[Update on anti-N-methyl-D-aspartate receptor encephalitis]. / Update Anti-N-Methyl-D-Aspartat-Rezeptor-Enzephalitis.

Autoimmune encephalitis is a group of autoimmune inflammatory disorders affecting both grey and white matter of the central nervous system. Encephalitis with autoantibodies against the N­methyl-D-aspartate receptor (NMDA-R) is the most frequent autoimmune encephalitis syndrome presenting with a characteristic sequence of psychiatric and neurological symptoms. Treatment necessitates a close interdisciplinary cooperation. This article provides an update on the current knowledge on diagnostic standards, pathogenesis, and treatment strategies for anti-NMDA-R encephalitis from psychiatric and neurological perspectives.

Review of patients' experiences with fungating wounds and associated quality of life.

OBJECTIVE: To investigate the evidence exploring the experiences of patients with fungating wounds and associated quality of life, and to subsequently provide recommendations to how these implications may be addressed in practice. METHOD: Using a systematic approach, a comprehensive literature search was conducted to investigate the most appropriate and relevant evidence regarding the experiences of patients with fungating wounds. RESULTS: Studies unveiled the enormity of the unrelenting, unique and devastating consequences that these wounds have on an individual's life and that every domain of their life is negatively affected. CONCLUSION: These findings must galvanise nurses to become aware of the extent of the devastation experienced and aspects of life affected by these wounds. The issues raised have multifaceted and challenging implications for practice; however, all aspects need to be addressed and satisfied in an attempt to improve the quality of life of individuals with fungating wounds.

Neuropsychological symptoms in paraneoplastic disorders.

Neuropsychological symptoms may rarely be part of a paraneoplastic neurological syndrome, mainly as limbic encephalitis. As the tumour will not be known in most patients upon presentation to the neurologists, state-of-the art diagnosis including the use of anti-neuronal antibodies and FDG-PET is essential for adequate management of these patients.

Paraneoplastic limbic encephalitis: neuropsychiatric presentation.

Limbic encephalitis as a distinct clinicopathological entity is becoming increasingly familiar to neurologists. However, despite its classical clinical presentation of mental status changes and behavioral abnormalities, the disorder is not well known in the psychiatric literature and premortem diagnosis is rare. We recently participated in the care of a patient who spent two months on a psychiatric service and in whom a medical disorder was consistently suspected but not confirmed until autopsy revealed paraneoplastic limbic encephalitis and two primary systemic malignancies. A detailed neuropsychiatric description of this clinical entity is provided from presentation to autopsy with review of the literature.

Neurologic paraneoplastic syndromes.

Several neurologic paraneoplastic disorders are believed to be caused by an autoimmune reaction against antigen(s) co-expressed by tumour cells and neurons. Of the paraneoplastic syndromes, the evidence for an autoimmune etiology is strongest for the Lambert-Eaton myasthenic syndrome, in which autoantibodies downregulate voltage-gated calcium channels at the presynaptic nerve terminal. For other syndromes, including cerebellar degeneration, multifocal encephalomyelitis, sensory neuronopathy, limbic encephalitis, opsoclonus-myoclonus, stiff person syndrome, and retinal degeneration, the autoimmune theory is supported by the presence of specific antineuronal antibodies. These antibodies serve as a useful diagnostic tool, but their actual role in causing neuronal injury and clinical disease remains unclear. Further understanding of immunopathogenesis awaits successful experimental models. Among different syndromes, a varied proportion of patients shows neurologic improvement with immunosuppressive treatments; it is likely that many patients have already suffered irreversible neuronal injury at the time of diagnosis.

Paraneoplastic limbic encephalitis associated with bronchogenic carcinoma: a case report.

Paraneoplastic limbic encephalitis is a rare clinical entity, associated most often with the oat cell carcinoma of the lung. Clinically, it presents with affective changes in personality, memory loss, confusional state, hallucinations, and seizures; with dementia being the common feature as the disorder progresses. Response to treatment is disappointingly poor.

