Initial and follow-up brain MRI findings and correlation with the clinical course in Wilson's disease

We performed pretreatment brain MRIs in 25 patients with neurologically symptomatic Wilson's disease (WD) and clinical and MRI follow-up in 16 of them. All 25 pretreatment MRIs revealed abnormalities, with abnormal high-signal intensity (HSI) in bilateral thalami being the most common (92%). HSI lesions in the brainstem (84%) and the basal ganglia (72%) were also common. Brain atrophy was present in 88% of the 25 patients. In the follow-up period of 5 to 24 months, during which the patients were treated with D-penicillamine, both HSI lesions and neurologic symptoms improved in 88% of the 16 patients, but the brain atrophy did not change.     

Dysosteosclerosis: clinicoradiologic findings including brain MRI

A S. mansoni adult worm cDNA expression library was screened with sera from baboons in a early phase after infection. The clones that were positive with the early infection sera were examined for reactivity with pre-infection sera and heterologous infection sera. In order to discriminate a positive antibody reaction from the reactivity due to residual anti-E. coli antibodies, an unrelated cDNA clone was plated with the positive clone. The unrelated clone provided the negative background and the contrast necessary to discern a positive antibody reaction. In this way, we were able to eliminate selected clones that were positive with the pre-infection sera or heterologous infection sera. This characterization of the expression library clones enabled us to quickly target only clones with the desired pattern of antibody reactivity for sequencing, subcloning, and expressing.     

A study of MRI and clinical neurology in acute cerebellar infarcts

We studied clinical manifestations of sixteen patients with cerebellar infarcts diagnosed by MRI. In fourteen of them, the stroke developed abruptly with vertigo, which continued for several days. At the early stage of illness, ataxia was obscure. But after vertigo and nausea disappeared, nine cases showed truncal ataxia, while limb ataxia was found in only five. Their vertigo was rotatory and aggravated by head movement. Gaze-evoked nystagmus was observed in only 5 cases. Four patients preferred to take unilateral posture since they experienced less vertigo. The side of their lesions was the lower side of their posture. Limb ataxia was more frequent in SCA-involving cases than in SCA-non involving cases (3 out of 6 vs 2 out of 10, respectively). On the other hand, headache was more frequent in PICA-involving cases than in PICA-non-involving cases (6 out of 11 vs 1 out of 5, respectively). Ataxic gait was seen more in medial branch-involving cases than medial branch non-involving cases (5 out of 6 vs 4 out of 10, respectively). One patient died due to obstructive hydrocephalus.     

A clinical, MRI and neurophysiological study of acute transverse myelitis

There is paucity of studies correlating the MRI and evoked potential changes in acute transverse myelitis (ATM). We studied ten patients with ATM (age range 14-57 years; 8 men, 2 women) who were subjected to clinical, MRI and neurophysiological evaluation. The latter included median and tibial somatosensory evoked potentials (SEP), motor evoked potentials (MEP) to upper and lower limbs and concentric needle EMG. The outcome was defined on the basis of three month Barthel Index score. All the patients had pronounced lower limb and three had upper limb weakness. Magnetic resonance imaging scans revealed diffuse to hypointense lesions in T1, which became hyperintense in T2 in all except one patient, who had patchy hyperintense lesions in both T1 and T2 sequences suggesting haemorrhage. The signal changes extended at least three segments above the sensory level. Tibial SEP and central motor conduction time to tibialis anterior (CMCT-TA) were abnormal in nine patients each. Median SEP was normal in all, but CMCT to abductor digiti minimi (CMCT-ADM) was abnormal in four patients. The extent of MRI signal alterations and CMCT-TA correlated with the outcome. Seven patients had a poor outcome, in them MRI changes extended 10 spinal segments or more. In these patients, MEP on lumbar stimulation was either unrecordable or of low amplitude and extensive fibrillations were present in the lower limb muscles. From this study, we conclude that in ATM, extensive MRI changes, unrecordable MEP to lower limbs especially on lumbar stimulation and evidence of denervation in leg muscles seem to predict a poor outcome.     

