Treatment of progressive paralysis associated with cervical myelopathy and suspected amyotrophic lateral sclerosis: A case report.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is an intractable progressive disease, with an incidence of 2.2- 2.3 per 100,000 individuals, which is not extremely low. ALS symptoms are accompanied by spinal myeloradicular motor deficit; its differential diagnosis is must because progressive paralysis needs emergency surgery. CASE DESCRIPTION: A 64-year-old man with suspected ALS showing progressive paralysis with cervical myelopathy was diagnosed as normal after performing a nerve conduction study preoperatively. Postoperative diffuse fasciculation after posterior decompression allowed the diagnosis of ALS through needle electromyography (EMG). Thereafter, the patient's condition slowly deteriorated and he died after 16 months. CONCLUSION: Surgery might aggravate ALS symptoms; however, surgery for progressive paralysis in patients with suspected ALS is required for distinguishing patients with non-ALS paralysis. Approximately 70% of cases have spinal-onset ALS lacking typical cranial nerve symptoms; thus, to prevent unnecessary surgery, surgeons should at least know the characteristic features of ALS and should be aware that early diagnosis requires needle EMG for definitive diagnosis of ALS.

Development of a microsatellite marker set applicable to genome-wide screening of cynomolgus monkeys (Macaca fascicularis).

To develop a microsatellite marker set applicable to genome-wide screening of cynomolgus monkeys (Macaca fascicularis), 148 microsatellite markers were selected from the human genome database. The polymorphisms and inheritance of PCR products were determined by screening twenty unrelated monkeys and by analysis of three families, respectively. As a result, 106 primers (72%) gave PCR products of the size expected for humans and rhesus monkeys. Among these products, polymorphism and single-gene inheritance in cynomolgus monkeys was observed for 66 markers (62%). The average number of alleles at the 66 polymorphic loci was 5.86 (range 2-10), and average heterozygosity was 0.63 (range 0.10-0.88). This is the first report of microsatellite markers for cynomolgus monkeys. Chromosomal mapping of these markers is now in progress.

A case report of compensation in daily life of a person with memory problems: detailed functional use of memory aids over a five-year period.

Numerous studies have been conducted on memory aids for memory-impaired people. However, it is not known how they use these memory aids in a functional, practical way. A 20-year-old patient (MH) was monitored for five years to identify what memory aids or other means she used and how she used them to compensate for her memory problems, e.g., forgetting what was said by others in a few minutes and getting lost or turning in the wrong direction on a walk or in a building. Results indicated MH did not necessarily always use memory aids such as a notebook or calendar to compensate for her memory problems, although MH and her mother reported that she frequently used them in daily life. She coped with memory problems by using various "resources" besides the memory aid. These facts suggest that it may be necessary to redefine functionally useful compensations, which include both memory aids and resources in daily life.

Recognition and its impairment regarding autobiographical memories in a patient with hippocampal amnesia.

Autobiographical memories of one case (Y.K.) were assessed before and after onset of hippocampal amnesia. He was a 56-yr.-old male patient who used to work in an office. The findings can be described as follows. First, Y.K.'s recognition performance regarding his premorbid and postmorbid personal semantics along with premorbid autobiographical incidents was significantly greater than chance, and recognition of premorbid autobiographical incidents was within chance. Given information before onset, a relationship was suspected between frontal lobe dysfunction and Y.K.'s autobiographical problem. The possibility that an amnesic patient could acquire semantic information after onset is discussed.

Developmental order driving the relationship between executive functions and theory of mind: a case study.

Several studies have suggested there is a developmental link between executive functions and theory of mind. However, the developmental order driving the relationship is not well understood. The main reason is that the development of executive function parallels the development of theory of mind in normally developing children. In this paper, a case (H.Y.) is reported. H.Y. had lived in a persistent vegetative state for 6 years after encephalitis at the age of 10. He showed a developmental order driving the relationship between executive functions and theory of mind. These findings are consistent with recent suggestions that development of executive function might be important as a predecessor of either the ability to understand false beliefs or the ability to express that understanding.

