[Emergency operation and hypothermic therapy for Stanford type A acute aortic dissection in the state of coma].

We report of a 77-year-old woman who was admitted to our hospital in coma by emergency. A computed tomography scan revealed acute aortic dissection (Stanford type A). We established selective antegrade cerebral perfusion within 3 hours of the onset and then performed ascending aortic replacement. In the state of hypothermia (35 degrees C), the patient was weaned from cardiopulmonary bypass. The patient was kept hypothermic until the operation was completed. We kept mild hypothermia (34.5 degrees C) in intensive care unit (ICU) for 40 hours. The patient was extubated at 94 hours after the operation. The patient was discharged from the hospital on foot on postoperative day 21.

Impedance pharyngography to assess swallowing function.

We evaluated impedance pharyngography (IPG), a new method to assess swallowing function based on changes in the electrical impedance of the neck during swallowing. The electrical impedance of the neck, recorded by the 4-electrode method, changed with the equivalent cross-sectional area of the route of the electric current due to reflex activities of related organs during swallowing. IPG waveforms accurately recorded the swallowing process, therefore. We recommend IPG for assessing swallowing function because we expect IPG to provide the following advantages over conventional diagnostic techniques: it is a quantitative method that allows for the objective assessment of swallowing function; it is a simple procedure that is convenient for the patient and could be used for screening; it is inexpensive and non-invasive, so could be performed repeatedly in situations such as rehabilitation; and it uses highly portable equipment suitable for community use.

Magnetic resonance imaging of thoracic epidural venous dilation in Hirayama disease.

Hirayama disease is a nonprogressive, asymmetric amyotrophy of the hands and forearms, possibly caused by compression of the lower cervical cord on neck flexion. The authors used phase-contrast MR angiography to study a patient with this disorder and observed abnormal spinal epidural venous dilation on neck flexion. In addition to mechanical compression of the lower cervical cord, venous congestion in the spinal canal may have a role in promoting anterior horn damage.

Japanese family with parkinsonism, depression, weight loss, and central hypoventilation.

BACKGROUND: The authors describe the clinical and pathologic characteristics of the Fukuoka 1 family, the first Japanese family recognized to have hereditary parkinsonism associated with depression, weight loss, and central alveolar hypoventilation. METHODS: The pedigree contains 14 family members spanning four generations, with five affected individuals. All available medical records were collected for affected members, including autopsy results. RESULTS: The inheritance pattern was autosomal dominant. The average age at onset of symptoms was 41 years. All patients had parkinsonism characterized by rigidity, bradykinesia, and resting and postural tremor. Bradykinesia and depression developed in the proband at age 43 years. He responded to levodopa in the initial stage only. A year later, he had weight loss and central hypoventilation leading to respiratory failure. Symptoms developed in his cousin at age 38 years. The proband's father developed a resting tremor and depression at age 43 years. The tremor was initially responsive to levodopa therapy, but the disease was relentlessly progressive, leading to severe bradykinesia, rigidity, weight loss, and respiratory distress. He died of respiratory failure at age 49 years. Autopsy showed marked neuronal loss and gliosis in the substantia nigra and locus ceruleus. Lewy bodies, neurofibrillary tangles, senile plaques, and other abnormal structures were not seen in the cortical and subcortical regions. CONCLUSIONS: The Fukuoka 1 family shares many clinical and pathologic features with five previously reported kindreds from North America and Europe, suggesting that this syndrome has a worldwide distribution and can occur in different ethnic populations.

Parainfectious encephalomyeloradiculitis associated with herpes simplex virus 1 DNA in cerebrospinal fluid.

We describe a patient with acute encephalomyeloradiculitis associated with herpes simplex virus 1 (HSV-1) DNA in the cerebrospinal fluid (CSF), and we also review 4 similar cases previously reported from Japan. A 59-year-old man presented with acute encephalitis and urinary retention. Initially, coma and CSF pleocytosis improved with acyclovir treatment, but brain stem encephalitis, transverse myelitis, and lumbosacral polyradiculitis subsequently occurred. These conditions responded to corticosteroid therapy and immunoadsorption plasmapheresis. Polymerase chain reaction detected HSV-1 DNA in the CSF during acute encephalitis but not thereafter. Serial magnetic resonance imaging revealed transient lesions in the thalamus and basal ganglia on both sides of the brain and in the pons, spinal cord, and cauda equina. Acute encephalomyeloradiculitis is a unique neurological syndrome that may be caused by HSV-1 infection of the central nervous system.

