We reviewed the MRI of 20 patients with a ranula (8 simple and 12 plunging) and ten with other cystic masses in the floor of the mouth and/or suprahyoid portion of the neck (three haemangiomas, two neuromas, one monomorphic adenoma, one lipoma, two lateral cervical cysts and one dermoid cyst). Histological diagnoses were obtained in all cases with the exception of one presumed haemangioma. Ranulas were all well-defined, homogeneous masses giving low signal on T1- and markedly high signal on T2-weighted images. While simple ranulas were all confined to the sublingual space, plunging ranulas were centered on the submandibular space and tended to spill into one or more adjacent spaces. They extended into the sublingual space anteriorly (producing a so-called tail sign) in eight of 12 cases and into the parapharyngeal space superiorly in five. Although they sometimes filled a considerable part of the parapharyngeal space, displacement of surrounding muscles or vessels was usually slight, which was thought to reflect the nature of extravasation pseudocysts. All other cystic masses in our study had one or more MRI finding different from those of ranulas and could be easily differentiated from them.
We describe two patients with sporadic amyotrophic lateral sclerosis (ALS), who had developed progressive external ophthalmoplegia of a predominantly supranuclear type while they survived on respirators, and displayed histopathological abnormalities both typical and atypical of ALS. Patient 1 was a 43-year-old man with ALS of 5-year duration, who had initially exhibited fulminant ALS, and remained on a respirator for 4 years. Patient 2 was a 51-year-old man with ALS of 13-year duration, who remained on a respirator for 8 years. Both patients died in a "totally locked-in state". Autopsy of both patients revealed not only histopathological abnormalities consistent with ALS, but also multisystem degeneration which involved the pontine tegmentum, substantia nigra, Clarke's dorsal nuclei and spinocerebellar tracts. In addition, Patient 2 displayed intracytoplasmic neuronal basophilic inclusion bodies which exhibited marked immunoreactivity to anti-ubiquitin antibodies. Our case reports indicate that the longer survival which is possible through the use of respirators may make one subgroup of ALS patients prone to develop atypical clinical and neuropathological features which are not observed during the natural course of ALS.
Eighteen patients were identified in whom ultrasound (US) of the kidney demonstrated a hyperechoic medulla. Diagnoses in the patients included gout in seven; Sjögren syndrome in two; medullary sponge kidney in two; primary aldosteronism in two; and Lesch-Nyhan syndrome, hyperparathyroidism, glycogen storage disease type XI, Wilson disease, and pseudo-Bartter syndrome in one each. The pathogenesis of the echogenicity was evaluated by comparing the findings from computed tomography and conventional radiography. It appears that a hyperechoic medulla is caused by hyperuricemia, medullary nephrocalcinosis, or hypokalemia. US is considered to be useful in evaluating renal involvement in patients with these diseases.
Intravascular large B-cell lymphoma (LBCL) is a rare and aggressive subtype of diffuse LBCL characterized by disseminated intravascular proliferation of neoplastic lymphocytes. Obstruction of blood flow by tumor cells in a variety of organs can cause an array of clinical changes, including alteration of the neural and spinal system and the respiratory system, as well as skin lesions. It is usually very difficult to diagnose intravascular LBCL in a patient simply from clinical symptoms or laboratory examinations. We here document our findings that serum prostatic acid phosphatase levels in both males and a female (2.2-24.0 microg/L) reflect the presence of intravascular LBCL, changing synchronously in response to chemotherapy. To determine whether prostatic acid phosphatase (PAP) might be a useful tumor marker for early diagnosis, we reviewed five intravascular LBCLs. Immunohistochemically, tumor cells in all cases were positive for anti-PAP antibody. The results were further confirmed in one case by Western-blot analysis and in another by the detection of amplified messenger RNA for PAP in microdissected tumor cells, respectively. PAP has not been detected in 17 lymphomas (diffuse LBCL, 8 cases; follicular lymphoma, 3 cases; T-cell lymphoma, 3 cases; Hodgkin lymphoma, 3 cases) by Western blot analyses. We conclude that serum PAP is a useful tumor marker for intravascular LBCL and that it deserves further investigation in this context.
12 cases of unilateral labyrinthectomy, 3 cases of VIIIth nerve section, 22 cases of streptomycin sulfate infusion into the middle ear cavity and 8 cases of bilateral vestibular a-functions underwent vestibular training. Our training is very useful for regaining equilibrium and for evaluating the effects of training on equilibrium by recording the gravity center movements.
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