Successful emergency treatment with a transjugular intrahepatic portosystemic shunt for life-threatening Budd-Chiari syndrome with portal thrombotic obstruction.

We report successful treatment of acute severe Budd-Chiari syndrome with portal venous thrombosis. The prognosis of patients with this condition is poor, because the therapeutic options are limited. A 38-year-old woman with polycythemia vera was admitted in a critical condition, and Budd-Chiari syndrome complicated by portal venous thrombosis was diagnosed. Tissue plasminogen activator and urokinase were infused systemically and were partially effective. Transjugular intrahepatic portosystemic shunting to reduce the high portal venous pressure was performed successfully and, eventually, her general condition improved. Our experience indicates that emergency transjugular intrahepatic portosystemic shunting is an effective therapeutic modality for controlling portal hypertension in patients with severe Budd-Chiari syndrome with portal venous thrombosis.

A clinicopathological study of a patient with familial amyotrophic lateral sclerosis associated with a two base pair deletion in the copper/zinc superoxide dismutase (SOD1) gene.

The recognition of mutations in the copper/zinc superoxide dismutase (SOD1) gene in familial amyotrophic lateral sclerosis (FALS) has been a landmark in ALS research. We report a clinicopathological study of a female patient with FALS showing a two base pair deletion in exon 5 of the SOD1 gene. Her clinical course was rapid and she died 2 years after the onset. The SOD1 activity was down to 30% of the normal level. Western blot analysis did not reveal the mutant protein which was expected to be approximately 2.4 kDa smaller than normal SOD1 protein in molecular mass. In contrast to the neuropathological findings of the previously reported cases showing the same mutation, our case was characterized by sparing of the dorsal column and the presence of only a modest number of intracytoplasmic eosinophilic inclusions showing weak or partial immunoreaction for neurofilament and negative reaction for SOD1. Thus, the same mutation in the SOD1 gene does not necessarily induce consistent pathological changes in the central nervous system.

MR imaging of Wallerian degeneration in the brainstem: temporal relationships.

Degeneration of the myelin sheath and axon distal to the most proximal site of axonal interruption secondary to axonal disease has been called wallerian degeneration. On MR imaging, wallerian degeneration of the pyramidal tract can be observed as an abnormal signal intensity, showing prolonged T1 and T2 relaxation times that correspond to the corticospinal tract, with or without shrinkage of the ipsilateral cerebral peduncle and pons. Review of MR studies in 150 cases of supratentorial cerebrovascular accidents showed abnormal signal alterations in the ipsilateral brainstem in 33 of the cases. Abnormal intensity in the ipsilateral brainstem was seen as early as 5 weeks after the supratentorial ictus and was fully evident after 10 weeks in all 33 cases. Signal alterations were strongest at about 3-6 months when compared with alterations seen at 10 weeks or even 10 months after the ictus. Shrinkage of the ipsilateral brainstem appeared as early as 8 months and was demonstrated in all cases 13 months after the ictus. MR seems to be the most effective technique for early detection of wallerian degeneration and may provide insight into its pathophysiological and chemical changes.

High ratio of HTLV-1-infected cells in HTLV-1 associated myelopathy (HAM).

Sixteen patients with HTLV-1 associated myelopathy (HAM) were examined for the presence of HTLV-1 provirus genome by Southern blot analysis of genomic DNA from peripheral blood mononuclear (PBM) cells. Random integration of the provirus was detected in 14 of 16 HAM patients. By contrast, the provirus genome could not be detected in 6 non-HAM HTLV-1 carriers, HAM patients were found to have significantly higher antibody titer to HTLV-1 in the sera compared with carriers. These features of HAM patients, i.e., detectable levels of provirus integration in PBM cells and high antibody titer to HTLV-1 in the sera, were noted in 2 wives of HAM patients with neurological signs and abnormalities. High anti-HTLV-1 antibody titer and detection of the provirus genome by Southern hybridizations may be useful for screening subclinical HAM cases and elucidating pathogenesis.

