of left hemiparesis and deteriorated consciousness. MR imaging of the brain showed right internal carotid artery (ICA) occlusion, and infarction in right middle cerebral artery (MCA)'s territory. The diagnostic biopsy of the paranasal sinus showed mucorales hyphae, indicating that the pathological diagnosis was mucormycosis. Despite of antibiotic therapy included of amphotericin-B administration and strict control of diabetic mellitus, his sinusitis was gradually spread. His condition progressively deteriorated, and finally died of sepsis. Post-mortem examination revealed a widespread mucor infiltration in the dura mater without skull bone invasion. This case presented with unilateral multiple cranial nerve involvements (Garcin syndrome) followed by left hemiparesis associated with rhinocerebral mucormycosis. It is suggested that mucormycosis should be considered in case of Garcin syndrome without osteolysis in the skull base.
A 31-year-old man was transferred to our emergency room (ER) with acute onset of high-grade fever and consciousness disturbance. His consciousness at ER was severely disturbed with restlessness. No apparent focal neurological signs were seen. MRI with diffusion-weighted images (DWI) showed high signal intensities at the corpus callosum, left cerebellar hemisphere, left deep white matter and right middle cerebellar peduncle. These lesions were low signals in apparent diffusion coefficient(ADC) map, indicating cytotoxic edema. EEG showed enhanced fast waves seen in predominantly frontal regions. CSF examination was normal except elevated initial pressure of 210 mmH2O. He was treated with high dose dexamethasone and acyclovir. His consciousness and high-grade fever with systemic inflammatory responses were dramatically improved after these treatments. Subsequent data showed hyperthyroidism with anti-thyroid stimulating hormone receptor antibodies. This case was thought to be a thyrotoxic encephalopathy with beneficial response to corticosteroid therapy. Abnormalities seen in DWI and EEG were normalized ten days later.