Extremely rare persistent primitive artery passing through the jugular foramen with symptomatic ipsilateral carotid artery stenosis.

Primitive arteries are persistent fetal anastomoses between the carotid and vertebral-basilar circulation. Although rare, persistent primitive arteries can remain at birth. A 73-year-old woman presented with cerebral infarction to the posterior circulation caused by symptomatic common carotid artery stenosis with an unnamed and extremely rare persistent primitive artery. This anomalous vessel branched from the extracranial internal carotid artery and passed through the ipsilateral jugular foramen into the posterior cranial fossa and merged into the basilar artery. To our knowledge, this is the first case of a persistent primitive artery passing through the jugular foramen with symptomatic common carotid artery stenosis.

[A Case Report of Spontaneous Cerebrospinal Fluid Otorrhea].

Spontaneous cerebrospinal fluid(CSF)otorrhea is less common than CSF leakage caused by trauma, and rarely occurs in adults. We report an adult case of CSF otorrhea. A 71-year-old woman with no traumatic or otologic history was hospitalized due to bacterial meningitis. After hospitalization, CSF leakage started suddenly from the left external ear canal. A high resolution CT scan with intrathecal administration of contrast material revealed CSF leakage in the left ear canal and multiple bone erosions in both the tegmen mastoideum and the posterior fossa aspect of the petrous bone. We performed closure and surgery via the middle fossa approach. We identified a bone defect in the tegmen mastoideum but could not detect any obvious abnormality in the dura mater. We placed both a pericranial flap and a free abdominal fat on the middle base of the skull as sealing materials. There was no recurrence of CSF otorrhea following surgery. In this surgery, the use of a multilayered closure technique is very important to avoid the recurrence of CSF leakage.

Co-infection with Neisseria mucosa in a patient with tuberculous otitis media.

Tuberculous otitis media (TOM) is a rare manifestation caused by Mycobacterium tuberculosis with low incidence rates among extrapulmonary tuberculosis cases. Diagnosis is often delayed because of the presence of several clinical manifestations and the high prevalence of secondary bacterial infections. Few reports have attributed secondary bacterial infections in patients with TOM to commensal Neisseria. Thus, understanding the pathogenic mechanisms and clinical features of commensal Neisseria is important, considering its recent presentation as an infection-causing pathogen. Neisseria mucosa is a commensal inhabitant in humans and is generally considered non-pathogenic but can cause infection in rare cases. Here, we report an atypical secondary infection caused by Neisseria mucosa in an 81-year-old woman with TOM being treated for pulmonary tuberculosis. Direct purulent otorrhea smear microscopy revealed no acid-fast bacilli using Ziehl-Neelsen staining, whereas the phagocytosis of gram-negative cocci by white blood cells was confirmed using Gram staining. Otorrhea culture revealed the growth of N. mucosa. Subsequently, M. tuberculosis infection in the otorrhea was identified using a culture-based method. Vigilance is critical for the early detection of TOM to prevent further complications. This report raises awareness regarding TOM and provides insight into the pathogenicity of N. mucosa in otitis media.

Non-A Blood Type Is a Risk Factor for Poor Cardio-Cerebrovascular Outcomes in Patients Undergoing Dialysis.

The clinical impact of ABO blood type on cardio-cerebrovascular outcomes in patients undergoing dialysis has not been clarified. A total of 365 hemodialysis patients participated in the current study. The primary endpoint was defined as a composite including cardio-cerebrovascular events and cardio-cerebrovascular death. The primary endpoint was observed in 73 patients during a median follow-up period of 1182 days, including 16/149 (11%) with blood type A, 22/81 (27%) with blood type B, 26/99 (26%) with blood type O, and 9/36 (25%) with blood type AB. At baseline, no difference was found in the echocardiographic parameters. Multivariable Cox regression analyses revealed that blood type (type A vs. non-A type; hazard ratio (HR): 0.46, 95% confidence interval (95% CI): 0.26-0.81, p = 0.007), age (per 10-year increase; HR: 1.47, 95% CI: 1.18-1.84), antiplatelet or anticoagulation therapy (HR: 1.91, 95% CI: 1.07-3.41), LVEF (per 10% increase; HR: 0.78, 95% CI: 0.63-0.96), and LV mass index (per 10 g/m2 increase; HR: 1.07, 95% CI: 1.01-1.13) were the independent determinants of the primary endpoint. Kaplan-Meier curves also showed a higher incidence of the primary endpoint in the non-A type than type A (Log-rank p = 0.001). Dialysis patients with blood type A developed cardio-cerebrovascular events more frequently than non-A type patients.

