Clinical neurological abnormalities in young adults with Asperger syndrome

Children with Asperger syndrome (AS), a neurodevelopmental disorder falling in the autism spectrum disorders, have an increased rate of neurological abnormalities, especially in motor coordination. While AS is a lifelong condition, little is known about the persistence of neurological abnormalities in adulthood. Twenty young adults with AS were compared with 10 healthy controls using a structured clinical neurological rating scale. The score for neurological abnormalities was higher in the AS group. In addition, a subscore for neurological soft signs indicating defective functioning of the central nervous system with a non-localizing value was significantly higher in the AS subjects. This preliminary study indicates that neurological abnormalities, soft signs in particular, represent a non-specific vulnerability factor for AS. Consistent with other features of AS, neurological abnormalities seem to persist into adulthood.     

Neurological complications of acute multifocal placoid pigment epitheliopathy

Acute multifocal placoid pigment epitheliopathy (AMPPE) is an autoimmune chorioretinal disease that can be complicated by neurological involvement. There is limited information on this potentially treatable condition in the neurological literature. The objective of this patient series is to describe the neurological complications of AMPPE. We retrospectively identified patients with neurological complications of AMPPE seen at Auckland Hospital between 2008 and 2013 and summarised cases in the literature between 1976 and 2013. We identified five patients with neurological complications of AMPPE at Auckland Hospital and 47 reported patients. These patients demonstrated a spectrum of neurological involvement including isolated headache, stroke or transient ischaemic attack, seizures, venous sinus thrombosis, optic neuritis, sensorineural hearing loss and peripheral vestibular disorder. We propose criteria to define AMPPE with neurological complications. A cerebrospinal fluid (CSF) lymphocytosis in a patient with isolated headache may predict the development of cerebrovascular complications of AMPPE. Patients with cerebrovascular complications of AMPPE have a poor prognosis with high rates of death and neurological disability among survivors. Predictors of poor outcome in those who develop neurological complications of AMPPE are a relapsing course, generalised seizures and multifocal infarction on MRI. All patients with neurological complications of AMPPE, including headache alone, should be investigated with an MRI brain and CSF examination. Patients with focal neurological symptoms should receive intravenous (IV) methylprednisolone followed by a tapering course of oral steroids for at least 3 months. Patients with AMPPE and an isolated headache with a CSF pleocytosis should be treated with oral steroids.     

HIV-related neurological syndromes reduce health-related quality of life

BACKGROUND: Human immunodeficiency virus (HIV) infection frequently results in neurological complications but the impact of different neurological syndromes on patients' quality of life remains unknown. METHODS: We investigated health-related quality of life (HRQoL) parameters among HIV/Acquired Immune Deficiency Syndrome (AIDS) patients with and without neurological disease, including 11 dimensions of HRQoL within the Medical Outcomes Short-form Health Survey-HIV. RESULTS: Comparisons of sociodemographic and systemic clinical variables did not differ between HIV/AIDS patients with (n=94) and without (n=75) neurological disease. However, patients with neurological diseases exhibited significantly lower HRQoL scores compared to matched controls, which was most evident among HIV/AIDS patients with cognitive impairment and sensory neuropathy. Prospective analysis revealed diminishing HRQoL scores prior to neurological diagnosis followed by a progressive and sustained improvement in HRQoL scores after intervention over a 96-week period. CONCLUSIONS: These studies indicate that while HIV-related neurological diseases are associated with reduced HRQoL scores, enhanced neurological care has a positive impact on HIV/AIDS patients' overall well-being.     

NEUROLOGICAL DISORDERS and DETRUSOR HYPERREFLEXIA

In 152 consecutively selected patients with detrusor hyperreflexia (DH) 96 (63 per cent) had neurological disorders. Thirty-two patients did not show any primary neurological or urological cause for DH. This group was chosen to elucidate the evaluation of possible neurological symptoms in relation to the urological symptomatology. Nine had died and one failed to appear to the neurological examination. Twelve (63 per cent) of the 22 examined patients showed signs of lesions in different parts of the central nervous system, particularly cerebrovascular diseases and myeloneuropathy. Six had had neurological symptoms for years. In two the urological symptoms were first to appear. In the group with no neurological complaints the urological symptoms had existed for 2–30 years. No essential difference was found in the degree of voiding disturbance whether or not neurological signs were disclosed. It is concluded that the discovery of DH should be followed by a neurological examination to disclose further signs of lesions in CNS. Likewise, an extended urological examination with demonstration of DH might help in the evaluation of an obscure neurological disease.     

