The natural history of neurological manganism over 18 years.

We investigated the clinical features and progression of four patients with chronic manganese intoxication, 18 years after cessation of exposure. Because the results were to be compared with previous observations, we employed the same scoring system. The clinical manifestations were foot dystonia, wide based gait, rigidity, and difficulty in walking backwards. Resting tremor was rarely seen, but tongue tremor was found in 2 patients. The asymmetry initially present in 2 patients persisted 18 years later. Measurements had previously revealed rapid progression in the initial 10 years. We found a plateau over the following decade.

Functional MR imaging of the human sensorimotor cortex after toe-to-finger transplantation.

BACKGROUND: A model of toe-to-finger transplantation has been used in studying peripheral nerve regeneration and central reorganization. It was found that recovery of sensory perception depends not only on peripheral reinnervation but also on central integrative mechanisms. OBJECTIVE: Our aim was to investigate functional changes of the brain and somatotopic representation of the transplanted toes after toe-to-finger transplantation. MATERIALS AND METHODS: Six patients who had toe-to-finger transplantation from 3 to 8 years earlier underwent motor and sensory functional MR imaging studies of transplanted toes and opposite corresponding normal fingers. The motor task was performed by repetitively tapping of the transplanted toe or finger against the thumb, whereas the sensory task was applied by tactilely stimulating the pulp of the transplanted toe or finger. RESULTS: The main activation areas from both types of stimulations were located in the expected location of the finger homunculus of the primary sensorimotor cortex. In addition, activated volumes from the transplanted toes were significantly greater than those from the opposite fingers (P = .017 for motor task and P = .005 for tactile sensory task, paired samples Student t test). CONCLUSIONS: Functional recruitment in the primary sensorimotor cortex seemed to have occurred following toe-to-finger transplantation. The transplanted toe was somatotopically represented in the hand area.

Acute painful neuropathy in thallium poisoning.

Dysesthesia, allodynia, distal muscle weakness, and sensory impairment were noted in two patients with acute thallium intoxication. Two months later, nerve conduction studies showed an axonal degeneration. Sural nerve biopsy disclosed a decreased fiber density in the large myelinated fibers. Quantitative sensory testing also revealed an impairment of pinprick, temperature, and touch sensations. Cutaneous nerve biopsy confirmed a loss of epidermal nerves indicating an involvement of the small sensory nerves.

Late CT manifestations in spontaneous lobar hematoma.

PURPOSE: The purpose of this work was to determine the late CT manifestations of spontaneous lobar hematoma. METHOD: Serial CT studies were performed in 75 cases. The initial study was done during the acute stage of stroke, and the subsequent one was performed at least 6 months later. RESULTS: There were 20 cases (27%) with no trace of previous hemorrhage, 14 (19%) with a slit cavity, 11 (15%) with a small round cavity, 20 (27%) with pseudoinfarct, 8 (11%) with ventricular deformity but no residual cavity, and 2 (3%) with only calcification at the site of the hemorrhage. CONCLUSION: Our data show that the typical residual slit was relatively uncommon in lobar hematomas and that, depending on their size, hematomas would disappear, reduce to a small cavity, or form a pseudoinfarct.

Effects of Betel chewing on the central and autonomic nervous systems.

Betel chewing has been claimed to produce a sense of well-being, euphoria, heightened alertness, sweating, salivation, a hot sensation in the body and increased capacity to work. Betel chewing also leads to habituation, addiction and withdrawal. However, the mechanisms underlying these effects remain poorly understood. Arecoline, the major alkaloid of Areca nut, has been extensively studied, and several effects of betel chewing are thought to be related to the actions of this parasympathomimetic constituent. However, betel chewing may produce complex reactions and interactions. In the presence of lime, arecoline and guvacoline in Areca nut are hydrolyzed into arecaidine and guvacine, respectively, which are strong inhibitors of GABA uptake. Piper betle flower or leaf contains aromatic phenolic compounds which have been found to stimulate the release of catecholamines in vitro. Thus, betel chewing may affect parasympathetic, GABAnergic and sympathetic functions. Betel chewing produces an increase in heart rate, blood pressure, sweating and body temperature. In addition, EEG shows widespread cortical desynchronization indicating a state of arousal. In autonomic function tests, both the sympathetic skin response and RR interval variation are affected. Betel chewing also increases plasma concentrations of norepinephrine and epinephrine. These results suggest that betel chewing mainly affects the central and autonomic nervous systems. Future studies should investigate both the acute and chronic effects of betel chewing. Such studies may further elucidate the psychoactive mechanisms responsible for the undiminished popularity of betel chewing since antiquity.