Microinvasive squamous cell carcinoma arising within seborrheic keratosis.

Squamous cell carcinoma (SCC) arising within seborrheic keratosis (SK) is rare. We report an 84-year-old woman who presented with a rapidly growing black tumor on her left palpebral eyelid of several years' duration. Clinical examination revealed an elevated hemorrhagic black tumor that measured 0.9 x 0.9 x 0.6 cm. A clinical diagnosis of SK was made, but basal cell carcinoma could not be ruled out; therefore, excision with wide margins was performed. Histologically, the tumor was symmetrical and composed of benign basaloid cells with pseudohorn cysts in a reticulated pattern. The tumor showed heavy melanin deposition. The features were indicative of SK. An atypical cell cluster was seen in the central low area. These cells showed keratin pearls, individual keratinization, mitotic and apoptotic figures, nuclear atypia, and microinvasion, indicating microinvasive SCC. Immunohistochemistry revealed the microinvasive SCC area was true SCC. This case suggests that microinvasive SCC can arise within pigmented reticulated SK.

Malignant glomus tumor of the palm: a case report.

The author herein reports on a glomus tumor of the palm. A 71-year-old man consulted our hospital because of a tumor on the left palm. The tumor was deeply seated, and MRI and CT showed a deep cystic tumor adjacent to the bone. An excision of the tumor was therefore performed. Grossly, the tumor was red and partly cystic. The tumor was well defined from the surrounding tissues, and measured 25 × 24 × 22 mm. Microscopically, the tumor consisted of epithelioid perivascular cells (glomus cells) located around the blood vessels. Cystic changes and hyalinization areas were scattered. The tumor cells had moderately hyperchromatic nuclei. Nuclear pleomorphism was noticed, nucleoli were absent and apparent mitotic figures were not recognized. There were no areas of necrosis. Immunohistochemically, the glomus cells were positive for vimentin and α-smooth muscle actin. They were negative for cytokeratins, epithelial membrane antigen, CD34, CD31, factor VIII-related antigen, S100 protein, p53 protein, desmin and melanosome. The Ki-67 labeling was 5%. The tumor was diagnosed as a malignant glomus tumor because of its deep location and size > 2 cm , according to the criteria of one group. The tumor recurred 12 months later, and a further excision was performed. No metastases were found. Now, the patient is being strictly followed up.

[A case of proximal-type Hirayama disease associated with neck axial rotation].

Hirayama disease is characterized by juvenile onset of unilateral muscular atrophy of a distal upper extremity. The pathogenic mechanism of Hirayama disease is cervical cord compression by the posterior dura with forward displacement in the neck flexion position. A few cases of 'proximal-type Hirayama disease' have been described as showing muscular weakness and atrophy of the proximal upper extremities caused by the pathogenic mechanism similar to that of Hirayama disease. We report herein the case of a 16-year-old boy with proximal-type Hirayama disease, who developed symptoms after he began kyudo (Japanese traditional archery). Neurological examination revealed bilateral weakness of the muscles innervated by C5 and C6 segments (the deltoid, biceps brachii, brachioradialis), bilateral mild sensory disturbance in the radial side of the forearm, absent tendon reflexes of the biceps brachii and brachioradialis with preserved triceps reflex, pyramidal signs of the bilateral lower extremities (pathologically brisk reflexes of lower extremities, Babinski's signs). MR images in the neck flexion position showing expansion of the posterior extradural space and forward displacement of the spinal cord at the C3/4, C4/5, C5/6 and C6/7 disk levels. CT myelogram revealed spinal cord compression not only in neck flexion but also in neck left axial rotation. His symptoms improved after the restriction of neck flexion and axial rotation. Weakness of the upper extremities improved after 2 months. Pyramidal signs of the lower extremities disappeared after 18 months. The pathogenic mechanism in this case may be associated with not only neck flexion but also neck axial rotation.