脑出血患者血清肝细胞生长因子神经生长因子水平与神经功能缺损的相关性

目的 分析脑出血患者肝细胞生长因子(HGF)、神经生长因子(NGF)水平与神经功能缺损的相关性.方法 选取濮阳市安阳地区医院2018-01—2020-01收治的237例脑出血患者为研究对象,均于入院时检测HGF及NGF水平,于发病72 h时评估神经功能缺损程度,分析HGF和NGF水平与患者神经功能缺损的相关性.结果 2...     

Severity of alcohol dependence and its relationship to neurological soft signs,neuropsychological impairment and family history

We examined the relationship between severity of alcohol dependence, subtle neurological impairment, neuropsychological deficits and genetic vulnerability among 36 day hospital attenders who satisfied the DSM-III-R criteria for alcohol dependence. Severity of alcohol dependence was unrelated to the presence of a family history, but was correlated with neurological soft signs and neuropsychological impairment. Neurological soft signs were correlated with neuropsychological impairment on both Trail A and Trail B. Patients with an affected first-degree relative exhibited more neurological soft signs. These data indicate that severity of alcohol dependence is related not only to neuropsychological impairment, but also to subtle neurological deficits which may not be apparent on conventional neurological examination. Patients with a positive family history of alcohol dependence may be particularly susceptible to the neurological sequelae of alcohol dependence, or may have neurological deficits which antedate their alcohol dependence.     

Obstetric complications and neurological abnormalities in neuroleptic-naive psychotic patients

Studies addressing the relationship between a history of obstetric complications (OCs) and neurological abnormalities in schizophrenia have produced contradictory findings. Using a pre-posttreatment design in a neuroleptic-naive sample of psychotic patients, we examined the relationship of a history of OC to primary and drug-induced neurological signs. Fifty neuroleptic-naive non-affective psychotic inpatients were assessed for a history of OC by using the McNeil-Sjöström scale, and for neurological signs including parkinsonism, dyskinesia, akathisia and catatonia, which were rated before and after inception of neuroleptic treatment. A subsample of 28 patients were also examined for neurological soft-signs. Ratings of OCs were related to admission levels of parkinsonism, dyskinesia, akathisia and neurological soft-signs, but not to levels of catatonia. By obstetric period, pregnancy complications were related to levels of parkinsonism, dyskinesia, and neurological soft-signs, and neonatal complications were related to levels of akathisia. Drug-induced neurological signs were not associated with a history of OCs. We argue that the association pattern between a history of OCs and primary neurological signs from several domains suggests a causal link among these variables. Having a history of OCs does not convey a vulnerability for developing drug-induced neurological signs in the short term.     

Neurological complications of porphyria.

The aim of this study was to evaluate and describe the importance of neurological complications in patients with a confirmed diagnosis of porphyria. Clinical details are presented for a cohort of 14 patients who presented with one of four categories of symptoms: seizures, polyneuropathy, transient sensory-motor symptoms and cognitive or behavioural abnormalities. Ascertainment of porphyria was often incidental and in many patients neurological complications preceded the definitive biochemical diagnosis. Porphyria is a group of diseases whose clinical picture is often complex and heterogeneous, but neurological complications are not uncommon. When indicated, differential diagnosis of neurological signs and symptoms should include porphyria, as the incidence of the disease is probably underestimated. Part of the clinical picture can be transient and it is often initially disregarded. A family history and recurrence of otherwise unexplained neurological symptoms should alert the clinician to a possible diagnosis of porphyria for patients with neurological presentations.     

