Unilateral neonatal cystic disease of the kidney as first manifestation of tuberous sclerosis]

Traumatic optic neuropathy (TON) causes blindness of varied severity and occurs infrequently as a complication of closed head injury. A case is presented of TON that occurred in a patient who suffered complete T4 paraplegia from a motorcycle accident but in whom no severe head injury took place. In this case, high-dose intravenous methylprednisolone was begun for the spinal cord injury and repeated 24 hours later for the TON. Vision improved from near total blindness to 20/400 in the left eye (OS) and 20/130 in the right eye (OD). Two weeks later, however, the patient's vision suddenly worsened. Magnetic resonance imaging (MRI) using fat suppression confirmed a lesion along the optic nerve consistent with TON. A third course of methylprednisolone again led to improved vision. The steroids were then tapered orally over 2 weeks and the patient had no further relapses. Moderate to severely impaired vision of 20/ 400 OS and 20/130 OD continues to interfere with the patient's function and spinal cord rehabilitation program. It was concluded that a steroid taper was important in maintaining initial visual gains in this case. Awareness of TON and careful attention to the patient's clinical course can minimize deficit and maximize functional outcomes.     

Orthostatic hypotension with syncope--a problem of hypertensive therapy in the aged

STUDY OBJECTIVE: To assess the efficacy of the "laryngeal lift" maneuver in improving laryngoscopic visualization to facilitate endotracheal intubation. DESIGN: Blinded study. SETTING: Operating room at Meridia Huron Hospital. PATIENTS: 305 patients receiving general anesthesia for elective surgery requiring intubation. (Five patients were eliminated from the study because we elected to intubate these patients awake and sedated.) INTERVENTIONS: Following induction of anesthesia and paralysis with muscle relaxants, laryngoscopic views of each patient were evaluated by the laryngoscopist before and after the laryngeal lift was performed by an anesthesiologist assisting the laryngoscopist. Each patient served as his or her own control group. The anesthesiologist was blinded to the results obtained by the laryngoscopist. All Grade I laryngoscopic views were eliminated (198 patients). Five patients were eliminated on the basis of obesity or atlantoaxial subluxation. The laryngeal lift was performed on the remaining 102 patients, representing Grade II to Grade V laryngoscopic views. MEASUREMENTS AND MAIN RESULTS: A modification of the original classification of laryngoscopic views by Cormack and Lehane was used: Grade I = full view of glottis; Grade II = only posterior commissure visible; Grade III = arytenoids visible; Grade IV = epiglottis visible; Grade V = no glottic structure visible. In 98 of 102 cases (96%), the maneuver improved visualization by at least 1 grade. There was no evidence of change in the 4 remaining cases. CONCLUSIONS: The laryngeal lift should be part of the anesthesiologists' armamentarium in helping the laryngoscopist who is faced with Grades II, III, IV, and V laryngoscopic views to enhance visualization of the larynx and thus facilitate endotracheal intubation.     

Plasma cell interstitial pneumonia as a manifestation of multicentric Castleman's disease

A 12-year-old boy was admitted to our hospital because of abnormal shadows on a chest radiograph, slight fever, and superficial lymphadenopathy. Laboratory examination showed anemia (Hb 9.9 g/dl) and hyperimmunoglobulinemia (IgG 5469 mg/dl) without M protein. A chest CT scan showed bilateral diffuse shadows and bilateral hilar lymphadenopathy. Biopsy specimens of an inguinal lymph node and a lung showed many lymphoid follicles with germinal centers, and marked infiltration of mature plasma cells in the interfollicular area without destruction of follicular structures. The polyclonality of the plasma cells was confirmed by immunohistochemistry. The patient was not treated because these results excluded malignant disease and he was asymptomatic. At the age of 17 years, he was admitted to our hospital again because of dyspnea and a tendency to bleed. Interstitial pneumonia, hyperimmunoglobulinemia (IgG 13900 mg/dl), and anemia (Hb 6.6 g/dl) were found, along with thrombocytopenia (2.5 x 10(4)/mm3) and proteinuria. The serum interleukin-6 level was high: 177 pg/ml. Bronchoalveolar lavage fluid contained many plasma cells. Therapy with corticosteroids and immunosuppressant medication was effective. Our diagnosis was plasma cell interstitial pneumonia as a manifestation of multicentric Castleman's disease.     

Phimosis as a manifestation of chronic graft-versus-host disease after allogeneic bone marrow transplantation

Chronic GVHD is one of the major complications of BMT, involving a variety of organs, but rarely involving the genitourinary system. We report a patient who simultaneously developed extensive chronic GVHD and phimosis after BMT. From the clinical course and pathological findings, chronic GVHD was considered to be responsible for the phimosis. Despite intensive immunosuppressive therapy, the phimosis persisted. Phimosis is a rare complication after BMT, which may often remain neglected. Possibility of this complication should be considered in patients with chronic GVHD.     

Orthostatic tremor as a cause of difficulty in standing still

Orthostatic tremor is characterized by a fine tremor of lower limb muscles that produces instability while standing still and alleviates on walking or sitting. We report two patients, aged 54 and 72 years old, in whom the tremor caused falls. The clinical features, a negative neurological examination, the alleviation on walking or sitting and the good response to clonazepam allowed the diagnosis. This disease should be considered in the differential diagnosis of standing still instability.     