Müllerianosis of the urinary bladder: clinical and immunohistochemical findings

BACKGROUND: Intranasal desmopressin has been used extensively to treat primary nocturnal enuresis. While it has proven to be a safe, effective agent for many who are affected by this condition, the potential for complications exists. OBJECTIVES: To report a case of severe hyponatremia associated with a generalized tonic-clonic seizure in a 10-year-old boy who had been receiving intranasal desmopressin nightly for nocturnal enuresis and to briefly review therapeutic options for nocturnal enuresis; and to present the role of desmopressin. SETTING: Georgetown University Medical Center, Washington, DC. INTERVENTION: Fluid restriction and intravenous isotonic saline solution with 5% dextrose was administered to raise the serum sodium level. OUTCOME: Prevention of further seizures with normalization of serum sodium levels without any obvious neurological sequelae. CONCLUSIONS: This case illustrates the importance of weighing the benefits and risks of intranasal desmopressin therapy.     

MRI evaluation of the brain in infantile neuronal ceroid-lipofuscinosis. Part 2: MRI findings in 21 patients

The purpose of this study was to demonstrate the course of infantile neuronal ceroid-lipofuscinosis with brain magnetic resonance imaging (MRI) in children aged 3 months to 11 years. Twenty-one patients and 46 neurologically normal controls of the same age were examined. The images were evaluated visually; then signal intensities were measured and related to those of references. MRI abnormalities were detectable before clinical symptoms. The radiologic picture of the brain varied with the duration of the disease. Pathognomonic MRI findings in the early stage of the disease were generalized cerebral atrophy, strong thalamic hypointensity to the white matter and to the basal ganglia, and thin periventricular high-signal rims from 13 months onward on T2-weighted images. In patients over 4 years old, cerebral atrophy was extreme, and the signal intensity of the entire white matter was higher than that of the gray matter, which is the reverse of normal. This study showed that the abnormalities seen on MRI progress rapidly during the first 4 years of life, then stabilize, in conformity with the clinical and histopathologic pictures of infantile neuronal ceroid-lipofuscinosis.     

MRI findings in headbangers

Magnetic resonance imaging (MRI) findings in a mentally retarded adult female who exhibits headbanging behavior are presented. Radiographic changes include enlargement of the diploic space in the parietal and occipital bones, and gray matter loss adjacent to the bony changes. This pattern of injury is compared with skull changes previously reported in headbangers, and neuronal injury seen in boxers (dementia pugilistica) and Minimata disease.     

A case of diffuse fasciitis and its MRI findings

A 36-year-old woman with diffuse fasciitis and her MRI findings are reported. The patient developed pain and swelling in the calves, right forearm, and left arm, predominantly in the right calf muscle. Her body temperature was 38.4 degrees C, the ESR 104 mm/2 hours and white blood cell count 8,000/microliter without eosinophilia. The fascia and muscle were biopsied from her right calf. The light microscopy showed that the fascia was thickened and infiltrated with mononuclear cells with no eosinophils, mostly in perivascular areas. The muscle fibers were spared. MRI of legs revealed marked hyperintensities on T2 and mild hyperintensities on T1-weighted images in the fascia, superficial flexor muscles, especially soleus muscle, and Achilles tendons. Moderate dose of prednisolone was very effective and the abnormal signals on MRI almost disappeared within a month. T2 weighted MRI was very useful to detect the lesions and to evaluate the course of diffuse fasciitis.     

MRI findings in Wernicke encephalopathy

During the past 7 years from January 1991 to November 1997, we experienced 31 cases of aortic root reconstruction utilizing Carrel patch method. Concomitant procedure were performed in 9 cases of them due to another cardiovascular disease. Complicated cardiovascular disease included 3 cases of ischemic heart disease, 3 cases mitral regurgitation and one case of Aortic arch aneurysm. Several concomitant procedures were performed; 5 cases of CABG, 2 cases of mitral annuloplasty, one case of CABG with mitral valve replacement and one case of aortic arch replacement. The mean extra corporeal circulation time was 190.6 +/- 39.3 minutes and aortic clamp time was 147.8 +/- 34.2 minutes in these 9 cases. There were no significant differences of operative results between the simple root reconstruction group and the concomitant procedure group. We concluded that the aortic root reconstruction using the Carrel patch method has few complications. Although further long-term follow-up is required, our experiences suggest that the aortic root reconstruction with the concomitant procedure can be carry out safely with the aid of appropriate assistance method.     

