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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.

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