Single photon emission computed tomography (SPECT) in a patient with Wilson's disease.

Single photon emission computed tomography (SPECT) with 133Xe inhalation was studied in a patient with Wilson's disease, who had the low-density lesions in the bilateral ganglia and a small calcified lesion in the left basal ganglia on computed tomography (CT). SPECT showed the regional cerebral blood flow (rCBF) of the basal ganglia area. The values of the rCBF in these areas are 77 to 91 ml/100g/min in the right, and 56 to 77 ml/100g/min in the left. Further study is needed to prove the pathological role of rCBF for the basal ganglia lesion in the patient with Wilson's disease.

Regional cerebral blood flow assessed by 133Xe inhalation and emission tomography: normal values.

Regional cerebral blood flow (rCBF) in the cerebral hemispheres and the cerebellum was measured by single photon emission computed tomography with inhalation of 133Xe in 39 normal volunteers at test. The goal of this study was to assess the normal flow pattern and its variations. Five parallel tomographic slices through the brain were recorded with a resolution element of 1.7 X 1.7 X 2.0 cm (full width at half maximum). The blood flow distribution showed that the predominantly gray matter areas displayed flow approximately double that of the predominantly white matter regions. The CBF distribution was practically symmetrical with a side-to-side difference averaging 1.4 +/- 1.4 ml/100 g/min. This means that a difference exceeding 4.2 ml/100 g/min (approximately 9% of mean CBF) is abnormal with a confidence level of below 5%. The measured average CBF and cerebellar blood flow were 56 +/- 7 and 54 +/- 6 ml/100 g/min (mean +/- 1 SD), respectively. A significant correlation was found between CBF and PCO2, and between CBF and age. Repeat measurements in an additional 30 subjects showed a day-to-day variability of -0.2 +/- 6.4 ml/100 g/min of the difference between the first and the second measurement. This corresponds to random methodological and biological errors of 6.4/square root 2 = 4.6 ml/100 g/min and is a measure of the overall intraindividual variability. Xenon-133 tomography is atraumatic and affords rCBF images free of the superposition artifacts that practically invalidate the nontomographic approaches in the studies of cerebrovascular disease. The rCBF tomograms are blurred, particularly due to Compton scatter. Relative to this factor, the errors caused by local variations in the tissue:blood partition coefficient are less important.

[A case of spontaneous spinal epidural hematoma presenting Brown-Séquard syndrome].

Although there have been a few reports of spontaneous spinal epidural hematoma, it may be difficult to make a correct diagnosis of the lesion because of atypical clinical symptoms. The authors reported a case of spontaneous spinal epidural hematoma presenting Brown-Séquard syndrome, with a review of literature. This 75-year-old male experienced a sudden onset of severe dorsal neck pain during sleep associated with right hemiparesis which deteriorated quickly to hemiplegia, without loss of consciousness, nausea, vomiting and vertigo. The patient has a history of hypertension and has been treated for chronic hepatitis for one year. On admission, neurological examination revealed right hemiplegia with normotensive deep tendon reflexes, and loss of pain and temperature sensations below the level of C5 on his left side. Position sense of fingers and toes was diminished on his right side, and hyperesthesia was recognized at the area of C4 level. Consciousness disturbance, cranial nerve signs, and urinary incontinence were not seen. Right retrograde vertebral angiograms showed no abnormal vascular shadow, even though anterior spinal artery was visible at the level of C8 through C5. Cervical CT scan revealed a left-side dominant extradural high density mass which situated dorsally at around the upper part of C2 body changing it's position to dorsolaterally as it descended to the lower part of C5. On contrast enhancement study, only the margin of the lesion was partially enhanced, which might be the hematoma membrane. On the third day after the onset, laminectomy from C2 to C5 was performed and the epidural hematoma was evacuated.(ABSTRACT TRUNCATED AT 250 WORDS)