Isolated vertical diplopia as the initial manifestation of presumed pretectal and anterior hypothalamic germinomas.

A 21-year-old man with a 5-month history of diplopia caused by isolated vertical ocular misalignment had normal laboratory studies, including brain magnetic resonance imaging (MRI). Eight months after the onset of diplopia, he reported dry mouth, polydipsia, polyuria, and absent sweating. Examination now disclosed light-near dissociation of the pupillary responses, convergence-retraction nystagmus, and upgaze palsy. MRI revealed enhancing suprasellar and pretectal masses presumed to be germinomas. Two years after brain irradiation and systemic chemotherapy, no lesions are apparent on MRI and hypothalamic dysfunction has partially resolved. In a young patient with isolated vertical diplopia and normal brain imaging, one should consider an early pretectal syndrome and inquire after manifestations of hypothalamic dysfunction.     

Two cases of pineal germinoma with granulomatous inflammation.

We report two cases of pineal germinoma with remarkable chronic granulomatous inflammation. In the first case, the pineal mass was totally removed via an occipital transtentorial approach as symptoms were due to direct mass effect. In the second case, endoscopic third ventriculostomy and tissue biopsy was performed to alleviate worsening hydrocephalus. Pathological examination of specimens of both cases showed chronic granulomatous inflammation associated with a few germ cell tumor nests, which demonstrated positive staining for placental alkaline phosphatase. Both patients received post-operative craniospinal irradiation with no subsequent neurological deficits. Follow-up magnetic resonance imaging (MRI) of the second case showed an asymptomatic, shrunken residual tumor mass. MRI of the first case showed no residual or recurrent disease. Thus, a pineal mass with unusual features on MRI and chronic granulomatous inflammation on histopathology, should raise the suspicion of germinoma. In cases with symptomatic mass effect, open resection can be considered. In cases with lesser mass effect, conventional therapeutic modalities without resection can achieve a good outcome, as for other germinomas.     

Glomerular glucocorticoid receptor expression is reduced in late responders to steroids in adult-onset minimal change disease.

BACKGROUND: Compared to children, adult patients with minimal change disease (MCD) tend to have a slower response to steroids, but little is known about the factors influencing the steroid responsiveness in these patients. In this study, we investigated the difference in the expression of the glomerular glucocorticoid receptor (GCR) according to steroid responsiveness in 28 adult-onset MCD patients. METHODS: Based on the response to steroid treatment, the patients were divided into early responders (ER, n=20) and late responders (LR, n=8) according to the response to steroids on the basis of 4 weeks of treatment. The clinical and laboratory findings, and the glomerular mRNA and protein expression of GCR and nephrin, assessed by real-time polymerase chain reaction and immunohistochemistry, respectively, were compared between the ER and LR groups. Ten microscopic haematuric patients in whom renal biopsy was performed and revealed no histological abnormalities were included for control (C). RESULTS: The mRNA expression of GCR was significantly lower in the LR than that in the ER group (P<0.01), whereas it was comparable between the C and ER groups. GCR protein expression was also decreased in the LR compared with the C and ER groups. In contrast, there was no significant difference in nephrin mRNA expression among the three groups. On the other hand, the GCR mRNA expression correlated inversely with the time to complete remission (r= -0.49, P<0.05), but not with the amount of proteinuria at presentation. CONCLUSION: In conclusion, the levels of glomerular GCR expression may be a useful predictor of steroid responsiveness in adult-onset MCD patients.     

In utero development of the fetal gall bladder in the Korean population.

OBJECTIVE: To provide reference ranges of the fetal gall bladder in the Korean population. MATERIALS AND METHODS: Fetal gall bladder development was evaluated in well-dated, non-anomalous fetuses in the Korean population between February and April 2003 and the visualization rate and reference values were determined from the obtained data. RESULTS: The visualization rate of the fetal gall bladder increased as gestation advanced to a plateau above 90%, which was maintained between 16 and 34 weeks. The measured parameters from the fetal gall bladder had a significant positive relationship with gestational age (p = 0.000 for all cases), and the correlation of length and area with the gestational age (r = 0.741 and r = 0.690, respectively) was better than the correlation of width, height, and volume with gestational age. The repeatability coefficients and coefficients of variation between the two operators were 5.56 mm and 12.9% for the length and 344.11 mm(2) and 33.52% for the area. The median length of the fetal gall bladder in the Korean population was not significantly different from the mean length of gall bladders in the Caucasian and African-American populations (p = 0.915). CONCLUSION: We have provided reference values for the fetal gall bladder throughout the gestation period in the Korean population.     

