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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The detection, monitoring, and quantification of subclinical hepatic encephalopathy present difficult problems for the clinician caring for patients with liver disease. Traditionally, pencil and paper tests such as signature-writing have been used at the bed-side to measure early encephalopathy. More recently, the Trail-making test has been employed to detect and quantify encephalopathic changes. While the electroencephalogram has provided information about the extent of clinically-obvious encephalopathy, it has only recently proved useful in the detection of subclinical disease. In these cases, evoked potentials and spectral analysis methods have discriminated between 35 and 62% of patients with subclinical hepatic encephalopathy. The present study used the method of feedback electroencephalography to detect and quantify differences in cortical arousal in 5 cirrhotic patients and 5 normal age-matched controls. Subject were also compared with respect to baseline measures of cortical arousal. Finally, arousal during feedback EEG stimulation was correlated with Trail-making test performance. The data revealed that cirrhotic patients can be discriminated from normal controls by baseline (80% detection) and feedback stimulation conditions (100% detection). In addition, feedback EEG reactivity (cortical arousal) was inversely correlated with Trail-making test performance (-0.86, P less than 0.01). The results indicate that simple features of the conventional EEG can reliably discriminate cirrhotic patients from normals. Clinical application of the feedback method in other metabolic encephalopathies, as well as in hypoaroused states secondary to, for example, narcolepsy, is discussed.
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PMID:Feedback EEG in the detection of subclinical hepatic encephalopathy: a preliminary report. 258 89

The neuropathology of narcolepsy is unknown. Recently, Plazzi et al. (1) reported magnetic resonance imaging (MRI) abnormalities in the pontine tegmentum of three patients with long-standing idiopathic narcolepsy. Considering the localization of the neuroradiological findings in the pontine reticular formation, where rapid eye movement (REM) sleep is generated, the authors suggested a causal relationship between narcolepsy and MRI abnormalities. Frey and Heiserman, however, found pontine MRI abnormalities in only two of 12 patients with narcolepsy both of whom had long-standing hypertension (2). Pullicino et al. noted similar pontine MRI abnormalities in patients with subcortical arteriosclerotic encephalopathy-like ischemic rarefaction of the pons (3). Thus, the changes noted by Plazzi et al. may have been caused by small-vessel disease rather than narcolepsy. To assess whether altered pontine MRI signals are a regular feature of idiopathic narcolepsy, we selected randomly from our database seven patients with narcolepsy with cataplexy. Of these seven, three agreed to have brain MRIs; their cases are described below. None had pontine MRI abnormalities.
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PMID:MRI findings in narcolepsy. 935 Nov 30

Increasing experience indicates that anti-Ma2-associated encephalitis differs from classical paraneoplastic limbic or brainstem encephalitis, and therefore may be unrecognized. To facilitate its diagnosis we report a comprehensive clinical analysis of 38 patients with anti-Ma2 encephalitis. Thirty-four (89%) patients presented with isolated or combined limbic, diencephalic or brainstem dysfunction, and four with other syndromes. Considering the clinical and MRI follow-up, 95% of the patients developed limbic, diencephalic or brainstem encephalopathy. Only 26% had classical limbic encephalitis. Excessive daytime sleepiness affected 32% of the patients, sometimes with narcolepsy-cataplexy and low CSF hypocretin. Additional hormonal or MRI abnormalities indicated diencephalic-hypothalamic involvement in 34% of the patients. Eye movement abnormalities were prominent in 92% of the patients with brainstem dysfunction, but those with additional limbic or diencephalic deficits were most affected; 60% of these patients had vertical gaze paresis that sometimes evolved to total external ophthalmoplegia. Three patients developed atypical parkinsonism, and two a severe hypokinetic syndrome with a tendency to eye closure and dramatic reduction of verbal output. Neurological symptoms preceded the tumour diagnosis in 62% of the patients. Brain MRI abnormalities were present in 74% of all patients and 89% of those with limbic or diencephalic dysfunction. Among the 34 patients with cancer, 53% had testicular germ-cell tumours. Two patients without evidence of cancer had testicular microcalcification and one cryptorchidism, risk factors for testicular germ-cell tumours. After neurological syndrome development, 17 of 33 patients received oncological treatment (nine also immunotherapy), 10 immunotherapy alone, and six no treatment. Overall, 33% of the patients had neurological improvement, three with complete recovery; 21% had long-term stabilization, and 46% deteriorated. Features significantly associated with improvement or stabilization included, male gender, age <45 years, testicular tumour with complete response to treatment, absence of anti-Ma1 antibodies and limited CNS involvement. Immunosuppression was not found to be associated with improvement but was clearly effective in some patients. Fifteen patients (10 women, five men) had additional antibodies to Ma1. These patients were more likely to have tumours other than testicular cancer and to develop ataxia, and had a worse prognosis than patients with only anti-Ma2 antibodies (two women, 21 men); 67% of deceased patients had anti-Ma1 antibodies. Anti-Ma2 encephalitis (with or without anti-Ma1 antibodies) should be suspected in patients with limbic, diencephalic or brainstem dysfunction, MRI abnormalities in these regions, and inflammatory changes in the CSF. In young male patients, the primary tumour is usually in the testis, in other patients the leading neoplasm is lung cancer.
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PMID:Clinical analysis of anti-Ma2-associated encephalitis. 1521 14