卒中后抑郁与急性期神经功能缺损程度的相关研究

目的 探讨脑卒中后抑郁（post stroke depression,PSD）发生率以及与急性期神经功能缺损程度的关系。方法 对289例住院诊断为脑卒中的患者,用改良的爱丁堡斯堪的那维亚神经功能缺损评分表（SSS）对卒中急性期进行评分,根据急性期神经功能缺损情况分为三组。用汉密尔顿抑郁量表进行随访调查抑郁评分。结果 PSD的发病率在随访1年期间中随着时间的延长逐渐增加;PSD的发病率和重度PSD的发病率随神经功能缺损程度的加重而增加;PSD的程度随着神经功能缺损程度增加而加重。结论 PSD是脑卒中后的常见并发症,卒中后1年中发病率逐渐增加,其严重程度与卒中急性期的神经功能缺损程度一致。     

Neurological signs in parents of schizophrenic patients

The importance of neurological abnormalities in relatives of schizophrenic patients is not completely clear and has been questioned. The hypothesis that neurological abnormalities are trait markers for a vulnerability to develop schizophrenia was tested in 32 parents of patients with schizophrenia and 34 healthy controls. A comprehensive and standardized neurological assessment battery was used. The examiners were blind as to whether they tested a parent of a patient or a healthy control. Four function domains were investigated; higher cerebral functions, cranial nerve functions, general motor functions and gait. There were no significant differences between parents of patients and healthy controls on any of the neurological function domains, or on the total number of neurological abnormalities. No difference was found between parents with a positive family history of schizophrenia spectrum disorders, parents with a negative family history and controls. Results suggest that the neurological functions investigated are not related to a genetic liability to develop schizophrenia.     

A 3-year prospective study of neurological soft signs in first-episode schizophrenia

Neurological soft signs are biological traits that underlie schizophrenia and are found to occur at higher levels in at-risk individuals. The expression of neurological soft signs may be modifiable during the onset of the first psychotic episode and the subsequent evolution of the illness and its treatment. This study investigates neurological soft signs in 138 patients with first-episode schizophrenia and tracks the expression of motor soft signs in the following 3 years. For the 93 patients who have completed the 3-year follow-up, we find that neurological soft signs are stable in the 3 years that follow the first psychotic episode, and that neurological soft signs are already elevated at the presentation of first-episode psychosis in medication-naive subjects. The level of neurological soft signs at clinical stabilization is lower for patients with a shorter duration of untreated psychosis. Although the quantity of neurological soft signs does not significantly change in the 3 years that follow the first episode, the relationship between neurological soft signs and negative symptoms does not become apparent until 1 year after the initial episode. A higher level of neurological soft signs is related to a lower educational level and an older age at onset, but the level of neurological soft signs does not predict the outcome in terms of relapse or occupational functioning.     

WHO/WFN Survey of neurological services: a worldwide perspective

According to the findings obtained in the context of a Global Initiative on Neurology and Public Health carried out by the World Health Organization (WHO), there has been a lack of reliable and comparative data on services and other resources for neurological disorders in many parts of the world. In view of these findings and in collaboration with the World Federation of Neurology (WFN), WHO has recently organized an international Survey of Country Resources for Neurological Disorders, which involved 109 countries and covered over 90% of the world's population. This large WHO/WFN collaborative endeavour collected expert information on a number of aspects of neurological care provision around the world including availability of neurological services in primary care; human resources for neurological disorders; sub-specialized neurological services; primary method of financing of neurological care; and disability benefits for patients with neurological disorders. The WHO/WFN Survey results clearly demonstrate that there are inadequate resources for patients with neurological disorders in most parts of the world, and highlight inequalities in the access to neurological care across different populations, and in particular in those living in low-income countries and in developing regions of the world. The key findings of the WHO/WFN Survey including their impact on delivery of neurological care around the world are presented and discussed in this paper. The entire set of WHO/WFN Survey results including numerous tables, graphs and accompanying commentaries can be found in the WHO/WFN Atlas of Country Resources for Neurological Disorders, which is available on request from WHO or at http://www.who.int/mental_health/neurology/ .     

Hyperbaric oxygen therapy: Can it prevent irradiation-induced necrosis?