Isolation and characterization of a new simian retrovirus type D subtype from monkeys at the Tsukuba Primate Center, Japan.

Exogenous type D simian retroviruses (SRV/D) are prevalent in captive and feral populations of various macaque monkeys. Thus far, five subtypes of SRV/Ds have been reported, three of which (SRV-1, -2 and -3) have been molecularly characterized. Two SRV/D strains (N27 and T150) were isolated from seropositive cynomolgus macaques at the Tsukuba Primate Center (TPC) in Japan, showing clinical signs of SRV/D infection, including anemia and persistent unresponsive diarrhea. Electron microscopy demonstrated that both SRV/D isolates have a virion morphology typical of type D retrovirus. The SRV/D N27 and T150 isolates were essentially the same based on sequence analysis. From homology analysis of the entire gag sequence, the N27 isolate is closely related to the other known SRV/Ds but is distinct from the three molecularly characterized SRV/Ds. Thus, we have tentatively designated the N27 and T150 viruses isolated from TPC cynomolgus macaques as SRV/D-Tsukuba (SRV/D-T).

Survey of captive cynomolgus macaque colonies for SRV/D infection using polymerase chain reaction assays.

The exogenous simian type D retroviruses (SRV/Ds) are prevalent in macaque monkeys and sometimes cause immunodeficiency with anemia, weight loss, and persistent unresponsive diarrhea. SRV/D isolates are classified as subtypes 1 to 6, and the entire sequences of the gag region of SRV/D-1, -2, and -3 and SRV/D-Tsukuba (SRV/D-T) have been determined. We designed specific primers in the gag region of SRV/D-T that enabled us to directly detect by polymerase chain reaction (PCR) SRV/D-T proviral DNA sequences in DNA extracted from whole blood. Using this assay and another PCR assay that detects multiple SRV/D subtypes, we performed a survey for SRV/D infection in our specific pathogen-free (SPF) and conventional colonies at Tsukuba Primate Center (TPC). In the SPF colony, no SRV/D signal was detected in any animal. On the other hand, SRV/D-T was detected in 11 of 49 animals (22.5%) in the conventional colony. SRV/D-T was the only SRV/D subtype detected. Consequently, SRV/D-T is the major SRV/D subtype present in cynomolgus monkeys at TPC.

A Ferromagnetic Interaction between Cu(2+) Centers through a [CrO(4)](2-) Bridge: Crystal Structures and Magnetic Properties of [{Cu(acpa)}(2)(&mgr;-MO(4))] (M = Cr, Mo) (Hacpa = N-(1-Acetyl-2-propyridene)(2-pyridylmethyl)amine).

The reaction of [Cu(acpa)](+) with [MO(4)](2)(-) (Hacpa = N-(1-acetyl-2-propyridene)(2-pyridylmethyl)amine and M = Cr and Mo) in water-methanol or water-acetonitrile solution affords dinuclear copper(II) complexes with metalate bridges, [{Cu(acpa)}(2)(&mgr;-CrO(4))].4CH(3)OH.4H(2)O (1) and [{Cu(acpa)}(2)(&mgr;-MoO(4))].4H(2)O (2), respectively. The crystal structures and the magnetic properties have been studied. Complexes 1 and 2 are isomorphous and the structures are made up of discrete dimers in which two copper(II) ions are bridged by the [MO(4)](2)(-) anion. The coordination geometry about the copper(II) ions is square planar with a N(2)O chelate group from acpa and an oxygen atom from [MO(4)](2)(-). Magnetic susceptibility measurements for 1 revealed that a ferromagnetic interaction between copper(II) ions is propagated through the [CrO(4)](2)(-) bridge and the coupling constant (2J) was evaluated to be 14.6(1) cm(-)(1) (H = -2JS(1).S(2)). In 2, two copper(II) ions bridged by [MoO(4)](2)(-) anion are antiferromagnetically coupled with the 2J value of -5.1(4) cm(-)(1). The ferromagnetic interaction in 1 is explained by means of the orbital topology of frontier orbitals. Crystal data: 1, monoclinic, space group P2(1)/m, a = 8.349(2) Å, b = 17.616(3) Å, c = 10.473 Å, beta = 107.40(2) degrees, Z= 2; 2, monoclinic, space group P2(1)/m, a = 8.486(2) Å, b = 18.043(3) Å, c = 9.753(2) Å, beta = 95.82(2) degrees, Z = 2.