Exercise training in juvenile dermatomyositis.

OBJECTIVE: To investigate the effects of a supervised exercise training program on health parameters, physical capacity, and health-related quality of life in patients with mild and chronic juvenile dermatomyositis (DM). METHODS: This was a prospective longitudinal study following 10 children with mild and chronic juvenile DM (disease duration >1 year). The exercise program consisted of twice-a-week aerobic and resistance training. At baseline and after the 12-week intervention, we assessed muscle strength and function, aerobic conditioning, body composition, juvenile DM scores, and health-related quality of life. RESULTS: Child self-report and parent proxy-report Pediatric Quality of Life Inventory scores were improved after the intervention (-40.3%; P = 0.001 and -48.2%; P = 0.049, respectively). Importantly, after exercise, the Disease Activity Score was reduced (-26.9%; P = 0.026) and the Childhood Muscle Assessment Scale was improved (+2.5%; P = 0.009), whereas the Manual Muscle Test presented a trend toward statistical significance (+2.2%; P = 0.081). The peak oxygen consumption and time-to-exhaustion were increased by 13.3% (P = 0.001) and 18.2% (P = 0.003), respectively, whereas resting heart rate was decreased by 14.7% (P = 0.006), indicating important cardiovascular adaptations to the exercise program. Upper and lower extremity muscle strength and muscle function were also significantly improved after the exercise training (P < 0.05). Both the whole-body and the lumbar spine bone mineral apparent density were significantly increased after training (1.44%; P = 0.044 and 2.85%; P = 0.008, respectively). CONCLUSION: We showed for the first time that a 12-week supervised exercise program is safe and can improve muscle strength and function, aerobic conditioning, bone mass, disease activity, and health-related quality of life in patients with active and nonactive mild and chronic juvenile DM with near normal physical function and quality of life.

Cancer cachexia: prevention, early diagnosis, and management.

Cachexia is the most common paraneoplastic syndrome of malignancy and is characterized by anorexia, early satiety, severe body compositional change with weight loss, adipose and muscle loss, weakness (asthenia), anemia, and edema. Cause of death in as many as 20% of patients with cancer is associated with tumor-induced and treatment-related malnutrition and inanition. Early diagnosis of cancer malnutrition often is missed because of lack of attention by the oncology team. The importance of understanding the basics of nutritional oncology by the entire healthcare team (physician, nurse, pharmacist, dietitian, social worker, physical and speech therapists) and the patient and family is outlined with practical interventions being specified. An algorithm for an optimal nutritional approach in patients with cancer is included, with emphasis on early diagnosis and intervention for maintenance of nutritional, body compositional, and functional status of the oncology patients. Quality-of-life issues, pharmacologic intervention in cachexia, and necessity of cooperative oncology group involvement in nutritional oncology are discussed.

Paraneoplastic limbic encephalopathy.

Paraneoplastic limbic encephalopathy (PLE) is a remote, nonmetastatic complication of carcinoma. Neuropsychiatric symptoms consists of cognitive, affective, and behavioral changes that can predate the diagnosis of carcinoma by as much as 6 years. The most striking feature is the development of an amnestic syndrome without generalized intellectual decline. We report a case of PLE, including neuropsychological test results, and review the features of similar cases in the literature. The postulated causes and clinical implications of PLE are discussed.

Temporally-specific retrograde amnesia in two cases of discrete bilateral hippocampal pathology.

The role of the hippocampus in retrograde amnesia remains controversial and poorly understood. Two cases are reported of discrete bilateral hippocampal damage, one of which was a rare case of limbic encephalitis secondary to the human herpes virus 6. Detailed memory testing showed marked anterograde memory impairment, but only mild, temporally-limited retrograde amnesia that covered a period of several years in both autobiographical and factual knowledge domains. The absence of extensive retrograde amnesia in these two cases points to a time-limited role for the hippocampus in the retrieval of retrograde memories, and suggests that entorhinal, perirhinal, parahippocampal, or neocortical areas of the temporal lobe may be more critical than the hippocampus proper for long-term retrograde memory functioning. Our findings offer general support to theories of memory consolidation that propose a gradual transfer of memory from hippocampal to neocortical dependency.

