Eye movements as a marker for cerebellar damage in paraneoplastic neurological syndromes

Cerebellar disturbances can induce a variety of motor deficits, ranging from severe ataxia to mild deficits of fine motor control. Although motor disturbances appear as an important clinical feature in many neurological disorders, mild disturbances are often difficult to assess properly. Eye movement recordings using video-oculography in a group of patients with a paraneoplastic neurological disorder revealed subtle saccadic and smooth pursuit deficits when compared to controls. We conclude that an easy quantification of eye movement control may assist in the diagnosis and follow-up of mild motor disturbances in patients with neurological disorders, especially when such signs are not overt during clinical neurological examination.     

Diagnostic criteria for neurocysticercosis: some modifications are needed for Indian patients

In India and other less developed countries the diagnosis of neurocysticercosis is frequently difficult because several other prevalent neurological disorders can present with a similar clinical and neuroimaging picture. Currently available international criteria seem to be helpful for the diagnosis of neurocysticercosis, however, these criteria have been criticized for not being effective in differentiating several other infective and neoplastic diseases of central nervous system (CNS), like CNS tuberculosis, from neurocysticercosis. In this article, modifications in the recent diagnostic criteria given by Del Brutto et al (2001) are being suggested, so, it can become more suitable for Indian patients. In India the overwhelming majority of patients with neurocysticercosis have either single enhancing or less frequently multiple enhancing CT lesions. Imaging and clinical features of various infective conditions, like tuberculoma, fungal granuloma, and parasitic granuloma, and of neoplastic conditions like cerebral metastasis, are remarkably similar. Keeping this in mind, the modification suggested in this article is to replace epidemiological criteria with the section diagnosis of neurocysticercosis with caution in certain situations. These situations are middle or old age, evidence of pre-existing tuberculosis or malignancy, pre-existing HIV infection and in patients with grossly abnormal neurological examination. In these situations, in the absence of one of the absolute criteria, it should be essential to consider and exclude all other likely possibilities before making a diagnosis of neurocysticercosis. However, because of the high prevalence of several disorders with similar features it is difficult to make reliable diagnostic criteria for neurocysticercosis, which are easy to use, and have a high specificity and sensitivity.     

DSM-IV mental disorders and neurological complications in children and adolescents with human immunodeficiency virus type 1 infection (HIV-1).

AIM: - To study the types of psychiatric problem encountered in children infected with the human immunodeficiency virus (HIV) and their relationship to central nervous system disorder and the severity of infection. METHODS: - 17 HIV-infected children presenting with psychiatric problems were included. Mental disorders were evaluated according to DSM-IV criteria. Neurological disorders and progressive encephalopathy (presence or absence) diagnosis were evaluated by clinical and radiological examination. The severity of infection was assessed by the percentage of CD4 lymphocytes. RESULTS: - The most frequent diagnoses were major depression (MDD: 47%) and attention deficit hyperactivity disorder (ADHD: 29%). Major depression diagnosis was significantly associated with neuroimaging or clinical neurological abnormalities (p < 0.01). In contrast, no association was found between hyperactivity diagnosed according to DSM-IV criteria and central nervous system disorder. Percentage of CD4 lymphocytes were close to 0 for more than 80% of children presenting with psychiatric complications. CONCLUSION: - The very low % of CD4 lymphocytes of these children suggest that the appearance of a psychiatric complication should be regarded as a factor indicating severe HIV infection. Depressive disorders may be a clinical form of encephalopathy.     

A clinico-pathoanatomical study of multiple sclerosis diagnosis

The diagnosis of multiple sclerosis (MS) is clinical and verifiable at post mortem. Neuropathological examination of 518 consecutive patients with clinically definite MS revealed a correct diagnosis in 485 cases (94%). Clinical diagnosis had been established by a neurologist in all cases. Erroneous diagnosis included a variety of other neurological disorders. Also investigated was a randomly selected series of 33 patients with a clinical diagnosis of probable MS: post mortem confirmation of MS was obtained in circa 66%, for the remainder the error pattern was similar to the above. Clinical diagnosis of definite MS was correct in 94% cases. Laboratory tests and examinations have not radically improved diagnosis. Neuropathological examination may occasionally fail to demonstrate MS plaques if the optic nerves are not investigated.     