[New protocol for memory-related potential].

A new P300 testing protocol for evaluating memory function is proposed. Four stimuli (S1, S2, S3 and S4) are presented to the subject at 1.5 sec intervals and the subject is instructed to judge whether S4 was the same (80%) or not (20%) as S1, as well as whether S3 was the same (80%) or not (20%) as S2. The S2-S3 comparison acts as an attention shifting mechanism, and by inserting this comparison between S1 and S4, the subject must memorized S1 at the memory site in the brain until S4 is presented. This protocol was performed using both auditory and visual stimulations with 24 college students (age range: 19-28 years old) and a patient (23 years old) with bilateral medial temporal lobe lesions caused by limbic encephalitis. The P300 brain potentials after S3 and S4 were compared. The Wechsler memory scale-revised (WMS-R) test was also assessed in all subjects. The latency of P300 after S4 had a significantly (p < 0.01) longer than that after S3. The latency of P300 after S4 in the patient, who showed the severe auditory as well as the mild visual memory impairment, was longer than the mean + 2 SD in the normal subjects for auditory stimuli, but not for visual stimuli, whereas the latency of P300 after S3 in the patient was longer than the mean + 2 SD in the normal subjects only for visual stimuli. The brain potential after S4 might be more reflective of a degree of memory disorder as compared with that after S3. Furthermore, the latency of P300 after S4 showed a more significant correlation with some WMS-R scores (verbal memory, general memory and verbal delay) than the latency of P300 after S3. These results suggest that measuring the P300 latency after attention shifting in the new protocol is useful for the evaluation of memory functions.

Genetic analysis enables definite and rapid diagnosis of cerebrotendinous xanthomatosis.

Mutations in the sterol 27-hydroxylase gene (CYP27) cause cerebrotendinous xanthomatosis (CTX). Early diagnosis of CTX is crucial because treatment with chenodeoxycholic acid can prevent or reverse some of the neurologic disability associated with the disease. We report the identification of three types of mutations (Arg441Trp, Arg372Gln, and Arg441Gln) in the CYP27 gene in five patients with suspected CTX from four unrelated families by restriction endonuclease analysis.

Survey of herpes simplex virus infections of the central nervous system, including acute disseminated encephalomyelitis, in the Kyushu and Okinawa regions of Japan.

We analysed data from 27 patients with herpes simplex virus (HSV) infections of the central nervous system (CNS) found in a 1990-1992 survey in Kyushu and Okinawa, Japan. Patients ranged in age from one year to 70 years, with peaks seen in the 20s and 50s. Temporal lobe-limbic encephalitis was the most common HSV infection (13 patients), followed by meningitis (5), diffuse encephalitis (4), disseminated encephalomyelitis (ADEM) (3) and brain stem encephalitis (2). Another three patients with non-herpetic, non-paraneoplastic acute limbic encephalitis were presented. Our study indicates that HSV infection can course ADEM, although temporal lobe-limbic encephalitis or meningitis are more common. The early diagnosis of HSV-related ADEM is important because of the efficacy of the timely administration of corticosteroids.

[A case of Prader-Willi syndrome with bilateral middle cerebral artery occlusion and moyamoya phenomenon].

We report a case of Prader-Willi syndrome (PWS) complicated with juvenile stroke. The patient is a 19-year-old man with right hemiplegia, who has had a history of non-insulin-dependent diabetes mellitus (NIDDM) for ten years. The diagnosis of PWS was confirmed genetically by the method of fluorescence in situ hybridization which showed the deletion of chromosome 15. His brain MRI revealed abnormal signal intensities in the left basal ganglia and around the right trigone of the lateral ventricle. Angiographic examination showed occlusions of bilateral proximal middle cerebral arteries with basal moyamoya vessels. The left vertebral artery was also occluded at its origin. Only a few cases of PWS complicated with stroke have been reported before and, to date, there has been no case with arterial occlusion similar to our case. Though the cause of these arterial occlusions is unknown, it may be related to arteriosclerosis following NIDDM.

[Significance of hepatitis B virus antibody in motor neuron disease].

