Cloning of an insulin-like androgenic gland factor (IAG) from the blue crab, Callinectes sapidus: implications for eyestalk regulation of IAG expression.

In malacostracan crustaceans, sex differentiation is uniquely regulated by a hormone secreted by the male-specific androgenic gland (AG). An isopod AG hormone was the first to be structurally elucidated and was found to belong to the insulin superfamily of proteins. Recently, it has been found that the AGs of several decapod crustaceans express insulin-like androgenic gland factors (IAGs), whose function is believed to be similar to that of the isopod AG hormone. Here we report the isolation from the blue crab Callinectes sapidus of the full-length cDNA encoding a candidate insulin-like AG hormone, termed Cas-IAG. The predicted protein Cas-IAG was encoded as a precursor consisting of a signal peptide, the B chain, the C peptide, and the A chain in that order. While the AG was the main source of Cas-IAG expression, as found in other decapod species, the hepatopancreas of male Callinectes sapidus crabs displayed minor Cas-IAG expression. Eyestalk ablation confirmed the presence of a possible endocrine axis between the eyestalk ganglia and the AG, implying that Cas-IAG expression is negatively regulated by (a) substance(s) present in the eyestalk ganglia.

Identification and Characterization of an Insulin-Like Receptor Involved in Crustacean Reproduction.

Sexual differentiation and maintenance of masculinity in crustaceans has been suggested as being regulated by a single androgenic gland (AG) insulin-like peptide (IAG). However, downstream elements involved in the signaling cascade remain unknown. Here we identified and characterized a gene encoding an insulin-like receptor in the prawn Macrobrachium rosenbergii (Mr-IR), the first such gene detected in a decapod crustacean. In mining for IRs and other insulin signaling-related genes, we constructed a comprehensive M. rosenbergii transcriptomic library from multiple sources. In parallel we sequenced the complete Mr-IR cDNA, confirmed in the wide transcriptomic library. Mr-IR expression was detected in most tissues in both males and females, including the AG and gonads. To study Mr-IR function, we performed long-term RNA interference (RNAi) silencing in young male prawns. Although having no effect on growth, Mr-IR silencing advanced the appearance of a male-specific secondary trait. The most noted effects of Mr-IR silencing were hypertrophy of the AG and the associated increased production of Mr-IAG, with an unusual abundance of immature sperm cells being seen in the distal sperm duct. A ligand blot assay using de novo recombinant Mr-IAG confirmed the existence of a ligand-receptor interaction. Whereas these results suggest a role for Mr-IR in the regulation of the AG, we did not see any sexual shift after silencing of Mr-IR, as occurred when the ligand-encoding Mr-IAG gene was silenced. This suggests that sexual differentiation in crustaceans involve more than a single Mr-IAG receptor, emphasizing the complexity of sexual differentiation and maintenance.

Cell mediated immunity to human myelin basic protein in Vogt-Koyahagi-Harada syndrome.

An immunological basis for neurological involvement in Vogt-Koyanagi-Harada syndrome was sought by means of the migration-inhibition factor technique with human myelin basic protein. The test was carried out in four patients who had recent manifestations of the syndrome, two of whom were evaluated both before and after initiation of steroid treatment; in one patient 4 years after recovery from the syndrome; in three patients having uveitis of other causes, and in 12 healthy controls. The results were positive in all four patients who had recent manifestations, whereas they were negative in all the others. This finding may constitute evidence of a cell-mediated immunity toward components of the nervous system in this disease entity.

Inhibition of macrophage migration by choroidal malignant melanoma-associated antigens in patients with unveal melanoma.

The specific cell-mediated immunity of the lymphocytes of eight patients with choriodal malignant melanoma (MM) to four extracts of choroidal MM-associated antigens was tested with the aid of the MIF technique. Seven of the patients with choroidal MM responded to at least one of the four extracts used, whereas patients with choroidal nevus or carcinoma as well as healthy controls did not respond to any of the MM choroidal extracts. There was no response to iris extracts obtained from the enucleated eyes with MM in any of the subjects tested.

Spontaneous hyphema and vessel anomaly.

We describe a case of spontaneous hyphema, in which results of fluorescein angiography revealed that the condition was caused by anomalous iris vessels. A cavernous hemangloma of the ipsilateral orbit was discovered. We stress the importance of performing fluorescein angiography in all cases of spontaneous hyphema.

Malignant optic glioma in a 70-year-old patient.

In a 70-year-old man with glioma of the optic nerves and tracts, the initial symptom was a unilateral loss of vision that progressed rapidly and was followed by amaurosis of both eyes. All diagnostic radiological procedures were negative. Four months after the onset of the disease, the patient developed hemiplegia, became comatose, and died. Post-mortem examination revealed a glioblastoma multiforme of both optic nerves, chiasma, and optic tracts that extended posteriorly into the left thalamus and medial geniculate body. The tumoral thickening of the optic nerves was absent in the intracanalicular part, a finding that concurred with the normal radiological appearance of the optic foramen. Glioblastoma multiforme of the optic pathways should be included in the differential diagnosis of acute visual failure in elderly people, even though the final diagnosis may be possible only at postmortem examination.

Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). METHODS: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.

Amaurosis fugax at downward gaze.

A 54-year-old man with a past history of hypertension, diabetes mellitus, hypercholesterolemia and two myocardial infarctions presented with repeated attacks of amaurosis fugax in the right eye. The fact that the amaurosis occurred only on downward gaze is sufficient evidence to exclude carotid atheromatous disease and to imply an orbital etiology.