The metabolic anatomy of paraneoplastic cerebellar degeneration.

Eleven patients with acquired cerebellar degeneration (10 of whom had paraneoplastic cerebellar degeneration [PCD]) were evaluated using neuropsychological tests and 18F-fluorodeoxyglucose/positron emission tomography to (1) quantify motor, cognitive, and metabolic abnormalities; (2) determine if characteristic alterations in the regional cerebral metabolic rate for glucose (rCMRGlc) are associated with PCD; and (3) correlate behavioral and metabolic measures of disease severity. Eighteen volunteer subjects served as normal controls. Although some PCD neuropsychological test scores were abnormal, these results could not, in general, be dissociated from the effects of dysarthria and ataxia. rCMRGlc was reduced in patients with PCD (versus normal control subjects) in all regions except the brainstem. Analysis of patient and control rCMRGlc data using a mathematical model of regional metabolic interactions revealed two metabolic pattern descriptors, SSF1 and SSF2, which distinguished patients with PCD from normal control subjects; SSF2, which described a metabolic coupling between cerebellum, cuneus, and posterior temporal, lateral frontal, and paracentral cortex, correlated with quantitative indices of cerebellar dysfunction. Our inability to document substantial intellectual impairment in 7 of 10 patients with PCD contrasts with the 50% incidence of dementia in PCD reported by previous investigators. Widespread reductions in PCD rCMRGlc may result from the loss of cerebellar efferents to thalamus and forebrain structures, a "reverse cerebellar diaschisis."

Paraneoplastic limbic encephalitis with immature ovarian teratoma--a case report.

Paraneoplastic limbic encephalitis (PLE) is a remote, nonmetastatic complication of carcinoma. Neuropsychiatric symptoms usually predate the diagnosis of cancer by 3 months to 6 years and very rarely the symptoms develop after the diagnosis of malignancy. We report the first case of limbic encephalitis associated with an immature ovarian teratoma. Within the month following the diagnosis of the tumor with pathologic stage Ia, somewhat acutely she developed neuropsychiatric symptoms that was exclusively a limbic disorder with impairments in almost every realm of limbic function. This case may show us that it is important to recognize the neuropsychiatric symptoms of PLE as the first manifestation of a very small malignant ovarian tumor and to aggressively try to identify the underlying cancer.

Primary central nervous system lymphoma presenting as autonomic dysfunction.

We describe a patient with primary central nervous system lymphoma (PCNSL) who presented with symptoms of subacute onset of dysautonomia. Autonomic testing indicated a peripheral autonomic neuropathy while magnetic resonance imaging revealed brainstem involvement. We propose that this patient's autonomic dysfunction could be the result of a paraneoplastic syndrome and PCNSL should be considered in the differential diagnosis of dysautonomia.

Neuropsychological functioning in a patient with paraneoplastic limbic encephalitis.

A 54-year-old woman with clinically diagnosed paraneoplastic limbic encephalitis secondary to adenocarcinoma of the lung is described. Neuropsychological evaluation revealed intact visual perception, visual construction, language, speeded processing, and verbal abstract reasoning in the presence of a severe anterograde amnesia for both verbal and visual information. A profound consolidation problem is discussed in view of other diseases of the mesial temporal lobes resulting in impaired consolidation of new material.

[Schizophreniform psychosis in paraneoplastic limbic encephalitis]. / Schizophreniforme Psychose bei paraneoplastischer limbischer Enzephalitis.

Paraneoplastic limbic encephalitis is seldom mentioned in the psychiatric literature and premortem diagnosis is rare. Affective symptoms, agitation, and memory impairment are the core features, which can predate the diagnosis of carcinoma. We report the case of a patient with bronchial carcinoma, whose clinical picture could hardly be distinguished from that of endogenous schizophrenia.