Narcolepsy is characterized by excessive daytime sleepiness (EDS), cataplexy and other abnormal manifestations of REM sleep. Recently, it was discovered that the pathophysiology of idiopathic narcolepsy-cataplexy is linked to orexin ligand deficiency in the brain and cerebrospinal fluid. Orexin neurons localize in the posterior hypothalamic area, which was previously described as "waking center" by von Economo in 1920s. Hypersomnia due to orexin ligand deficiency can also occur during the course of other neurological conditions, such as hypothalamic tumor, encephalopathy and demyelinating disorder (i.e. symptomatic hypersomnia). We experienced 8 pediatric cases with symptomatic hypersomnia. These cases were diagnosed as brain tumor (n = 2), head trauma (n = 1), encephalopathy (n = 1), demyelinating disorder (n = 3) and infarction (n = 1). Six pediatric cases with orexin measurements from the literatures were additionally included and total 14 cases were studied. Although it is difficult to rule out the comorbidity of idiopathic narcolepsy in some cases, a review of the case histories reveals numerous unquestionable cases of symptomatic hypersomnia. In these cases, the occurrences of the hypersomnia run parallel with the rise and fall of the causative diseases. Most of symptomatic hypersomnia cases show both extended nocturnal sleep time and EDS consisting of prolonged sleep episodes of NREM sleep. The features of nocturnal sleep and EDS in symptomatic hypersomnia are more similar to idiopathic hypersomnia than to narcolepsy.
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PMID:[Symptomatic hypersomnia due to orexin deficiency in hypothalamic lesions]. 1698 34

Sleep disorders are common in dialysis patients. Insomnia is reported in almost 70% of the dialysed. Old age, presence of common sleep disorders, such as sleep apnea syndrome (SAS) and restless legs syndrome (RLS), comorbid clinical conditions, metabolic parameters and characteristics of dialysis, represent the main risk factors for insomnia. RLS is independently associated with uremia, affecting almost 30% of Caucasians dialysed. Pathophysiology of uremic RLS is still unclear. Although the exact pathogenetic mechanism remains unknown, the efficacy of kidney transplantation on RLS symptoms supports the involvement of renal function in this disturbance. SAS affects 30-80% of dialysis patients. The use of neurophysiological measures is necessary to diagnose SAS. This approach is not applicable in all dialysis patients; consequently, validated questionnaires might be useful to screen patients with a high risk of apnea. Risk of obstructive and central respiratory events are increased by renal failure and dialysis therapy. Excessive daytime sleepiness (EDS) is often reported by the dialysed population. Direct effects of uremic encephalopathy and of somnogenic cytokines have been suggested as the cause of EDS, in addition to the sleep disturbances that increase daytime sleepiness by impairing nocturnal sleep efficiency. Although less frequent, the presence of other sleep disturbances (such as nightmares and narcolepsy) should be carefully evaluated in the uremic population. Several sleep disturbances may potentially be treated but, if left untreated, may impair health status and increase the risk of mortality. However, literature and personal data suggest that undertreatment is common, calling to higher awareness of sleep disturbances among nephrologists.
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PMID:Sleep disturbances in dialysis patients. 1844 35