We present here an assumption that hepatitis B virus (HBV) is involved in the pathogenesis of motor neuron disease (MND) in part, because of very high incidence of HBV antibody in patients with MND. In this study HBV antibodies were determined in 34 patients with MND (15 with ALS, 7 with SPMA, 8 with PBP and 4 with PBP+SPMA), and it was revealed that hepatitis B surface antigen (enzyme immunoassay: EIA) was negative in all the patients, whereas hepatitis B surface antibody (HBs-Ab) (EIA) was positive in 25 patients (73.5%) (9 with ALS, 6 with SPMA, 7 with PBP and 3 with PBP+SPMA). In contrast, HBs-Ab was positive only in 2 out of 28 patients with brain infarction (7.1%), and in 1 out of 16 patients with spinocerebellar degeneration (6.3%), both of which were used as control. Of 22 patients with indefinite complaints, none was positive for HBs-Ab. Thus, the number of HBs-Ab positive individuals was significantly (P < 0.001) greater in patients with MND than that in the patients having other disorders of central nervous system. From the above result, we conclude that MND may be partially ascribed to HBV.

[Non-herpetic acute limbic encephalitis].

We reported four cases of acute encephalitis, in which MRI abnormalities localized in the limbic system. In the four cases, fever and consciousness disturbance were commonly found and convulsive seizures appeared in three. Within 10 days, their consciousness level became clear, but severe amnestic syndrome remained as sequela. Cerebrospinal fluid (CSF) at the acute stage revealed mild lymphocytic pleocytosis and elevated protein. MRI revealed abnormal signal intensities in both hippocampi and amygdaloid bodies, although CT scans were normal. Herpes simplex encephalitis selectively affects one or both temporal lobes and the limbic system, But our cases spared the temporal lobes. Additionally, serum and CSF antibody titers by CF and ELISA for herpes simplex virus (HSV) were within normal range from the acute to convalescent stages. Neither HSV type 1 and 2 DNA genomes by sensitive PCR-hybridization method were detected in CSF taken from the acute stage of all four cases. On the other hand, paraneoplastic limbic encephalitis is known as a subacute encephalitis predominantly involving the limbic system. MRI shows high signal intensities on T2-weighted in both medial temporal lobes. In our cases, this type of limbic encephalitis was excluded because of the acute onset and lack of malignancy. Accordingly, our four cases are regarded as non-herpetic acute limbic encephalitis. Several Japanese similar cases have been reported. Further etiological studies should be performed.

[Binswanger's type encephalopathy without alopecia and lumbago in young hypotensive patients].

Binswanger's type encephalopathy is characterized by progressive dementia and diffuse subcortical ischemic lesions associated with arteriosclerosis. Hypertension is believed to be a major pathogenic factor in causing this encephalopathy but there are some cases of the encephalopathy not suffering from hypertension. In 1985, Yamamura et al. and Fukutake et al. reported familial cases of normotensive juvenile Binswanger's type encephalopathy with alopecia and lumbago, and considered it to be possibly a new clinical syndrome. We reported three cases of relatively young-onset (under the age of 40) Binswanger's type encephalopathy with persistent hypotension. All three patients suffered from neither alopecia nor lumbago. Patient (male aged 40) had repeated episodes of ischemic stroke and had progressive dementia. Patients 2 (male aged 41) and 3 (male aged 34) were not in a state of dementia, but had a history of transient ischemic attacks, and at present are completely symptom-free. Though there were no risk factors for cerebrovascular disease in these cases, the repeated episodes of ischemic stroke and the existence of small multiple lacunes in the basal ganglia on CT and MRI suggest that the white matter damage was principally due to a vascular disorder. In these cases, persistent hypotension was characteristic and might be a factor for the induction and exacerbation of this encephalopathy. These three cases are different from the classic form of Binswanger's type encephalopathy based on hypertension. Normotensive cases have been described before, but our cases do not seem to fall into this category because the blood pressure constantly remained hypotensive.(ABSTRACT TRUNCATED AT 250 WORDS)

Differentiation of herpes simplex virus 1 and 2 in cerebrospinal fluid of patients with HSV encephalitis and meningitis by stringent hybridization of PCR-amplified DNAs.