Theory and clinical application of functional MRI: 3D-functional brain mapping

Functional magnetic resonance imaging (fMRI) was performed using a clinical 1.5 T MR scanner. Normal volunteers and patients with several neurological disorders were studied with somatosensory stimulation using sponge at right hand and visual stimulation using checkerboard pattern. Both fMR images by gradient echo echo planar imaging and three dimensional gradient echo images were studied. Reconstructed 3 dimensional functional brain mapping was superimposed on 3D anatomical images. Apparent signal increase was observed at contra lateral sensorimotor cortex and secondary sensory cortex with sponge stimulation. In the case of left homonymous hemianopia due to cerebral infarction, increasing signal was only observed surrounding left calcarine fissure by using stimulation of all visual field. In conclusion, fMRI and 3-D functional brain mapping has extremely high potentiality to examine pathophysiology of various neurological disorders.     

A proton magnetic resonance spectroscopic study in ALS: correlation with clinical findings

OBJECTIVE: To evaluate neuronal dysfunction in the motor region subcortical white matter in ALS using volumetric localized proton magnetic resonance spectroscopy (1H-MRS). METHODS: Sixteen patients with E1 Escorial definite, probable, or possible ALS and eight healthy age-matched control subjects were studied. The ALS patients were divided into those with limb onset (n = 8) and those with bulbar onset (n = 8). Measurements of the metabolic ratios N-acetylaspartate (NAA)/creatine and phosphocreatine (Cr+PCr), NAA/choline (Cho), and Cho/(Cr+PCr) were correlated with clinical assessments. RESULTS: We found no differences in the metabolic peak area ratios in the motor region when comparing the total ALS group and the control subjects. However, correlations were found between the NAA/(Cr+PCr) ratio and the E1 Escorial category (p = 0.03), the ALS severity scale (p = 0.01), and the Medical Research Council score (p = 0.06). No correlations were found between the NAA/(Cr+PCr) ratio and the Ashworth Spasticity Scale, reflex score, or disease duration (p > 0.16). Bulbar-onset patients had a lower NAA/(Cr+PCr) ratio in the motor region compared with limb-onset patients (p = 0.03). CONCLUSION: In vivo 1H-MRS of the subcortical white matter in the motor region is unlikely to be sensitive enough to detect early disease changes in ALS because there is considerable overlap between the metabolic peak area ratios from patients with ALS and normal control subjects. However, changes in the NAA/(Cr+PCr) metabolic peak area ratios correlate with clinical measures of disease severity, and this measure may be useful in monitoring disease progression.     

Spontaneous bacterial peritonitis: clinical study, microbiological findings and clinical course

One hundred and forty-four episodes of spontaneous bacterial peritonitis (SBP) treated in our service between July 1988 and September 1995 were studied retrospectively to assess the clinical presentation, microbiological findings, possible pathogens, treatment and course. Ascites, abdominal pain and fever were the most common symptoms. Only 3.5% of cases were asymptomatic. The outcome was fatal in 12 (8.33%). Among the factors analyzed, only a prothrombin time of less than 35% correlated significantly with a higher mortality rate (60% and 8.33%, respectively; p < 0.01). Ascitic fluid culture was positive in 43.05% of cases; significant differences existed between these patients and those with negative ascitic fluid culture with respect to clinical findings or course. Gram-negative microorganisms were those most frequently isolated (48.38%). Treatment was initiated within 12 hours in 77.7% of the patients, between 12 and 72 hours in 11.8% and later in 10.41%. Intravenous cefotaxime was administered in 86.1% of cases and other drugs or drug combinations in only 13.9%; the mortality rate was much lower with cefotaxime (2.4% vs 45%; p < 0.01).     

MRI findings in cerebral fat embolism

The purpose of this retrospective study was to demonstrate the MRI features of cerebral manifestations in patients with fat embolism syndromes in comparison with cerebral CT (CCT). Magnetic resonance imaging was performed according to standard protocols revealing multiple small non-confluent hyperintense intracerebral lesions larger than 2 mm on proton-density and T2-weighted images to various extents in three of four patients with clinically suspected cerebral fat embolism. Cerebral CT was negative in all patients. Our findings confirm that MRI can detect cerebral fat embolism with a higher sensitivity than CCT. Thus, MRI should be the first choice for imaging of cerebral fat embolism.     