Neuromyelitis optica (NMO) is a severe inflammatory, demyelinating disease, and its clinical characteristics include recurrent optic neuritis and longitudinally extensive transverse myelitis. The NMO-immunoglobulin (Ig) G auto-antibody (Ab), which binds to the aquaporin-4 (AQP4) water channel protein, is a marker for NMO. These clinical and immunological features have been used to distinguish NMO from multiple sclerosis (MS). In 1999, Wingerchuk et al. broadened the clinical criteria for diagnosing NMO to include "negative brain magnetic resonance imaging (MRI) at onset." However, after NMO-IgG/AQP4-Ab became a supportive criterion for diagnosing NMO, patients with NMO were frequently found to have symptomatic or asymptomatic brain lesions. In 2006, Pittock et al. reported that asymptomatic brain lesions were common in NMO, and that NMO brain lesions characteristically occurred in the hypothalamus and periventricular areas, which correspond to brain regions with high levels of AQP4 expression. Furthermore, Nakashima et al. detected abnormalities on brain MRI in 71% of NMO-IgG-positive Japanese patients. Patients with NMO have unique brain lesions that are clearly different from the lesions of patients with MS. In patients with NMO, involvement of the dorsal portion of the medulla oblongata causes intractable hiccups and nausea. Some studies described a hypothalamic lesion, and hypothalamic dysfunction could cause symptomatic hypersomnia, narcolepsy, and endocrinopathies. In some patients with NMO and NMO spectrum disorder who experience blood pressure fluctuations, vasogenic edema, manifesting as posterior reversible encephalopathy syndrome, may occur. In a recent report highlighting brain MRI with contrast enhancement, the most prominent feature that appeared to be a specific finding in NMO was "cloud-like enhancement" with multiple patchy enhancing lesions with a blurred margin. Another report showed that acute, large, edematous callosal lesions with heterogeneous intensity ("marbled pattern") occasionally occur in NMO. This review presents the characteristic clinical features of NMO according to the brain MRI findings and the features that can be used to distinguish NMO from MS.
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PMID:[Clinical features of NMO according to brain MRI findings]. 2084 4

We report a case of probable encephalitis presenting as narcolepsy with cataplexy, but with cyclical exacerbation and cognitive difficulties. Our patient continued to worsen despite treatment for narcolepsy and later was thought to have an immune-mediated encephalopathy. Treatment with intravenous gamma immunoglobulin (IVIG) led to complete recovery. Cyclic symptoms of narcolepsy with cataplexy are thus one presentation of probable immune-mediated encephalitis.
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PMID:Cyclic hypersomnolence with symptoms of narcolepsy with cataplexy: An unusual presentation of probable immune-mediated encephalitis. 2973 25

Objective Non-idiopathic CPP is caused by acquired or congenital hypothalamic lesions visible on MRI or is associated with various complex genetic and/or syndromic disorders. This study investigated the different types and prevalence of non-isolated CPP phenotypes. Design and Methods This observational cohort study included all patients identified as having non-idiopathic CPP in the database of a single academic pediatric care center over a period of 11.5 years. Patients were classified on the basis of MRI findings for the CNS as having either hypothalamic lesions or complex syndromic phenotypes without structural lesions of the hypothalamus. Results In total, 63 consecutive children (42 girls and 21 boys) with non-isolated CPP were identified. Diverse diseases were detected, and the hypothalamic lesions visible on MRI (n = 28, 45% of cases) included hamartomas (n = 17; either isolated or with an associated syndromic phenotype), optic gliomas (n = 8; with or without neurofibromatosis type 1), malformations (n = 3) with interhypothalamic adhesions (n = 2; isolated or associated with syndromic CNS midline abnormalities, such as optic nerve hypoplasia, ectopic posterior pituitary) or arachnoid cysts (n = 1). The patients with non-structural hypothalamic lesions (n = 35, 55% of cases) had narcolepsy (n = 9), RASopathies (n = 4), encephalopathy or autism spectrum disorders with or without chromosomal abnormalities (n = 15) and other complex syndromic disorders (n = 7). Conclusion Our findings suggest that a large proportion (55%) of patients with non-isolated probable non-idiopathic CPP may have complex disorders without structural hypothalamic lesions on MRI. Future studies should explore the pathophysiological relevance of the mechanisms underlying CPP in these disorders.
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PMID:High prevalence of syndromic disorders in patients with non-isolated central precocious puberty. 3032 96