Analgesic activities of spinal cord substance P antagonists implicate substance P as a neurotransmitter of pain sensation

Substance P (SP) injected into intraspinal (i.s.) spaces caused mice to vigorously scratch and bite their skins in an apparent reaction to a perceived cutaneous sensation. The scratching behavior was similar to the reciprocal hindlimb-scratching syndrome (RHS) described for intracranial (i.c.) SP injections. Radiotracer experiments, as well as potency and latency measurements, demonstrated that SP-induced scratching, whether induced by the i.c. or i.s. route, was due to SP receptor stimulation in the cervicothoracic cord. Similarly, biting was due to SP stimulation of the lumbosacral spinal cord. Mice coated with capsaicin, an irritant chemical, scratched and bit the coated areas in a manner similar to animals injected with i.s. SP. Standard analgesics depressed this scratching behavior elicited by topical capsaicin. Non-analgesic drugs, with the exception of amphetamine, did not affect capsaicin-induced pain. It is concluded that i.s. SP induces a painful sensory experience. Some piperazinone derivatives of substance P's C-terminal hexapeptide are shown to specifically antagonize the scratching induced by i.s. SP with little or no effect on motor behavior. These antagonists depressed scratching elicited by topical capsaicin and were analgesic on the hot-plate test. It is concluded that SP is a natural neurotransmitter for pain and that antagonism of endogenous SP systems causes analgesia.     

Saccular damage in patients with idiopathic sudden sensorineural hearing loss without vertigo

OBJECTIVE: Saccule could be damaged in patients with idiopathic sudden sensorineural hearing loss (ISSHL) with vertigo and with high-frequency sensorineural hearing loss. Thus, the saccule might be deteriorated subclinically in ISSHL cases without vertigo. Therefore, we investigated saccular damage in ISSHL patients without vertigo through vestibular evoked myogenic potentials (VEMP). STUDY DESIGN: A prospective study. SUBJECTS AND METHODS: Fifty-two patients with ISSHL without vertigo were enrolled in the study. We identified VEMP in patients with ISSHL and analyzed the association of VEMP with initial hearing threshold, each threshold according to frequency, the type of audiogram, and hearing recovery. RESULTS: For cases with absent VEMP, we found significant differences between patients with 90 dB or more hearing loss and those with a hearing loss less than 55 dB with frequencies over 1000 Hz. Patients with profound hearing loss presented significantly high abnormal and absent VEMP than patients with audiograms of other types. CONCLUSION: These findings suggest that the subclinical deterioration of the saccular neuroepithelium is associated with patients with ISSHL having profound hearing loss at the high frequency.     

Bombycis corpus Extract (BCE) Protects Hippocampal Neurons Against Excitatory Amino Acid-Induced Neurotoxicity

Bombycis corpus (BC) or Bombyx Batryticatus, a batryticated silkworm and white-stiff silkworm, is a drug consisting of the dried larva of silkworm, Mobyz mori L., dead and stiffened due to the infection of Beauveria (Bals.) Vuill. In a previous paper (Kim et al., Pharmacol. Res., 43, 12-16, 2001), BC was shown to protect amyloid-β-induced cytotoxicity. In the present study, we have found that BCE can prevent or reduce the neurotoxic actions in the hippocampus of the glutamate agonists N-methyl-D-aspartic acid (NMDA) in vitro or alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) and kainic acid in vitro. Pre-treatment with BCE (0, 1, 2, 5, and 10 μg/ml for 6-8 h) protected primary hippocampal cultures from embryonic day 18 (E18) embryos against NMDA-induced toxicity (0.1, 1, 10, and 50 mM/ml). BCE added either with NMDA (1 mM) or 1 h later had lesser, but still significant, protective actions. BCE also reduced NMDA-induced toxicity (1 mM). BCE (10 μg/ml) protected cultured neurons against the neurotoxic actions of either AMPA (25 μM) or kainic acid (1 mM) as well. Because the release of glutamate has been implicated in the neural damage after cerebral ischemia and other neural insults, these results suggest that BCE may contribute significantly to protect human brain to such damage.