Late-onset hearing loss in a mouse model of DFN3 non-syndromic deafness: morphologic and immunohistochemical analyses.

Recently, we reported that homozygous males and females of a mouse model of DFN3 non-syndromic deafness generated by the deletion of Brn-4 transcription factor showed profound deafness due to severe alterations in the cochlear spiral ligament fibrocytes from the age of 11 weeks, whereas no hearing loss was recognized in young female heterozygotes. It is known that a part of obligate female carriers of DFN3 showed progressive hearing loss. In the present study, we examined the late-onset effect of Brn-4 deficiency on the hearing organ of the mouse. About one third of heterozygous female mice revealed late-onset profound deafness at the age of 1 year. Furthermore, in these deafened heterozygotes, characteristic abnormalities in Reissner's membrane attachment and type II fibrocytes in the suprastrial zone became evident under light microscope, similar to homozygous female mice. A significant reduction in the immunoreactivity of connexin 26 (Cx26), connexin 31 (Cx31), Na,K-ATPase and Na-K-Cl cotransporter in the spiral ligament fibrocytes was observed in aged heterozygotes showing late-onset profound deafness. The late-onset phenotype observed in heterozygous mutant mice, being consistent with the progressive deafness observed in human female heterozygotes, may be explained by alterations of the ion transport systems in the spiral ligament fibrocytes.

Endocochlear potential and endolymphatic K+ changes induced by gap junction blockers.

OBJECTIVE: To examine the effects of gap junction blockers on the endocochlear potential (EP) and endolymphatic potassium concentration ([K(+)](e)). MATERIAL AND METHODS: The EP and [K(+)](e) were monitored using double-barreled ion-selective microelectrodes in the second turn of the guinea pig cochlea during perilymphatic perfusion. RESULTS: When the perilymphatic scalae of the cochlea were perfused with artificial perilymph containing 10 mM n-heptanol the EP was decreased by -8.8+/-1.4 mV (n=10), and this was accompanied by a decline in the [K(+)](e) of -6.7+/-2.1 mM (n=6). Perilymphatic application of 10 mM hexanol also produced declines in both the EP and [K(+)](e). In control studies, perilymphatic perfusion with 10 mM ethanol showed no remarkable changes in either the EP or [K(+)](e). Anoxia during perfusion with heptanol resulted in the generation of a negative EP, similar to the situation in controls. CONCLUSIONS: A decline in the EP together with a lowering of [K(+)](e) induced by long-chain n-alkanols, which act as gap junction blockers, may be explained by an interruption in potassium ion transport related to a gap junction dysfunction.

Surgical treatment of subperiosteal abscess of the orbit: Sendai's ten-year experience.

OBJECTIVE: One of the common complications of acute infection of the paranasal sinus is cellulitis of the orbit. This is secondary to the spread of infection through the very thin bony wall between the ethmoid sinuses and the orbit, the roof of the orbit in frontal sinusitis, and the floor of the orbit in maxillary sinusitis. When the infection does not penetrate the periorbita, it dissects under the periosteum and forms subperiosteal abscess. METHODS: We experienced 10 patients with subperiosteal abscess for 10 years from 1992 to 2002 that required surgical drainage. The age of the patients ranged from 4 to 76 years including five males and five females. RESULTS: Successful and safe drainage of the abscess with endoscopic sinus surgery was obtained in four patients while six patients required external surgery. The selection of the surgical approach depended upon the localization of abscess in the orbit. Five of the seven patients with visual disturbance resulted in a complete recovery of vision after surgery. However, there was no improvement of visual acuity in the other two patients who had already shown severe damage of vision prior to treatment. CONCLUSION: Ocular symptoms such as exophthalmos, double vision, and ptosis subsided completely in all patients. It is suggested that prompt diagnosis and surgical drainage before severe loss of visual acuity rescue or recover the vision.