Carbon disulfide vasculopathy: a small vessel disease.

We present the clinical manifestations of 4 male patients with acute stroke-like symptoms and polyneuropathy after long-term exposure to carbon disulfide (CS2) in a viscose rayon plant. The ages of onset of polyneuropathy ranged from 42 to 45 years with a duration of CS2 exposure between 6 and 21 years. The ages of onset of stroke were from 42 to 48 years. The risk factors for stroke including heart disease and diabetes were denied, except for smoking in 4, hyperlipidemia in 2 and hypertension in 1. At the initial visit in 1992, only 2 patients developed sudden onset of hemiparesis suggesting a lacunar stroke before the diagnosis of CS2 intoxication. Brain computed tomography (CT) scans showed low-density lesions in the basal ganglia in 2 patients, cortical atrophy in 1 and normal in 1. Brain magnetic resonance image (MRI) study disclosed multiple lesions in the corona radiata and basal ganglia on T(2)-weighted images in 3 patients and cortical atrophy in 1. After the diagnosis, they left their jobs for a CS2-free environment, and improvement of the working conditions was noted. During 5 years follow-up period, another 2 patients also developed an acute episode of stroke with hemiparesis. Brain CT and/or MRI follow-up studies in these 2 patients revealed new lesions in the basal ganglia and corona radiata. Intriguingly, a patient with previous stroke also developed new lesions in the bilateral thalami and brainstem. Carotid Doppler scan, transcranial Doppler scan and/or cerebral angiography did not show any prominent stenosis or occlusion in the major intracranial large arteries. We conclude that encephalopathy may occur in patients after long-term CS2 exposure, probably due to impaired cerebral perfusion. The lesions tend to occur in the basal ganglia, corona radiata and even brainstem, particularly involving the small-sized vessels. In addition, the cerebral lesions may progress even after cessation of CS2 exposure. Therefore, we suggest that CS2 exposure may be a risk factor for stroke.

Lateralization and prognostic value of proton magnetic resonance spectroscopy in patients with intractable temporal lobe epilepsy.

BACKGROUND: The aims of this prospective study were to investigate the stability of hippocampal metabolite ratios obtained by proton magnetic resonance spectroscopy (MRS), to evaluate the ability of MRS to determine the pre-surgical lateralization of seizure focus, and to assess the relationship between MRS results and postoperative outcomes in patients with intractable temporal lobe epilepsy. METHODS: Within- and between-acquisition variations were evaluated in 30 control subjects, using the chemical-shift imaging technique. The most stable metabolite ratio was then applied for pre-surgical evaluation of 14 patients with intractable temporal lobe epilepsy. RESULTS: The ratio between N-acetylaspartate (NAA) and choline-containing compounds (Cho) plus creatine-phosphocreatine (Cr), i.e., NAA/(Cho+Cr), had an overall smaller percentage change between measurements (13%-28%) than did the other ratios: NAA/Cho (18%-37%), NAA/Cr (11%-60%), and Cho/Cr (19%-51%). With a mean follow-up period of 27 months (range, 12-55 months) after an anterior temporal lobectomy, 10 patients were in Engel s class I (71%), 2 were in class II (14%), and 2 were in class III (14%). Lateralization by MRS was consistent with the operation side in 11 (79%) patients, including class I in 8 patients, class II in 1 patient, and class III in 2 patients. Ranges of reduced NAA/(Cho+Cr) values were similar between patients with different post-surgical outcomes: 0.26-0.47 for class I, 0.40-0.45 for class II, and 0.34-0.40 for class III (p=0.651). CONCLUSIONS: Proton MRS may be helpful in pre-surgical lateralization of the seizure focus in patients with intractable temporal lobe epilepsy; however, it cannot provide prognostic information about postoperative seizure control.

Correlation of neurological manifestations and MR images in a patient with Wilson's disease after liver transplantation.