The neurological examination in child psychiatry: a review of its uses

This review is an attempt to evaluate critically some of the recent literature on the clinical utility of the neurological examination in child psychiatric practice. Three separate but related issues are discussed; the accuracy of the examination in assessing cerebral dysfunction, the strength of the association between neurological signs and behaviour disorders and finally the clinical significance of this association. It is pointed out that not all neurological signs are of equal significance, but that developmental neurological signs which indicate cerebral immaturity are weakly associated not only with hyperactivity, but possibly with other behaviour disorders as well. Some of the methodological weaknesses in this literature are pointed out. Recent studies of children with motor impairment (clumsiness) are briefly mentioned as a potentially more fruitful avenue of research. It is concluded that the neurological examination may be useful in identifying sub-groups of behaviourally disturbed children who differ from other children with behaviour disorders in terms of etiology, prognosis or treatment needs. Unfortunately, little work has been done in this specific area and further research is needed.     

Headache with focal neurological signs or symptoms: a complicated differential diagnosis

Headache syndromes can be associated with focal neurological symptoms or signs. Good knowledge of primary headaches, a detailed history and a thorough clinical examination are prerequisites for their differential diagnosis. The neurological symptoms produced by the migraine aura are the most characteristic and recognisable. However, structural lesions, such as vascular malformations, can produce similar symptoms to migraine with aura, which highlights that paraclinical investigations are necessary in most patients with headache and focal neurological symptoms. In this review, we provide an overview of the differential diagnosis of the most common headache disorders with focal neurological symptoms or signs to refresh the practising neurologist's differential diagnostic knowledge for the clinical situation and to aid the teaching of neurology residents.     

Clinical description of the broad range of neurological presentations of COVID-19: A retrospective case series

BackgroundNeurological disorders associated with SARS-CoV-2 infection represent a clinical challenge because they encompass a broad neurological spectrum and may occur before the diagnosis of COVID-19.MethodsIn this monocentric retrospective case series, medical records from patients with acute neurological disorders associated with SARS-CoV-2 infection from medicine departments of an academic center in Paris area were collected between March 15^th and May 15^th 2020. Diagnosis of SARS-CoV-2 was ascertained through specific RT-PCR in nasopharyngeal swabs or based on circulating serum IgG antibodies.ResultsTwenty-six patients diagnosed with SARS-CoV-2 infection presented with neurological disorders: encephalitis (N = 8), encephalopathy (N = 6), cerebrovascular events (ischemic strokes N = 4 and vein thromboses N = 2), other central nervous system (CNS) disorders (N = 4), and Guillain-Barré syndrome (N = 2). The diagnosis of SARS-CoV-2 was delayed on average 1.6 days after the onset of neurological disorder, especially in case of encephalitis 3.9 days, encephalopathy 1.0 day, and cerebrovascular event 2.7 days.ConclusionsOur study confirms that COVID-19 can yield a broad spectrum of neurological disorders. Because neurological presentations of COVID-19 often occur a few days before the diagnosis of SARS-COV-2 infection, clinicians should take preventive measures such as patient isolation and masks for any new admission to avoid nosocomial infections. Anti-SARS-CoV2 antibody detection in RT-PCR SARS CoV-2 negative suspected cases is useful to confirm a posteriori the diagnosis of atypical COVID-19 presentations.     

PREDICTION IN VERY PRETERM INFANTS OF SATISFACTORY NEURODEVELOPMENTAL PROGRESS AT 12 MONTHS

This study was performed to find out how well ultrasound brain-scanning and neurological examination of very preterm infants, together and separately, predicted normal neurodevelopmental progress at 12 months corrected age. 111 infants born at less than 33 weeks gestation were scanned at discharge from the neonatal unit, and neurological examinations were performed at a gestation-equivalent age at or near term. During the first year of life repeated neurological and developmental testing was carried out. At 12 months a diagnosis of normal progress or of major or minor neurodevelopmental disorders was made. 56 (50 per cent) infants with both a favourable ultrasound scan and normal neurological findings had a 98 per cent (90 to 99 per cent) probability of normal progress at 12 months, and a 100 per cent (93 to 100 per cent) probability of having no major disorder. Separately, ultrasound scanning and neurological examination were not such good predictors of normal outcome, although they selected larger groups of infants with high probabilities of progressing normally. Ultrasound brain-scanning and neurological examination can be used in combination to identify potentially normal preterm infants when they are discharged to their homes.     