Spinal cord compression as initial manifestation of Hodgkin's disease. Report of a case

A case of Hodgkin's disease in which the first clinical manifestation was a radiculo-spinal compression is reported. The authors comment about the possible mechanisms to explain this radiculo-spinal compression in this granulomatosis.     

Orthostatic hypotension and low blood pressure in organic dementia: a study of prevalence and related clinical characteristics

OBJECTIVE: To determine the prevalence of orthostatic hypotension (OH), low blood pressure and dizziness, falls and fractures in patients with organic dementia. DESIGN: We prospectively studied 151 patients, assessing the prevalence of OH, hypertension, heart disorders, diabetes mellitus and the use of medication possibly associated with OH. SETTING: The patients were admitted to our psychogeriatric clinic as part of routine clinical investigation of their dementia. PATIENTS: Forty-six patients with Alzheimer's disease (AD), 28 patients with frontotemporal dementia (FTD) and 77 patients with vascular dementia (VaD) were investigated. MAIN OUTCOME MEASURE: Due to the paucity of information about the prevalence of OH in organic dementia, this study is mainly explorative in nature, thus preventing explicit hypothesis formulation. However, clinical impressions indicated a higher prevalence of OH in organic dementia than normally seen in healthy elderly. RESULTS: OH/low blood pressure was present in 39-52% of the patients. The majority reached their maximum systolic decrease within 5 minutes of standing, but in 20-30% the maximum blood pressure drop occurred after 5 minutes or later. In 38%, the systolic blood pressure drop was more than 40 mm Hg. Hypertension and heart disease was found only in AD and VaD, with no difference between those with and without OH/low blood pressure. Falls and fractures were common in orthostatic and hypotensive patients, with an incidence of more than 50% in AD and VaD. CONCLUSIONS: The results support our clinical impressions that OH and low blood pressure is common and an important factor in organic dementia.     

Headache as a manifestation of myocardial infarction

A 33-year-old pregnant woman at 26 weeks gestation, who had a history of bipolar mood disorder, type I, was admitted to the hospital for hypomania and poorly controlled diabetes mellitus. The patient had had her first episode of affective illness at age 28, after the birth of her second child. After an initial postpartum depression, she had cycled into a manic state. She had subsequently been hospitalized seven times for acute mania. A combination of valproate and chlorpromazine had proven effective in managing most of her manic episodes, while her two most severe episodes had been successfully managed with bilateral ECT.     

Matched unrelated allogeneic bone marrow transplantation for recurrent malignant lymphoma in a patient with X-linked lymphoproliferative disease (XLP)

A 14-year-old male and a maternally related cousin were diagnosed with X-linked lymphoproliferative disease (XLP) after developing recurrent B-NHL, characterized by long disease-free intervals and absence of an increased chemoresistance of the recurrent lymphomas. The demonstration of different clonal IgH gene rearrangements in two of the lymphomas from one of the patients further supports that the lymphomas were clonally unrelated. The cousin underwent matched related BMT, whereas the proband received a deliberately delayed MUD BMT in third CR. Both are in CR 68 months and 21 months, respectively, post-BMT. Delaying BMT probably contributes to reducing treatment-related morbidity. We suggest MUD BMT as a feasible curative strategy for XLP patients with B-NHL lacking matched related donors.     

Erectile dysfunction of arterial origin as possible primary manifestation of atherosclerosis]

We reviewed the records of 292 inpatients in the psychiatric ward of Kagoshima University Hospital who were referred from other medical facilities over a 5-year period in order to clarify age differences in the reason for referral. Patients were classified into groups of physically and mentally ill individuals based on indications for admission. Both groups were further divided into four subgroups based on age. The incidence of inpatients with physical illnesses increased with age. Conditions related to pregnancy, childbirth and the puerperium occurred at high frequency in female patients in the 20- to 39-year-old subgroup. Individuals in the 40- to 59-year-old and in the > or = 60 years subgroups suffered more frequently from neoplasms. The proportion of patients manifesting a defective state in all age subgroups with the exception of the under 19-year-old subgroup was significantly higher in the physical illness group than in the mental illness group. The proportion of patients in a depressive state in the > or = 60 years subgroup was significantly higher in the mental illness group than in the physical illness group. Hence, it is necessary to find a method to be able to cope with psychiatric patients with physical complications to solve this problem.     

Rhabdomyoma of unusual manifestation]

According to our knowledge in adult pericardial effusion has never been reported to be present in cases of cardiac rhabdomyoma. We present the history of an adult patient with pericardial effusion due to cardiac rhabdomyoma.     

Malignant lymphoproliferative lesions of the trachea]

Orthotopic liver transplantation is the only method of choice for many severe liver diseases with poor prognosis. The development of liver transplantation programmes is based on medical science achievements and high technology in surgery, anesthesiology and perfusiology. The experience of the Surgery Research Center, Russian Academy of Medical Sciences, made it possible to obtain positive results of liver transplantation and posttransplantation management. This in turn provided longer survival for 80% of recipients. At the same time the mortality of potential recipients was 60.4%, which is due to the greater shortage of donor organs and which is the main problem in the development of organ grafting in Russia. The fact that there is a high proportion of children who are recipients for the donor's liver requires that liver transplantation from living related donors should be developed.     

Hypoxia as a manifestation of neurogenic pulmonary dysfunction

Tryptoline, a metabolite of tryptamine, competitively inhibits serotonin accumulation in nuclei-free homogenate of rat "forebrains". After intraventricular injection the drug elicits an increase of serotonin levels in the same part of the brain whereas the monoamine oxidase activity is not altered.