Localization of aquaporins, water channel proteins, in the mouse eustachian tube.

CONCLUSION: Immunolocalization of the subtypes of water channel proteins, aquaporins (AQPs), was detected in the mouse eustachian tube (ET). AQPs are located continuously from the serous glands and the capillary vessels to the luminal side in the ET epithelium and may play an important role in the transportation of water to the surface of the ET lumen through the epithelium. OBJECTIVES: Although the water supply to the surface of the ET lumen is considered to be essential for closing of the ET, the pathway of the water in the ET is not fully understood. Since AQPs, a group of water transport proteins, have been reported to regulate water homeostasis, we examined the location of AQPs in the mouse ET. MATERIALS AND METHODS: Nine subtypes of AQPs were examined in paraffin embedded ETs of adult mice using the avidin-biotin peroxidase complex method of immunohistochemistry. RESULTS: Four subtypes of AQPs were detected in the mouse ET. AQP-1 was detected in fibroblasts, endothelial cells of capillary vessels and cartilage cells. AQP-3 was distinctly detected in the basal membrane of epithelial cells. AQP-4 was detected in the basal membrane of epithelial cells. AQP-5 was expressed in the luminal side of the ET epithelial cells and also in the apical surface of the cells of the serous glands.

Specific expression of Gsta4 in mouse cochlear melanocytes: a novel role for hearing and melanocyte differentiation.

Mammalian pigment cells produce melanin as the main pigment. Melanocytes, one of the two types of mammalian pigment cells, differentiate from the neural crest and migrate to a variety of organs during development. Melanocytes exist not only in the skin but also in other sites such as the cochlea where they are essential for hearing. Mitf(mi-bw) is one of the known recessive alleles of the mouse microphthalmia-associated transcription factor (Mitf) locus, which is essential for the development of pigment cells. Homozygous Mitf(mi-bw)/Mitf(mi-bw) mice have a completely white coat with black eyes and are deaf due to the lack of melanocytes. By comparing gene expression profiles in cochleae of wild-type and Mitf(mi-bw)/Mitf(mi-bw) mice, we now demonstrate the specific expression of glutathione S-transferase alpha 4 (Gsta4) in the stria vascularis. Gsta4 encodes one of the cytosolic glutathione S-transferases (GSTs) which participate in detoxification processes of many tissues. This gene is specifically expressed in intermediate cells of the stria vascularis, suggesting a novel function for cochlear melanocytes. Moreover, among mammalian pigment cells, expression of Gsta4 was restricted to cochlear melanocytes, suggesting that melanocytes in various tissues differentiate from one another depending on their location.

Detection of SRV/D shedding in body fluids of cynomolgus macaques and comparison of partial gp70 sequences in SRV/D-T isolates.

We previously reported the isolation of a novel subtype of SRV/D-Tsukuba (SRV/D-T) from two cynomolgus monkeys (Macaca facicularis) in the breeding colony of Tsukuba Primate Research Center (TPRC). We surveyed for SRV/D infection in the TPRC cynomolgus colony using SRV/D-specific PCR primer sets designed based on the entire gag region sequence. The only SRV/D subtype detected in the colony was SRV/D-T with a positive infection rate of 22.4% (n = 49). It has been reported that the mode of transmission of SRV/D is via contact with virus shed in the body fluids. In this report, to investigate the infection route of SRV/D-T in monkeys at TPRC, we performed virus isolation and PCR for detection of the SRV/D genome from peripheral blood mononuclear cells (PBMCs), plasma, saliva, urine, and feces. Virus isolation and PCR detection were positive in plasma, saliva, urine, and fecal samples from all monkeys on which virus was isolated from PBMCs. This suggests that the spread of SRV/D-T infection in TPRC is via contact with virus shed in saliva, urine, and/or feces. Also, comparison of sequences of gp70 on multiple SRV/D-T isolates revealed that there was little intra- and inter-monkey variation, suggesting that SRV/D-T is fairly stable.