Orthotopic liver transplantation (OLT) has been applied to patients with Wilson's disease (WD) for correction of irreversible liver cirrhosis. However, the neurological outcome and the correlation between clinical manifestations and neuroimage findings after OLT remain uncertain. We present a WD patient who showed an improvement in both liver functions and neurological manifestations after OLT. Serum levels of ceruloplasmin and copper returned to normal rapidly after the operation. His ataxic gait was improved 5 months later and dysmetria and tremor disappeared 11 months later. The high signal intensities on T2-weighted brain magnetic resonance images regressed at bilateral thalami 5 months later and disappeared in bilateral thalami and red nuclei 16 months after OLT. We conclude that the neurological improvement could be expected in WD patients after OLT. The improvement was correlated with the MRI changes in red nuclei and bilateral thalami.

The microbial flora of the gastrointestinal tract and the cleaning of flexible endoscopes.

Technologic advances in the last 30 years have resulted in the development of complex, expensive, and heat sensitive medical instrumentation, including flexible gastrointestinal endoscopes. Because of the design complexity and the region of use, gastrointestinal endoscopes present special challenges to cleaning. If instruments are not properly cleaned the disinfection or sterilization procedure can fail and increase the possibility of infection transmission from one patient to another. Although the cleaning process removes intestinal microflora, the washing process itself may introduce a saprophytic or environmental microbial flora. It has been repeatedly shown that endoscope cleaning, not the disinfection or sterilization procedure, controls the success of the reprocessing procedure.

Phantom finger phenomena and the effects of toe-to-finger transplantation.

Phantom finger phenomena and the effects of toe-to-finger transplantation were studied in 76 patients who had had traumatic finger amputation. Phantom finger phenomena were observed in 48 (63%) patients with the presence of phantom finger only in 30, phantom finger with sensation in nine, and phantom finger with motion also in nine. After toe transplantation, phantom finger phenomena disappeared immediately in about half of the transplanted fingers that had phantom phenomena before toe transplantation, and also in about half of the amputated fingers without the surgery. Conversely, phantom toe phenomena occurred in 13 (17%) patients. Although some patients had mild-to-moderate unpleasant phantom sensations, none had severe or distressing phantom finger pain or phantom toe pain. It is concluded that phantom phenomena occurred in both finger and toe amputations, and that toe-to-finger transplantation appeared to facilitate the disappearance of phantom phenomena not only in the transplanted fingers but also in the amputated but untransplanted fingers. Possible mechanisms for these observations are discussed.

Random X chromosome methylation patterns in the carriers with clinical phenotypic expressions of X-linked recessive bulbospinal neuronopathy.

OBJECTIVES: We report the unusual phenotypic expression in 2 female carriers of a family with X-linked recessive bulbospinal neuronopathy (X-BSN). We analyze the methylation pattern of the androgen receptor (AR) gene to inspect the possibility of non-random X chromosome inactivation to be the underlying mechanism. MATERIAL AND METHODS: Twenty-three members in 3 generations of a Taiwanese family were examined and studied for genomic DNA analysis. We analyzed the sequence of the first exon of the AR gene to identify the numbers of CAG repeats, and to determine the methylation pattern by using the restriction enzymes HpaII and HhaI. RESULTS: There were 3 probands and 5 carriers and 2 of the carriers manifested clinical symptoms. Sequence analysis revealed that the numbers of trinucleotide repeats ranged from 42 to 45 in one allele of the X-chromosome in the 5 female carriers. The restriction pattern of the HpaII and HhaI recognizable sites of the X-chromosome indicated a random methylation. CONCLUSION: Our data suggest that molecular genetic studies are important in confirming the diagnosis of X-BSN and early detection of female carriers, and the random or non-random methylation pattern of the X-chromosome is not a determining factor for partial expression of the abnormal AR gene in some carriers.

Serial magnetic resonance imaging in patients with Balò's concentric sclerosis: natural history of lesion development.

We reviewed the magnetic resonance imaging scans from 22 serial studies of 5 patients with Balò's concentric sclerosis collected during the past 3 years. The data showed the concentric lesions did not occur simultaneously but developed step by step in a centrifugal direction. The development of lesions was preceded by an enhancing ring relatively devoid of demyelination and was followed by progressive demyelination occurring mainly at the inner aspect of the enhancement. The same process recurred on the edge of the previous enhanced zone. Thus, an appearance of concentric rings with alternating demyelinated and relatively myelin-preserved bands was formed.