The presence of psychiatric disorders reduces the likelihood of neurologic disease among referrals to a neurology clinic

OBJECTIVES: This study aims to explore the prevalence and impact of psychiatric disorders on the likelihood of an organic, neurological explanation for symptoms among neurology referrals. METHODS: Consecutive new adult neurology referrals were screened for psychiatric disorders (PRIME-MD) prior to evaluation by neurologists, blinded to these results. Diagnoses were stratified into three categories: no neurological diagnosis, neurological-headache, and neurological-nonheadache. RESULTS: Of 235 patients enrolled, 79 (34%) received no neurological diagnosis, 54 (23%) headache and 102 (43%) a neurological diagnosis. Overall, 39% had an underlying psychiatric disorder. Patients with psychiatric disorders were less likely to have a neurological diagnosis (RR: 0.66, 95% CI: 0.48-0.90): 25% of patients with a neurological diagnosis had an underlying psychiatric disorder, compared to 43% among those with no diagnosis and 57% among those with headaches. CONCLUSION: Psychiatric disorders are common among neurology referrals, particularly those with headaches and are associated with a decreased likelihood of an underlying neurological process.     

Whipple's disease--a rare cause of neurological symptoms and disorders

Whipple's disease is a chronic infection caused by Gram-positive bacillus Thropheryma Whippelii picture with a wide range of clinical manifestation, not only systemic but also neurological. Seronegative arthritis or arthralgia may be the only presenting symptom, predating by years gastrointestinal, and also pulmonary, cardiac, renal and neurological manifestations. The diagnosis can be established based on the characteristic histopathological features found in the affected organ (foamy macrophages with a coarsely granular cytoplasm, which stains with PAS) and PCR of 16S ribosomal RNA of Tropheryma Whippelii. CNS involvement manifests with a broad range of neurological symptomatology: memory, consciousness, hypothalamic, psychiatric and behavioural disorders and other symptoms, which may mimic neurodegeneration, neuroinfection, stroke and tumour. In this review detailed neurological symptomatology, differential diagnosis and laboratory, neurophysiologic and radiologic findings are presented. Whipple's disease is potentially fatal but responds to antibiotic treatment. The current recommendations for treatment are discussed.     

Dissociated nystagmus as a common sign of ocular motor disorders in HIV-infected patients

In order to determine if ocular motor disturbances due to brainstem and cerebellar dysfunction provide a frequent and early marker for HIV infection of the brain, neurological examination was performed in 133 HIV-infected persons who were consecutively admitted to our hospital. In 22 patients (17%) we found no other reason for cerebellar or pontomesencephalic signs than HIV encephalopathy. Ocular motor disorders accounted for the most frequent signs of cerebellar and pontomesencephalic dysfunction. Ocular motor disorders mainly consisted of dissociated nystagmus (n = 12), gaze-evoked nystagmus (n = 10) and impaired smooth pursuit (n = 6). Cerebellar ataxic gait and dysmetria were present in 3 patients. Since dissociated nystagmus was the primary ocular motor disorder, we assume that the medial longitudinal fasciculus may be a predilected circumscribed area for HIV infection of the brain. We suppose that cerebellar and pontomesencephalic disorders may be an early marker for HIV encephalopathy because they were the only neurological signs found in 12 patients.     

Acute, recent and sometimes persistent Mycoplasma pneumoniae infections associated with neurologic manifestations. Discussion of causal relations

Neurological disorders associated with recent and sometimes persistent Mycoplasma pneumoniae infections were present in 9 patients, examined within the course of a year, during the 1980 epidemic in Saint-Etienne, France. Cases included 5 with acute polyneuritis, 2 with lymphocytic meningitis, 1 with a bilateral optic neuritis, and 1 with mild encephalitis presenting as an amnesic disorder. Causal relationships are examined with respect to semiological, biological, therapeutic and epidemiological data. Clinically an initial infection compatible with the mycoplasmic etiology and its time relationship with the nervous system lesion appear to be more significant than the inflammatory neurological symptoms and signs. It is often impossible to ascertain the efficiency of the antibiotic therapy, which thus cannot help to the aetiological diagnosis. From the biological point of view, though seroconversion by complement fixation test remains the basis of the diagnosis, it has been completed by the isolation of Mycoplasma pneumoniae in the blood of 3 of the patients, and by a longitudinal study of specific blood IgM levels in the 6 others. Presence in the CSF of these locally synthesised specific IgM during the early stages of the neurological disorders in 2 patients, constitutes a new significant fact for the physiopathological discussions and a basic fact to clarify the aetiological diagnosis. The concept of persistent infection is discussed with respect to the isolation of Mycoplasma pneumoniae in the blood at a late stage, and the abnormally long presence of high levels of specific serum IgM levels. This biological persistence does not always correspond to a chronic course of the clinical disease which was observed in only 3 of the patients. The mixed viral infection, present in 3 cases, is linked with immunity disorders due to Mycoplasma pneumoniae, which are mainly a cell immunity depression: this markedly complicates the analysis of causal relationships. Finally, the chronological order of the clinical and biological events remains of prime importance when studying each case individually, whereas epidemiological data are essential for establishing a posteriori that the neurological manifestations were true complications of the microorganism.     