Early MRI findings in Creutzfeldt-Jakob disease

We describe the MRI changes preceding the onset of myoclonus in two patients whose post-mortem examination confirmed the diagnosis of Creutzfeldt-Jakob disease (CJD). MRI showed changes in the striatum early in the course of CJD (2-6 months after the onset of apathy, interpreted as depression, and 1-2 months before the onset of further clinical symptoms). Only in one patient did electroencephalography record the typical triphasic sharp-waves, 1 month after MRI.     

Motilin induces gall bladder emptying and antral contractions in the fasted state in humans

BACKGROUND: Animal studies have shown that motilin affects gall bladder motility. In humans, no effect has been shown, but erythromycin, a motilin receptor agonist, induces gall bladder emptying. AIMS: To explore the effect of increasing doses of exogenous motilin on gall bladder volume and antral contractility in the fasted state in humans. METHODS: After an overnight fast, eight healthy men received increasing intravenous doses of Leu13-motilin (KW-5139) or 0.9% NaCl in a double blind, randomised fashion. Gall bladder volume and antral contraction frequency were determined by ultrasonography. RESULTS: Infusion of motilin increased plasma motilin levels. Motilin induced a reduction in gall bladder volume of 8.0 (5.0)%, 17.1 (5.0)%, 18.5 (4.7)%, and 16.1 (4.9)% of baseline volume at the end of infusion of 2, 4, 8, and 16 pmol/kg/min respectively, compared with mean stable gall bladder volumes during placebo infusion (p < 0.05). Antral contraction frequency increased during motilin infusion, but not during placebo infusion (p < 0.05). CONCLUSIONS: Exogenous motilin reducted fasting gall bladder volume and increased antral contractions. After reaching maximal reduction, the gall bladder volume did not decrease further during continuous motilin infusion at higher doses and stayed at the same reduced volume. The degree of gall bladder volume reduction during motilin infusion mimicked gall bladder emptying preceding antral phase III activity of the migrating motor complex in humans. This study indicates that motilin may play a physiological role in the regulation of gall bladder emptying in the fasted state.     

Neurotological auditory brain stem response findings in human immunodeficiency virus-positive patients without neurologic manifestations

Neurologic manifestations of human immunodeficiency virus (HIV) infection, rather than being a late complication of the disease, are principally correlated with the early central nervous system (CNS) localization of HIV. The CNS may be infected in the early stages of acquired immunodeficiency syndrome (AIDS) without evidence of neurologic disorders. Evoked potentials (visual, auditory, and somatosensory) and electronystagmographic test batteries have proven to be very sensitive in showing subclinical CNS disorders due to HIV. In this study, auditory brain stem response (ABR) and electronystagmographic test battery findings (smooth pursuit, saccades, caloric test) were performed in 29 neurologically asymptomatic, HIV-positive subjects at different stages of the disease. Compared to results of a control group, the ABR latencies of waves V, I, and III and interpeaks I-V and III-V were significantly increased in HIV patients. The same parameters did not differ significantly among the stages of the disease. In HIV-positive subjects, the accuracy of saccades was significantly reduced, while latency was normal. The velocity and the gain of pursuit were significantly reduced in HIV-positive patients, and 15 of 29 patients showed corrective saccades. Caloric tests revealed qualitative nystagmus abnormalities in 82% of HIV patients, while quantitative parameters were normal. The present results confirm that CNS involvement by HIV occurs early in the course of the disease. In particular, HIV does not seem to affect the labyrinth or the eighth cranial nerve, as demonstrated by the normality of the I-III value of the ABR and of the quantitative parameters of the caloric responses, but it does appear to involve the brain stem acoustic pathways, pontocerebellar pathways, and supratentorial areas.     

MRI findings in simultaneous bilateral meniscal lesions

This study investigates concurrent lesions of the knee that may be associated with bilateral simultaneous meniscal lesions. The MRI images of the knees of 22 patients with this condition were examined in detail. The results suggest a degenerative cause.     

Serial MRI findings of benign poliomyelitis

Clinical and electrodiagnostic evaluations were performed in 25 patients with electric burns who were injured 2 to 13 years before the study. Abnormal motor nerve conduct findings were detected in 12 of the patients. In nine patients, abnormalities were observed in more than one nerve. There was a positive relation noted between age and nerve lesions. Weakness was the most prominent clinical finding.