Relationship between the degree of endolymphatic hydrops and electrocochleography.

The purpose of this study was to evaluate the relationship between the endolymphatic space image obtained using magnetic resonance imaging (MRI) and the results of electrocochleography. Electrocochleography recordings were obtained from 25 ears of 24 patients, who underwent MRI 1 day after the intratympanic injection of gadolinium diethylenetriamine pentaacetic acid bismethylamide. The average summating potential to action potential (SP/AP) ratio in patients with significant endolymphatic hydrops in the cochlea was 54 +/- 17%. However, in some patients who had significant endolymphatic hydrops in the cochlea, the SP/AP ratio was not enlarged. This may imply that elevation of the SP/AP ratio is related to not only the degree of endolymphatic hydrops but also to the persistence of hydrops.

[Current Status of Clinical Ethics and Creation of Ethical Organizational Culture].

When an ethical problem arises in clinical practice, the process of discussion aimed towards a solution is important. The decision should be made by a multidisciplinary team rather than by an individual clinician. In clinical practice, highly feasible solutions are required. Clinical ethics consultation is useful as a service that supports the decision-making process. A future issue is to foster an organizational culture of openness that enables professionals to solve the problem in practice.

Image evaluation of endolymphatic space in fluctuating hearing loss without vertigo.

The objective of the present study was to investigate endolymphatic space images in patients with fluctuating hearing loss without vertigo, and to elucidate its underlying pathophysiology. Eight patients with fluctuating hearing loss without vertigo were included in this study. 3T MRI was taken, 24 h after intratympanic injection of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA). Electrocochleography and VEMP tests were performed to evaluate cochlear and vestibular functions. Endolymphatic hydrops were observed both in the cochlea and in the vestibule of all eight patients. Three patients out of six whose summating potential/action potential (SP/AP) ratio was recordable showed an elevation of SP/AP ratio. In the two patients with remarkable endolymphatic hydrops in the vestibule, VEMP was absent from the affected ear. In conclusion, 3T MRI after intratympanic injection of Gd-DTPA revealed endolymphatic hydrops both in the cochlea and in the vestibule in the patients with fluctuating hearing loss without vertigo.

[Autopsy case of Lissauer's general paresis with rapidly progressive left hemiparesis].

A 48-years-old man presented with slowly progressive bradykinesia, personality change and rapidly progressive left hemiparesis. On admission, he presented dementia, poor judgment, left hemiparesis. MRI revealed a widespread high intensity area in right hemisphere and MRA was almost normal. Serological tests of serum and CSF demonstrated high titers of antibodies to Treponema pallidum. He was treated for syphilis with daily penicillin injections without improvement. He died of sepsis eight months after admission. At autopsy, the brain weighed 1,100 g and the right cerebral hemisphere was atrophic, especially in frontal base, temporal, parietal, angular, and posterior regions covered by thickened, fibrotic leptomeninges. Microscopically, chronic meningoencephalitis was observed. Severe neuronal loss with gliosis was seen in the right cerebral cortices. Scattered rod-shaped microglia and inflammatory cell infiltration were visible in the cerebral parenchyma. The dorsal column of the spinal cord was not involved and meningovascular syphilis was unclear. The distribution of the encephalitic lesions was well correlated with the clinical and neuroradiological findings. This was a rare autopsy case presenting Lissauer's general paresis, clinically manifesting as rapidly progressive stroke-like episode.

Effect of an enlarged endolymphatic duct on bone conduction threshold.