The role of quantitative neurological examination in clinical neurotoxicology

The use of the clinical neurological examination to document abnormal signs in suspected neurotoxic disorders is described, recognizing that identifying an abnormal examination does not establish the cause of the problem. Several forms of quantitative tests of neurological function are discussed, and their application to the evaluation of neurotoxic disorders is reviewed. Although results of such testing are rarely specific for toxic exposure, these measures have important application in sequential evaluations to document small changes in neurologic function over time.     

Analysis of long-standing nociceptive and neuropathic pain in patients with post-polio syndrome

The purpose of this study was to analyze pain, both nociceptive and neuropathic, in patients with post-polio syndrome (PPS) and relate the pain to age at the initial polio infection, age at examination, to gender and disability. The study was conducted in a university hospital department. Patients with PPS were interviewed at their regular visits about pain, its character, intensity and localization. A clinical examination, including a thorough neurological examination, was performed. Data included age at time of polio infection, age at time of examination and gender. Pain intensity was measured with the VAS-scale and walking capability by the WISCI-scale. One hundred sixty-three (88 women, 75 men) patients were included in the study. Pain was present in 109 (67%). Pain was more frequently reported by women (82%) than by men (49%). 96 patients experienced nociceptive pain, 10 patients both neuropathic and nociceptive pain and three experienced pure neuropathic pain. Half of the patients with pain experienced pain in more than one body region. When neuropathic pain was present, another additional neurological disorder was diagnosed. Pain was more often found in younger patients (around 70%) than in older patients (around 50%). In summary pain is common in patients with PPS and most patients experienced nociceptive pain. Women have pain more often than men. Older patients experience pain more seldom than younger patients. Age at time of primary polio infection is important for the development of pain. When neuropathic pain is present, it is important to proceed with neurological examination to find an adequate diagnosis.     

The pathogenesis of fetal hypokinesia. A neurological study of 75 cases of congenital contractures with emphasis on cerebral lesions

A comprehensive prospective clinical study is presented of 75 cases of fetal hypokinesia and congenital contractures of various causes, with neuropathological investigation in 23 cases. With the data of medical history, neurological examination, laboratory tests and neuropathology an exact or probable nosological or syndromal diagnosis could be made in 61 cases. These cases were categorized by localisation of causal pathology in the subsequent levels of the developing motor system. In 14 of 61 cases developmental brain disorders (f.i. hydrocephalus, hydranencephaly, microcephaly) were the cause of fetal hypokinesia, often with perinatal death, whereas in 7 cases both cerebral and/or spinal cord lesions were found. Besides cerebral involvement was frequently present in cases with congenital contractures of other origin, concomitant or due to perinatal complications. In a large number of cases clinical evidence of spinal cord lesions, especially anterior horn cell degeneration was present. Myopathic disorders occurred in only four cases, whereas congenital myasthenia and congenital neuropathy were present in one case each. In cases without muscle weakness miscellaneous disorders including congenital skin anomalies and probably primary connective tissue disorders were encountered. The etiologic role of intrauterine viral infection is discussed.     

自身抗体对副肿瘤综合征诊断价值的探讨

目的　探讨自身抗体对副肿瘤综合征的诊断价值。方法　利用间接免疫荧光法对 48例疑诊副肿瘤综合征患者进行抗 Yo抗体、抗 Hu抗体、抗 Ri抗体测定和临床随访 ,并与正常人和神经系统其他疾病患者对照。结果　 48例中 ,1例病前有肺癌史 ,2例检查中发现肿瘤 ,4例随访 3～ 1 8个月后发现肿瘤。正常对照组和神经系统其他疾病组自身抗体均为阴性 ,患者组中有 1例呈副肿瘤性脑脊髓炎 ,其抗 Hu抗体阳性 ,相对分子质量为3 80 0 0 ,病理检查证实为小细胞肺癌。结论　自身抗体测定对此病的早期诊断有一定价值 ,但阳性率不高 ,其临床价值尚需进一步随访证实     

Diagnosing Parkinson's disease using videotaped neurological examinations: validity and factors that contribute to incorrect diagnoses.