Radiosurgery is an important non-invasive procedure for the treatment of tumors and vascular malformations. However, in addition to killing target tissues, cranial irradiation induces damage to adjacent healthy tissues leading to neurological deterioration in both pediatric and adult patients, which is poorly understood and insufficiently treatable. To minimize irradiation damage to healthy tissue, not the optimal therapeutic irradiation dose required to eliminate the target lesion is used but lower doses. Although the success rate of irradiation surgery is about 95%, 5% of patients suffer problems, most commonly neurological, that are thought to be a direct consequence of irradiation-induced inflammation. Although no direct correlation has been demonstrated, the appearance and disappearance of inflammation that develops following irradiation commonly parallel the appearance and disappearance of neurological side effects that are associated with the neurological function of the irradiated brain regions. These observations have led to the hypothesis that brain inflammation is causally related to the observed neurological side effects. Studies indicate that hyperbaric oxygen therapy (HBOT) applied after the appearance of irradiation-induced neurological side effects reduces the incidence and severity of those side effects. This may result from HBOT reducing inflammation, promoting angiogenesis, and influencing other cellular functions thereby suppressing events that cause the neurological side effects. However, it would be significantly better for the patient if rather than waiting for neurological side effects to become manifest they could be avoided. This review examines irradiation-induced neurological side effects, methods that minimize or resolve those side effects, and concludes with a discussion of whether HBOT applied following irradiation, but before manifestation of neurological side effects may prevent or reduce the appearance of irradiation-induced neurological side effects.     

Polyradiculoneuropathy in dourine-affected horses

Dourine is an equine protozoan disease caused by Trypanosoma equiperdum. Dourine-afflicted animals die after developing neurological clinical signs, such as unilateral paresis. The disease has been a problem for many years; however, the pathogenesis regarding the neurological clinical signs of dourine has been unclear. In the present study, we conducted a histopathological examination in order to investigate the mechanisms by which dourine-afflicted horses develop the accompanying neurological clinical signs. Four dourine-afflicted horses in Mongolia were evaluated. An apparently healthy horse exhibited multifocal neuritis without axonal or myelin degeneration. The other horses, which had obvious neurological clinical signs, also exhibited multifocal neuritis. In particular, the nerves that innervated areas associated with neurological clinical signs exhibited neuritis with demyelination in the latter horses. Inflamed, non-demyelinating nerves were infiltrated with B lymphocytes and T lymphocytes; while inflamed, demyelinating nerves were infiltrated with mononuclear phagocytes. Our observations revealed lesion progression in the nerves, such that polyradiculoneuropathy could explain the accompanying neurological clinical signs of dourine. To our knowledge, this is the first report to describe a pathogenic mechanism for the development of the neurological clinical signs found in dourine-afflicted horses.     

Which neurological diseases are most likely to be associated with “symptoms unexplained by organic disease”

Many patients with a diagnosis of neurological disease, such as multiple sclerosis, have symptoms or disability that is considered to be in excess of what would be expected from that disease. We aimed to describe the overall and relative frequency of symptoms 'unexplained by organic disease' in patients attending general neurology clinics with a range of neurological disease diagnoses. Newly referred outpatients attending neurology clinics in all the NHS neurological centres in Scotland, UK were recruited over a period of 15 months. The assessing neurologists recorded their initial neurological diagnoses and also the degree to which they considered the patient's symptoms to be explained by organic disease. Patients completed self report scales for both physical and psychological symptoms. The frequency of symptoms unexplained by organic disease was determined for each category of neurological disease diagnoses. 3,781 patients participated (91% of those eligible). 2,467 patients had a diagnosis of a neurological disease (excluding headache disorders). 293 patients (12%) of these patients were rated as having symptoms only "somewhat" or "not at all" explained by that disease. These patients self-reported more physical and more psychological symptoms than those with more explained symptoms. No category of neurological disease was more likely than the others to be associated with such symptoms although patients with epilepsy had fewer. A substantial proportion of new outpatients with diagnoses of neurological disease also have symptoms regarded by the assessing neurologist as being unexplained by that disease; no single neurological disease category was more likely than others to be associated with this phenomenon.     

Hereditary hemorrhagic telangectasia and spinal cord infarct: case report with a review of the neurological complications of HHT

Hereditary hemorrhagic telangectasia (HHT), also known as Osler-Weber-Rendu disease, is an autosomal dominant vascular dysplasia with high penetrance and variable expressivity. A wide variety of neurological complications have been reported in association with this condition. We report the first case of spinal cord infarction likely due to paradoxical embolization with HHT and review the literature on the neurological complications of this disorder. MEDLINE was employed to identify all published reports of HHT with neurological complications. We identified 44 references with a total of 436 cases of neurological complications of HHT. The most common complication was ischemic stroke and the main etiology for the vascular neurological complications in patients with HHT was pulmonary arteriovenous malformation. HHT should be considered in the differential diagnosis of any patient with cutaneous or mucosal telangiectasia or a history of unexplained epistaxis. HHT is associated with a diverse array of neurological disorders; most commonly ischemic and hemorrhagic stroke, transient ischemic attack, and brain abscess. While myelopathy secondary to arteriovenous malformation with HHT has been previously reported, this is the first instance of spinal cord infarction due to paradoxical embolization in this disorder.     