An enlarged endolymphatic duct and sac (EDS) that makes contact with the cerebrospinal fluid­dural interface plays an important role in the pathway of bone conduction and enhances bone conduction at lower frequencies. Objectives. We investigated whether the bone conduction threshold was improved when the EDS was enlarged. Subjects and methods. Twenty-three patients (46 ears) with large vestibular aqueducts underwent standard pure tone audiometry (PTA) and magnetic resonance imaging (MRI) to investigate the relation between the diameter of the endolymphatic duct (ED) and the air or bone conduction threshold. We also investigated the relation between the volume of the EDS and the air or bone conduction threshold. Results. All ears had a mixed type hearing loss. The air­bone gaps were significantly larger at 250 and 500 Hz than at higher frequencies. The bone conduction thresholds were significantly lower at 250 Hz and 1000 Hz when the diameter of the ED was large, whereas there was no relation between the diameter of the ED and the air conduction threshold. In addition, there was no correlation between the volume of the EDS and air or bone conduction thresholds.

Otoacoustic emissions, ear fullness and tinnitus in the recovery course of sudden deafness.

OBJECTIVE: This study aimed to investigate how the symptoms of ear fullness, tinnitus and otoacoustic emissions (OAE) change in relation to the recovery course of pure tone audiometry thresholds (PTA) in sudden deafness (SD). METHODS: This study analyzed follow-up data on ear fullness, tinnitus and otoacoustic emissions of eight SD patients with good hearing improvement (Group A) and eight SD patients with poor hearing improvement (Group B) in an attempt to elucidate the behavior of these symptoms in their recovery course. This study was done until there was no change in the PTA for more than 1 week and hearing recovery was no longer expected. RESULTS: All patients from both groups had ear fullness and tinnitus in association with the onset of SD. However, these symptoms improved only in Group A. showing a significant relationship between PTA recovery and the improvement of ear fullness annoyance (P<0.05), presence of tinnitus (P<0.01), improvement in tinnitus loudness (P<0.01) and in tinnitus annoyance (P<0.01). No patients (Group A or B) had OAE responses at their first examination. In Group A, OAE responses appeared simultaneously with improvement of hearing levels in five patients (63%) and it appeared later than hearing levels improvement in the other three patients (37%) from Group A. No patient from Group B showed OAE response on follow-up. CONCLUSION: SD patients with good hearing improvement (Group A) tended to have OAE responses and the sensations of the ear fullness and tinnitus improved almost simultaneously with hearing level improvement. Their PTA improvement occurred primarily in the low to mid frequencies, with high frequencies showing less recovery. When hearing recovery was not full, OAEs did not reappear for these frequencies. Patients with poor hearing improvement tended to have absent OAEs and persistent ear fullness and tinnitus.

Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings in a patient with cochlear otosclerosis.

A 51-year-old man had progressive hearing loss over more than 15 years. He had bilateral sensorineural hearing loss (SNHL). Computed tomography (CT) showed extensive bilateral demineralization of the cochlear capsule, which is characteristic of diffuse cochlear otosclerosis. Three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) of magnetic resonance imaging before enhancement revealed high signals in the cochlea and vestibule. Postcontrast 3D-FLAIR revealed enhancement of the basal turn of the left cochlea. This is the first published case of the breakdown of the blood-labyrinth barrier in a patient with cochlear otosclerosis. Our findings suggest that the breakdown of the blood-labyrinth barrier is associated with a part of SNHL in cochlear otosclerosis.

[Case of cutaneous Pseudallescheria boydii infection caused by trauma].