Comparative immunohistochemical localizations of aquaporin-1 and aquaporin-4 in the cochleae of three different species of rodents.

The species-specific difference of the immunohistochemical localization of aquaporin-1 (AQP1) and aquaporin-4 (AQP4) was investigated in the cochleae of the 3 different species of rodents, including guinea pig, mouse and Mongolian gerbil. In the guinea pig cochlea, intense AQP1-like immunoreactivity was present in the type III fibrocytes in the spiral ligament and the mesenchymal cells just below the basilar membrane. Immunostaining was also found in some type IV fibrocytes in the spiral ligament, fibrocytes in the spiral limbus and mesenchymal cells lining the perilymphatic space against the bony otic capsule. In contrast, no remarkable immunostaining was found in the basilar membrane of the mouse cochlea. The medial part of the Reissner's membrane was positively immunostained with anti-AQP1 antibody only in the mouse cochlea. In the gerbil cochlea, AQP1-like immunoreactivity was weak compared with the other 2 species. AQP4 was found in the cochlear supporting cells, including Claudius cells, Hensen's cells and inner sulcus cells of the 3 rodent species. AQP4 was also expressed in some interdental cells of the spiral limbus. Weak immunoreactivity was also found in the root cells only in the upper turns of the guinea pig cochlea. In contrast, no detectable immunoreactivity was found in the root cells of the other 2 species. The results obtained in the present study provide the first evidence for the existence of the species differences in the expression of the AQP1 and AQP4 proteins in the rodent cochlea.

Expression of glutamate transporter GLAST in the developing mouse cochlea.

The immunohistochemical localization of glutamate transporter GLAST in the developing mouse cochlea was studied at different ages between 0 and 30 days after birth (DAB). In the adult mouse cochlea, intense GLAST-like immunoreactivity was found in the supporting cells adjacent to the inner hair cells of the organ of Corti, the type II and suprastrial fibrocytes of the cochlear lateral wall, the fibrocytes of the spiral limbus and the satellite cells surrounding the spiral ganglion cells. At 0 DAB, weak GLAST-like immunoreactivity was found in the supporting cells around the immature inner hair cells. Immature fibrocytes in the cochlea were also positively immunostained. At 3 DAB, weak immunostaining of GLAST appeared in the immature satellite cells in the spiral ganglion. The GLAST-like immunoreactivity in the supporting cells around the inner hair cells, in the fiborocytes in the spiral ligament and the spiral limbus and in the satellite cells in the spiral ganglion increased progressively during the second postnatal week, and reached the adult level at 15 DAB. This time course correlates with the electrophysiological onset and maturation of the mouse auditory function, which is mediated by glutamatergic neurotransmission. These results suggest that the expression of GLAST may be needed for the efficient removal and metabolism of the released glutamate in the cochlea and may play important roles in the onset and maturation of the auditory system.

Endoscopic sinus surgery in cases of cholesterol granuloma of the maxillary sinus.

Cholesterol granuloma is usually associated with middle ear disease and is very rare in the paranasal sinuses. We report a case of cholesterol granuloma originating in the maxillary sinus of a 52-year-old female. Endoscopic sinus surgery was performed on the left maxillary sinus, and the cholesterol granuloma was successfully removed by the middle meatal antrostomy. Light microscopic examination showed granulomatous tissue with typical cholesterol clefts, multinucleated foreign body giant cells, small areas of hemorrhage, hemosiderin-laden macrophages and plasma cells. We also describe the details of the endoscopic surgical techniques employed in the treatment of this disorder.