Field work is commonly required in movement disorders research. Sending neurologists into the field can be logistically challenging and costly. Alternatively, neurological examinations may be videotaped and reviewed later. There is little knowledge of the validity of the videotaped neurological examination in the diagnosis of Parkinson's disease (PD). We examined the validity of the videotaped Unified Parkinson's Disease Rating Scale (UPDRS) motor examination in the diagnosis of PD, and sought to determine which factors are associated with incorrect diagnoses. PD patients and controls were enrolled in a familial aggregation study between August of 1998 and June of 2000, and as part of that study each was examined by a physician who performed an in-person UPDRS motor examination. Each also underwent a second, videotaped UPDRS motor examination. Based on the review of this videotape, a neurologist, who was blinded to the previous clinical diagnosis, assigned a diagnosis of PD or normal. A total of 211 of 231 PD patients (sensitivity = 91.3%), and 170 of 172 controls (specificity = 98.8%) were correctly identified based on the videotape. True positives had a higher mean rest tremor score (1.7 vs. 0.3; P < 0.001), action tremor score (0.9 vs. 0.3; P < 0.001), bradykinesia score (11.2 vs. 7.4; P = 0.02), and disease of longer mean duration (8.9 vs. 5.8 years; P = 0.001) than false negatives. False negatives did not differ from true positives in terms of age, total dose of levodopa, Hoehn and Yahr score, or rigidity, gait and posture, or facial masking scores (each assessed during the in-person examination). The videotaped UPDRS motor examination is a useful means of diagnosing PD and provides an alternative approach for the diagnosis of PD in field studies. A limitation is that patients with milder PD of shorter duration may not be recognized as PD.     

Human T-lymphotropic virus type II and neurological disease

Human T-lymphotropic virus type I (HTLV-I) and type II (HTLV-II) are closely related retroviruses with similar biological properties and common modes of transmission. HTLV-I infection is endemic in well-defined geographic regions, and it is estimated that some 20 million individuals are infected worldwide. Although most infected individuals are asymptomatic carriers, some 2 to 5% will develop a chronic encephalomyelopathy, HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). In contrast with HTLV-I, the role of HTLV-II in the development of neurological disorders is much less clear. HTLV-II is endemic in many native Amerindian groups and epidemic in injecting drug users (IDUs) worldwide. To evaluate the role of HTLV-II in neurological disease, we have critically reviewed all reported cases of HTLV-II-associated disorders. This has confirmed that although rare infection is associated with a disorder clinically similar or identical to HAM/TSP. However, most reports that have attributed infection to a range of other neurological disorders are difficult to evaluate in that in many cases either the association appears to be fortuitous or the presentations were confounded by a background of concomitant human immunodeficiency virus-1 infection and/or active IDU. In view of the many HTLV-II-infected individuals in urban areas of North America and Europe, neurologists should be aware of the potential clinical consequences of this infection.     

Disabling neurological syndromes: prevalence amongst hospitalized neurological patients

Neurological patient populations are usually described by diagnosis or in terms of functional disability measures but rarely by their clinical syndromes. A point-prevalence study was conducted assessing 349 neurological inpatients to determine the frequency and co-occurrence of disabling neurological syndromes, considering a wider spectrum including pain, emotional, neuropsychological, vegetative and sensorimotor syndromes. Of the study patients, 61% ( n  = 224) had sensorimotor syndromes, 53% ( n  = 185) had neuropsychological disorders, 40% ( n  = 139) of the patients suffered from pain, emotional disorders were found in 36% ( n  = 122) and vegetative disorders in 33% ( n  = 113). Although frequency varied by neurological diagnosis, these disabling conditions were found across all inpatient groups of diagnosis. Similarly, disorders outside the motor domains grouped according to their Barthel Index showed a striking frequency in patients considered as activities of daily living independent, reflecting a wider spectrum of disability that functional measures are not able to capture. Of the study population, 68% ( n  = 237) suffered from co-occurring disorders from different categories (pain, emotional, neuropsychological, vegetative and sensorimotor syndromes). There is a high prevalence and co-occurrence of disabling syndromes in neurological inpatients. These proportions reflect the neurological workload in a patient population and should be considered in future rehabilitation research and allocation of resources.