Osteoporosis in osteogenesis imperfecta

Osteogenesis imperfecta (OI) is a kind of genetic osteoporosis, whose clinical severities vary from patients to patients. In type I OI, some patients appear normal without investigating bone mineral density (BMD) by DXA. In severe OI children, pamidronate infusion therapy is effective, however it is still uncertain when to stop the pamidronate therapy without having the problems of bone fracture healing. Also, the appropriate therapy for adult and post-menopoause OI patients remains to be clarified in future.     

Isolated dissecting aneurysm of the brachiocephalic artery associated with contained rupture

A 79-year-old man with no history of trauma complained of a sudden onset of headache and backache. Computed tomography showed an isolated dissecting aneurysm 4 cm in diameter associated with contained rupture at the takeoff region of the brachiocephalic artery. Surgery was performed on an emergent basis. The proximal aortic arch was successfully replaced using antegrade selective cerebral perfusion. The patient was easily weaned from cardiopulmonary bypass and recovered uneventfully.     

Painful snapping in rheumatoid knees

Summary. Painful snapping caused by a solitary intra-articular nodular mass was found in 11 knee joints of 8 patients with rheumatoid arthritis. The masses were characteristically situated in the anterolateral aspect of the lateral femoral condyle of each joint, and appeared to jump and slip in and out of the patellofemoral articulation during flexion and extension. The snapping caused by the solitary mass was noticed as the joint flexed from 15° to 30° in 9 knees and from 50° to 60° in 3. Histopathological examination of the masses revealed typical findings of a rheumatoid nodule in 4 knee joints and myxomatous degeneration and collagen necrosis in 7.

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Résumé. 11 genoux présentant un ressaut douloureux à cause d’un nodule intra-articulaire ont été opérés chez 8 patients atteints d’arthrite rhumato? de. Les nodules se situaient sur la face latérale du condyle fémoral externe et s’introduisaient dans l’articulation fémoro-patellaire lors de la flexion du genou. Le ressaut survenait aux environs de 15 à 30° de flexion pour 9 genoux et 50 à 60° de flexion pour 3. L’examen histologique a montré un aspect typique de nodule rhumato?de dans 4 cas et une dégénérescence myxo?de avec nécrose collagène dans les 7 autres cas.

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Accepted: 19 March 1997     

An autopsy case of origin-unidentified meningeal carcinomatosis presenting with monoradiculopathy multiplex in the lower extremities]

A 54-year-old man with a history of partially dissected epidermoid cyst in the left cerebellopontine angle suffered from a slowly progressive dysesthesia and weakness in the lower extremities and trunk. Neurological examination revealed segmental muscular weakness and sensory disturbance in those regions, giving rise to the possibility of monoradiculopathy multiplex, as well as loss of tendon reflexes, dysuria and right facial nerve palsy. Electrophysiological studies indicated irregular motor axonopathy or neuronopathy and interruption of more central sensory pathways than the lumbosacral spinal nerve roots with spared peripheral sensory nerves. Although MRI demonstrated enhanced lesions in the cauda equina and lumbosacral leptomeninges, CSF cytology or a cauda equina biopsy showed no malignancy. His symptoms gradually progressed and he died 15 months after the onset. The autopsy failed to reveal any tumors in the general systemic internal organs. Histopathology demonstrated meningeal carcinomatosis with squamous-type carcinoma cells scattered in the cerebrospinal leptomeninges, and perineurium in almost all the spinal and cranial nerve roots, causing severe axonal degeneration. The dorsal root ganglions escaped tumor cell invasion. Absence of the malignant tumors in the systemic organs and the history of the operated epidermoid cyst indicate that the tumor may be the cause of the meningeal carcinomatosis in this case. Meningeal carcinomatosis almost always shows rapid progression and extremely poor prognosis with several month survival in general, and little attention has been paid that it can exhibit symptoms and signs of segmental involvement in the lumbosacral regions. Our present case prompts us to bear in mind that patients with this condition can survive fairly long, and raises the possibility that a careful neurological examination with segmental involvement will reveal such a feature more frequently than considered so far in this condition.     

Autonomic nervous function and localization of cerebral activity during lavender aromatic immersion.

Changes in the autonomic nervous activity can be induced by various sensory and emotional stimuli. The authors examined whether the power spectral analysis of heart rate variability (HRV) could detect changes in autonomic tone following a lavender aroma treatment or not. Healthy young women (n=10, 23+/-3 years old) underwent continuous electrocardiographic (ECG) monitoring before and after (10, 20, 30 minutes) the lavender fragrance stimuli. HRV was expressed by three indices: low (0.04-0.15 Hz) and high (0.15-0.40 Hz) frequency components (nLF and nHF, respectively) as well as LF/HF ratio. Increases in the parasympathetic tone were observed after the lavender fragrance stimulus as seen as increases in the HF component and decreases in the LF/HF. Additional measurement with positron emission tomography (PET) demonstrated the regional metabolic activation in the orbitofrontal, posterior cingulate gyrus, brainstem, thalamus and cerebellum, as well as the reductions in the pre/post-central gyrus and frontal eye field. These results suggested that lavender aromatic treatment induced not only relaxation but also increased arousal level in these subjects.     

Multiple aneurysms of the visceral arteries with migrating vascular bruit on postural change: A case report

Multiple aneurysms of the gastroepiploic artery and the ileocecal branch of the superior mesenteric artery were found in a 68-year-old male patient by angiography. The patient presented with one-hour postprandial epigastric pain of 10 years duration. Abdominal bruit was auscultated at the two different sites, one of which shifted downwards upon upright position. From the freely movable nature of the great omentum, this bruit, migrating upon postural change, was most likely from the gastroepiploic artery aneurysms. The aneurysms were excised and the abdominal bruit disappeared. The etiology of the aneurysms was suggested to be arterial fibrodysplasia histologically. From this experience, it was stressed that postural change should be added to a routine physical examination to rule out an aneurysm from the freely movable great omentum.