Levels of naturally occurring microorganisms on surgical instruments after clinical use and after washing.

Surgical instruments exposed to sterile body sites should be contaminated with relatively low levels of microbial contamination or bioburden; however, few studies in the literature have determined the quantitative level and types of contamination. A study was conducted at 2 clinical sites to determine the level of microbial contamination of surgical instruments after clinical use and after washing. Quantitative assays showed that bioburden levels were in the range of 0 to 4415 colony forming units per instrument after clinical use, and 88% of the instruments had bioburden levels lower than 1000. As expected, a reduction in counts occurred after washing; however, in some cases, higher counts were found on the instruments after the washing process. Although the washing procedure is effective in reducing the microbial levels deposited on the surgical instruments during use, a recontamination process occurs that results in increased counts after washing. The low bioburden level after washing consists of predominantly vegetative microorganisms that present a relatively low challenge to sterilization and disinfection systems.

Proton chemical shift imaging of the hippocampus in patients with complex partial seizures.

BACKGROUND: Cerebral metabolites can be evaluated non-invasively using in vivo proton magnetic resonance spectroscopy (MRS). Decreased N-acetylaspartate (NAA) and increased choline-containing compounds (Cho) and creatine-phosphocreatine (Cr) have been found in the hippocampus of patients with complex partial seizures (CPS). METHODS: We prospectively studied hippocampal proton MRS of 10 patients with CPS and 12 control subjects by using the chemical shift imaging (CSI) technique. The spectral data were analyzed in terms of the ratio between the integral peak area of NAA and that of (Cho + Cr). RESULTS: Compared with the control group, patients with CPS showed a significantly lower NAA/(Cho + Cr) ratio, both in the anterior and posterior hippocampus (p value = 0.001 and 0.002, respectively). Metabolic abnormalities of the hippocampus were detected using proton CSI in all the patients with normal MRI results (4 patients) and those with normal EEG results (3 patients). Lateralizations using proton CSI were obtained in all the 10 patients in this study, including concordant lateralization in the 6 patients with MRI-detectable abnormalities. CONCLUSION: The hippocampal abnormalities in patients with CPS can be detected early using proton CSI than using MRI or surface EEG. Lateralization of the seizure focus using proton CSI is possible, but further correlation with the surgical outcome in a larger study group is necessary.

Proton MR spectroscopy in patients with complex partial seizures: single-voxel spectroscopy versus chemical-shift imaging.

BACKGROUND AND PURPOSE: Proton MR spectroscopy has recently been applied to the evaluation of seizures, but few comparisons have been made between different clinical spectroscopic techniques. Our goal was to determine whether there is a significant difference between hippocampal NAA/(Cho+Cr) ratios obtained by single-voxel spectroscopy (SVS) and by chemical-shift imaging (CSI). METHODS: Twelve healthy adults and eight patients with complex partial seizures were studied on a 1.5-T MR scanner using a proton SVS method. Another 12 healthy adults and 10 patients with complex partial seizures were recruited for a proton CSI study, which was performed on a different 1.5-T MR system. The NAA/(Cho+Cr) ratio was calculated from the integral peak areas by curve fitting. The two-tailed t-test was used for statistical analysis. RESULTS: The mean value +/- standard deviation of the hippocampal NAA/(Cho+Cr) ratio in healthy control subjects was 0.63 +/- 0.07 by SVS, with 0.62 +/- 0.15 for the anterior hippocampus and 0.65 +/- 0.11 for the posterior hippocampus by CSI. There was no significant difference between the control group data obtained by SVS and those by CSI, nor was there a regional difference in the CSI NAA/(Cho+Cr) ratio in the hippocampus. Relative to the control group, the patients with seizures had a significant decrease in the NAA/(Cho+Cr) ratio in the abnormal hippocampus: -28% by SVS, and -24% in the anterior hippocampus and -18% in the posterior hippocampus by CSI. Proton SVS and CSI detected hippocampal abnormalities, unilateral or bilateral, in all patients of each group. CONCLUSION: Under similar measurement conditions, proton SVS and CSI provide similar NAA/(Cho+Cr) ratios among healthy control subjects, and they possess comparable ability for detecting hippocampal abnormalities in patients with complex partial seizures.