NEUROLOGICAL AND MOTOR FUNCTIONING OF 10–12-YEAR-OLD CHILDREN WHO SHOWED MILD TRANSIENT NEUROLOGICAL SYMPTOMS IN THE FIRST FIVE DAYS OF LIFE

This study concerns itself with the neurological functioning of 11-year-old children who neonatally presented a variety of shortlived or transient symptoms. These symptoms, while shortlived, are similar in nature to symptoms which other authors have shown to be correlated with later increased rate of neurological deviance. By using the data from a birth cohort of 9,006 consecutive deliveries, we attempted to discover whether such symptoms, when only transiently present in the newborn, are correlated with later neurological functioning. Results indicate that the subjects with transient neonatal symptoms presented more neurological deviance at age 10–12 than did matched controls.     

Correlation of viral load and CD8 T-lymphocytes with development of neurological manifestations in vertically HIV-1-infected infants. A prospective longitudinal study

To assess the predictive power of immunological and virological markers for the development of neurological syndromes, 39 HIV-1-infected infants with a mean age of 4.05+/-0.5 months and without neurological manifestations at enrolment were studied. They had neither been previously treated with antiretroviral therapy, nor had their mothers been given such treatment during pregnancy. They were routinely assessed for signs of neurological impairment during follow-up (19.54+/-3.37 months). Cox regression analysis was used to evaluate the risk of appearance of neurological signs, associated to viral load and T-lymphocyte subsets. A HR > 1 for viral load, and <1 for CD8+, but not for CD4+T-lymphocyte percentage, was observed, indicating that higher viral load and lower CD8+ T-lymphocytes percentages are risk factors for developing neurological signs. By applying the Kaplan-Meier method we found that infants with viral load > 5 1og10 copies/ ml or <20% CD8+T lymphocyte had higher relative risk for developing neurological impairment than those with these two parameters below or above these values, respectively. Finally, CD8+ T lymphocyte had a stronger prognostic value to predict neurological manifestations than viral load. Our data strongly suggest that in the early postnatal period viral load and CD8+ percentages are useful markers in predicting neurological impairment. To our knowledge, this is the first time that CD8+ T-lymphocyte levels are related to development of neurological disorders in AIDS.     

Brain-stem auditory evoked potentials in children with perinatal encephalopathies.

OBJECTIVE: We sought to describe if neurological damage, in terms of brain lesions, syndrome and syndrome severity led to abnormalities in the brain-stem auditory evoked potentials (BAEPs) in order to provide a profile of children that could be used as an indicator of subsequent neurological sequelae. We analyzed the BAEPs from a group of children having prior evidence of neurological damage and determined the presence of neurological sequelae when the subjects were 3 years old. METHODS: Brain-stem auditory evoked potentials (BAEPs) were carried out in a group of 154 children with perinatal neurological damage. The children were classified with neurofunctional (clinical and EEG alterations) or organic and neurofunctional brain disease (clinical, EEG and image alteration) and were all followed from the first month of life and serially for 3 years. We used principal component analysis (PCA), clustered analysis and linear correlation to determine association between BAEPs, risk factors and future sequelae. RESULTS: Latencies of BAEPs decreased significantly with age, and the time of conduction was modified by the presence of neurological damage. All statistical analyses suggested positive and significant associations between risk factors (trophism and condition at birth), and the latencies of waves I, III and V as well as with IPL III-V (interpeak latency) and I-V. PCA showed that IPL I-III was also positively associated with condition at birth, severity of the neurological syndrome and encephalopathy. In addition, we found that the presence and type of sequela reflected changes in the latencies of the waves, as well as IPLs, primarily those of IPL I-III. CONCLUSIONS: Our results suggest that statistical methods are often needed to analyze neurological damage. The relation between BAEPs, risk factors and neurological sequelae allowed us to obtain a profile of children, which can be then used as an aid in the prognosis of children having a risk of developing neurological sequelae.