Pseudallescheria boydii is a ubiquitous filamentous fungus. We report a case of cutaneous P. boydii infection of the left knee in a 79-year-old Japanese man who was receiving oral predonisolone (25 mg/day) for radiation pneumonitis after radiation therapy on left breast cancer. He presented with a 2-week-history of a lesion on the left knee. A biopsy specimen from the skin lesion revealed granulomatous inflammation with hyphae. Culture of the pus from the skin specimen confirmed the diagnosis of cutaneous P. boydii infection. rDNA ITS sequence was analyzed to confirm the mycological diagnosis. The patient was treated orally with 200 mg/day of itraconazole. The lesion was gradually cured and left a hypertrophic scar. Cutaneous injury may be responsible for an incidence of localized infection. Such rare fungus infection in immunocompromised patients who have a persistent traumatic skin ulcer needs to be ruled out. An opportunistic infection in immunocompromised patients can be life-threatening and prompt treatment based on accurate diagnosis is important.

[Case report of nocardia brain abscess that needs to be distinguished from metastatic brain tumor].

We report a 62-year-old woman presenting with nocardia brain abscess that mimics metastatic brain tumor. Six months before admission to our hospital, she presented with persistent hemoptysis, and a friable endobronchial mass was detected at another hospital. However, a definite diagnosis had not been made, and then she presented with hemianopia. Her laboratory analysis results showed that she had no immunological abnormalities. T1-weighted magnetic resonance images of the brain with Gd-DTPA revealed hyperintensive multiple masses with surrounding edema. We suspected this case as metastatic brain tumor of unknown origin, and the patient underwent biopsy of the supraclavicular lymph node. Histopathology results indicated abscess, and gram staining of material obtained from the abscess showed Gram-variable rods, which were determined upon culture as acid-fast Gram-positive branching rods and identified as Nocardia asteroides. Accordingly, we diagnosed the patient as having nocardia brain abscess, and she was treated with an oral antibiotic (TMP-SMX). Four months later, the abscesses disappeared. The patient recovered after prolonged TMP-SMX therapy with no recurrence in over 9 months.

Visualization of endolymphatic hydrops in patients with Meniere's disease.

OBJECTIVE: Recently, there have been many reports of intratympanic gentamicin therapy for the treatment of intractable Meniere's disease. Intratympanic administration of steroids has also been used to treat sudden sensorineural hearing loss. We attempted to visualize how the intratympanically administered drug enters the inner ear. METHODS: Gadolinium hydrate diluted eightfold with saline was injected intratympanically through the tympanic membrane using a 23 G needle in nine patients with inner ear diseases. With a 3 Tesla magnetic resonance imaging (MRI) unit, three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) imaging was performed. RESULTS: 3D-FLAIR MRI clearly revealed that the gadolinium entered the perilymphatic space and delineated the perilymphatic and endolymphatic spaces of the inner ear. In patients with endolymphatic hydrops, the perilymphatic space surrounding the endolymph was small or had disappeared. Gadolinium appeared first in the scala tympani of the basal turn of the cochlea and the perilymphatic space of the vestibule. One day after the intratympanic injection of gadolinium, the gadolinium was observed in almost all parts of the perilymph. Six days after the intratympanic injection, the gadolinium had almost disappeared from the inner ear. CONCLUSION: We reported the first visualization of endolymphatic hydrops in patients with Meniere's disease. The relationship between the image of the endolymphatic space and functional tests, such as electrocochleography and vestibular-evoked myogenic potential, must be examined in the near future. It is important for the development of intratympanic drug therapies for inner-ear diseases to investigate how the drugs enter and leave the inner ear.

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma with a CD15(+)CD30(-) phenotype.

Primary cutaneous aggressive epidermotropic CD8(+) T-cell lymphoma (CD8(+)TCL) is an extremely rare entity with distinct clinicopathological features. While the CD15 antigen is typically associated with classic Hodgkin's lymphoma, aggressive peripheral T-cell lymphomas, including advanced stage cutaneous T-cell lymphomas, rarely express this molecule. We report a case of primary cutaneous aggressive epidermotropic CD8(+)TCL, in which lymphoma cells are CD15(+)CD30(-) with a medium-to-large pleomorphic phenotype. Although the functional characteristics of CD15 expression in the cutaneous lymphomas are not fully understood, the poor prognosis of primary cutaneous aggressive epidermotropic CD8(+)TCL might be associated with the presence of this molecule in our case.