Bilateral cortical representation of the intrinsic lingual muscles.

Motor potentials of the intrinsic lingual muscles were recorded by surface electrode in a patient with left hypoglossal neuropathy. Electrical stimulation of the right hypoglossal nerve had contralateral lingual muscle potentials representing the crosstalk volume conducted electromyography signals. Magnetic stimulation of either cerebral hemisphere yielded motor responses on the right half of the tongue with more contribution from the crossed corticobulbar fibers. Follow-up studies showed an improvement of dysphagia and left lingual motor potentials.

Natural bioburden levels detected on flexible gastrointestinal endoscopes after clinical use and manual cleaning.

BACKGROUND: Colonoscopes present a special bacterial decontamination challenge because the colon has a large and diverse microbial population. METHODS: Bioburden of colonoscope insertion tube surfaces and suction channels were determined after use and after manual cleaning. RESULTS: After use bioburden in suction channels averaged 7.0 x 10(9) colony-forming units (cfu). Cleaning reduced this level to 1.3 x 10(5). Cleaning of tube surfaces reduced the after-use bioburden from a level of 5.1 x 10(5) to 2.2 x 10(4) cfu. Gram-negative rods accounted for approximately 99% of the bioburden within the suction channel after use and after cleaning. After use flora were predominantly Escherichia coli and Bacteroides. The flora shifted to waterborne Pseudomonas organisms, and other members of the family Enterobacteriaceae after cleaning. Gram-positive bacteria were the primary isolates from the device surfaces both after use (56%) and after cleaning (47%). Because gram-positive cocci and diphtheroids are a part of the normal microbiota of the skin, these bacteria may have been introduced by the hospital environment or by handling. CONCLUSIONS: After the cleaning of in-use colonoscopes, fewer than 10(6) vegetative bacteria could be recovered. This value is several logs lower than some previous estimates. This finding may be useful in the formulation of sterilization and disinfection cycles. Microflora from the colonoscopes indicated that the cleaning process introduced waterborne and enteric microorganisms, which highlights the importance of sanitation in the device reprocessing area.

X-linked recessive bulbospinal neuronopathy: clinical and molecular studies in a Taiwanese family.

We describe clinical, biochemical, and molecular studies on a Taiwanese family with X-linked recessive bulbospinal neuronopathy. There were three probands and five female carriers among the 23 members examined. The clinical manifestations included progressive muscle weakness, diffuse fasciculation, postural tremor, muscle cramps, dysarthria, dysphagia, diabetes, and gynecomastia. Phenotypic expression varied among the affected subjects. Two carriers also had postural tremor and perioral fasciculation. Endocrine tests were normal except for a mild increase in serum testosterone and/or growth hormone in one patient and one carrier. Type IV hyperlipoproteinemia was observed in two patients, one carrier, and one healthy offspring. Molecular genetic studies confirmed elongation of the CAG triplet repeat in exon 1 of the gene for the androgen receptor. Sequence analysis showed that there were 42 to 43 CAG repeats in the three probands and 42 to 45 in the five carriers. The mutant allele had a tendency to increase by one or two repeats from one generation to the next. The length of CAG repeats at which the mutant allele became unstable was shorter in our family than in previous reports. The normal allele was also unstable and had a tendency to shrink by one of five repeats during transmission. These findings suggest that the number of CAG triplet repeats is variable in both the mutant and normal alleles.

Long-term progression in chronic manganism: ten years of follow-up.

We studied the long-term clinical course of five patients with chronic manganese intoxication. The mean scores of the King's College Hospital Rating Scale for Parkinson's disease increased from 15.0 +/- 4.2 in 1987 to 28.3 +/- 6.70 in 1991 and then to 38.1 +/- 12.9 in 1995. The deterioration was most prominent in gait, rigidity, speed of foot tapping, and writing. Tissue concentrations of manganese in blood, urine, scalp hair, and pubic hair returned to normal. Follow-up MRIs did not show paramagnetic high-signal intensity on T1-weighted images. The data indicate that clinical progression in patients with manganese parkinsonism continues even 10 years after cessation of exposure.