Differentiation of herpes simplex virus (HSV) types 1 and 2 in cerebrospinal fluid of 17 patients with serologically diagnosed HSV encephalitis and meningitis or acute limbic encephalitis was determined by stringent hybridization of polymerase chain reaction--amplified DNAs. Ten of 17 patients were positive; six with HSV 1 isolates and four with HSV 2 isolates. We detected HSV type 1 in two cases of meningitis, although meningitis is generally thought to be caused by type 2. Additionally, HSV type 2 was found in one case of acute adult encephalitis, which is generally due to HSV type 1. HSV DNAs could be detected for over 1 month after onset, although our patients included several prolonged and recurrent cases. HSV DNA genomes were not detected in three cases of acute limbic encephalitis. Our study indicates that this method can be used for type differentiation in HSV CNS infections.

Relapsing acute disseminated encephalomyelitis associated with chronic Epstein-Barr virus infection: MRI findings.

A 25-year-old women had a fever, left cervical lymphadenopathy, neurological symptoms and signs, CSF pleocytosis and persistent high serum antibodies to the Epstein-Barr virus (EBV); she had a recurrence 1 year later. She was thought to have relapsing acute disseminated encephalomyelitis associated with chronic EBV infection. MRI revealed abnormalities, mainly in the right basal ganglia and left midbrain. At the time of the recurrence, further abnormalities appeared in the opposite basal ganglia and right cerebral white matter.

Changes in distribution of alpha-tocopherol and cholesterol in serum lipoproteins and tissues of rats by dietary PCB and dietary level of protein.

An experiment was conducted with growing rats to examine the influence of dietary addition of 0.1% polychlorinated biphenyls (PCB) and dietary level of protein on the distributions of alpha-tocopherol and cholesterol in serum lipoproteins and tissues. Addition of PCB increased serum alpha-tocopherol. This was mainly due to the increment in the fractions of chylomicrons/very-low-density lipoproteins (VLDL) and high-density lipoproteins (HDL). This chemical also increased serum cholesterol and HDL cholesterol. The contents of alpha-tocopherol and cholesterol in HDL fraction were higher in the high protein groups as compared to the low protein groups, regardless of PCB intake. PCB intake increased the concentrations of alpha-tocopherol in tissues including spleen, lung, kidney, testis, muscle, adipose tissue, and brain. High protein diet increased the levels of alpha-tocopherol in these tissues as compared to low protein diet. PCB intake also increased adrenal alpha-tocopherol, which was potentiated with low protein diet. The increment in liver alpha-tocopherol by PCB intake was observed only in the animals fed on low protein diet.

Computed tomography for the assessment of blunt abdominal trauma.

Computed tomography (CT) was carried out on 70 patients with blunt abdominal trauma. Damage to the abdominal organs was clearly demonstrated by CT. The positive rates of CT in traumatic lesions were higher than those of ultrasound (US). Intraperitoneal hemorrhage was well demonstrated by US. Serum GPT levels were significantly elevated in patients with liver damage (p less than 0.001). CT proved to be useful for detecting damage to organs in patients with abdominal trauma.

Effects of dietary xenobiotics on the metabolism of copper, alpha-tocopherol and cholesterol in rats.

The effects of dietary addition of some xenobiotics on tissue levels of copper and alpha-tocopherol in rats were studied with special reference to serum cholesterol. The chemicals added here were 0.05% polychlorinated biphenyls (PCB), 0.3% caffeine, 0.05% 1,1,1-trichloro-2,2-bis(p-chlorophenyl)ethane (DDT), 0.3% flavone, 0.3% chloretone, 0.3% 2-tert-butyl-4-methoxyphenol (BHA), 0.15% sodium phenobarbital, 0.15% phenytoin, 0.2% sodium pentobarbital, and 0.15% phenylbutazone. Of these chemicals, dietary PCB, caffeine, DDT, flavone, chloretone, BHA and phenytoin raised the level of copper in liver, kidney, or serum. Serum alpha-tocopherol and cholesterol also increased with the additive or synergistic effects on these metabolisms. Moreover, these combined addition of dietary 0.02% PCB with 0.2% BHA seemed to cause additive or synergistic effects on these metabolism. Moreover, these chemicals raised serum ceruloplasmin activity. In general, serum copper, ceruloplasmin activity and alpha-tocopherol were correlated with serum cholesterol in these dietary manipulations.