Entoptic [corrected] phenomena in pregeniculate and postgeniculate hemianopsia with splitting of macula by perimetry.

With splitting of macula by perimetry, the entopic phenomena (the perception of one's own foveal xanthophilic pigment and macular vessel leukocytes) were utilized to study the character of macular sparing or splitting in patients with pregeniculate or postgeniculate hemianopsia. In the pregeniculate group, 11 of 14 eyes perceived the Haidinger brushes figure as a half circle corresponding to the perimetric macular splitting, whereas flying corpuscles were not perceived at all or were less numerous on the hemianoptic side than on the normal field side. In contrast, six of seven patients with postgeniculate lesions perceived the Haidinger brushes figure as a complete circle, four of six perceived fewer flying corpuscles on the hemianoptic side than on the normal field side, and two patients reported perceiving an equal number in all quadrants. This study indicated that in pregeniculate hemianopsia there is usually a true splitting of the macula, whereas in postgeniculate hemianopsia, there is some macular sparing even when perimetry shows macular splitting.

Reading-evoked visual dimming.

PURPOSE: To carry out a neuroradiologic investigation in a monocular 49-year-old patient who during the past five years described symptoms of dimming of central vision in his left eye, which was provoked only by reading. METHODS: Computed tomography and magnetic resonance imaging were performed. RESULTS: An orbital apex intraconal tumor situated laterally to and above the optic nerve was found. CONCLUSIONS: Reading-evoked visual dimming can be a variant of gaze-evoked amaurosis. The optic nerve displaced laterally and superiorly, and stretched by the act of reading, may be compressed between the tumor above and the contracted inferior rectus muscle inferiorly.

Nasal visual field loss with intracranial lesions of the optic nerve pathways.

Five patients developed nasal visual field defects as a result of involvement of the intracranial portion of the optic nerves. The cause in each patient, respectively, was as follows: (1) dolichoectatic carotid arteries, (2) optochiasmatic arachnoiditis, (3) meningioma of the olfactory groove, (4) pituitary apoplexy, and (5) pituitary chromophobe adenoma. The common factor in these cases was probably impaired circulation in the prechiasmal arterial anastomotic network. The nasal visual field loss present in these cases was characterized by a pattern similar to that seen in glaucoma but with impairment of visual acuity. The superior nasal visual field was usually normal and the lower temporal visual field often defective.

Narrow-band (540NM) green-light stereoscopic photography of the surface details of the peripapillary retina.

We used narrow-band (540-nm) green-light stereoscopic fundus photography to study the surface details of the peripapillary retina in 14 patients. Each photograph was divided into four areas; the papillomacular bundle, the upper arcuate bundle, the lower arcuate bundle, and the nasal sectorial fibers. We scored each area on a scale of 0 (normal) to 3 (total loss of nerve fiber layer), and compared these scores to those obtained by testing the corresponding visual fields. The scores were the same in 88 of 111 comparisons; the retinal scores were higher in 17 comparisons and the visual field scores were higher in six comparisons. This photographic technique makes the nerve fiber layer easier to see and evaluate and the detection of defects becomes less difficult.

Visual fields in metastatic choroidal carcinoma.

A large choroidal metastasis was diagnosed in the right eye of a 44-year-old man referred for admission to hospital because of 'retinal detachment.' At the same time in the second, symptomless, eye a very small metastasis was observed. Close follow-up during the next month showed an extremely rapid deterioration of visual acuity and visual field in this eye. This is thought to be characteristic of such metastatic tumours in contrast to the slower progress of choroidal malignant melanoma. The application of local radiotherapy to the same eye led to an impressive improvement in the visual acuity and visual field. The source of these bilateral choroidal metastases, which was found only after the patient's death, proved to a bronchial carcinoma.

Argon laser treatment of senile retinoschisis.

Fifty-seven eyes of 49 patients with senile retinoschisis were treated by argon laser photocoagulation and followed up for 3 to 6 years. In most of the treated eyes the retinoschisis either completely or partially collapsed and in none did the retinoschisis or loss of visual field progress further. No long-term complications due to the treatment were noted. In none of the treated eyes did retinal detachment develop. In view of this the indications at present for argon laser treatment in retinoschisis may be extended to include any case in which the retinoschisis progresses beyond the equator and visual field loss continues. This may prevent further visual field damage and other rare complications of slowly progressive retinoschisis and facilitate the many years' follow-up of elderly patients with retinoschisis who develop cataracts.

Traumatic bone cyst resembling apical periodontitis.

Among the pseudocysts of the jaws, the traumatic bone cyst is known as an asymptomatic lesion often noted unintentionally during routine radiographic examinations. The lesion neither devitalizes the teeth within its borders, nor does it cause resorption of their roots. The well-demarcated traumatic bone cyst often projects into the intraradicular septa and hence has been described as having scalloped borders. The following presentation is of a traumatic bone cyst that resembled periodontal pathology in its appearance.

Metastatic follicular thyroid carcinoma to the maxilla.

We present a unique case of metastatic follicular thyroid carcinoma to the hard palate and the maxillary sinus, a case that to our knowledge has not been reported before. Various malignant tumours that metastize to the maxilla are reviewed, and the therapeutic approach to follicular thyroid carcinoma metastasis to that area is also discussed. Follicular thyroid carcinoma should be included in the list of tumours that metastasize to the maxilla.