Major Neurological Signs Research Articles

Autoimmune psychosis: a review of diagnostic and treatment guidelines

IntroductionOver this decade, there has been progressive growth and evolution of the concept of autoimmune encephalitis. However, international consensus overly focus on major neurological signs, while discarding some attenuated presentations, sometimes with just psychiatric manifestations. It was only very recently that a new concept arouse from this disorder, named autoimmune psychosis, which can mimic schizophrenia. Unfortunately, there is still a lack of a structured approach of psychotic patients to cover this disorder. This has numerous implications, namely on management and prognosis of these patients. Not only, these patients have an increased risk of neuroleptic malignant syndrome, but it is also important to intervene early in the course of disease.ObjectivesTo conduct a review of the diagnostic and treatment guideliens of autoimmune psychosisMethodsThe authors conducted a non-systematic review, by resorting to the pubmed database, on the concept of autoimmune psychosis and updated proposals of diagnostic orienting lines.ResultsRecently, in 2016, Graus et al proposed diagnostic criteria for possible autoimmune encephalitis, in which the authors acknowledge subacute onset of psychiatric symptoms as a possible clinical manifestation. The authors accept normal diagnostic tests, provided that new neurological focal findings exist. Since then, there have been described a list of signs/symptoms, which should raise suspicion for this diagnostic on psychiatric patients, so called red flags. In accordance to diagnostic guidelines for autoimmune psychosis, defined by Pollak et, the presence of this symptoms should lead clinicians to perform diagnostic exams, as MRI, eletroencephalogram and blood serological tests and lumbar puncture. However, others criticize this initial lineup arguing that some patients could be missed, because they do not have any neurological signs, and so they propose new diagnostic criteria.ConclusionsAutoimmune psychosis represents an attenuated clinical form of autoimmune encephalitis, although demanding the same medication and prompt initiation of treatment as other autoimmune encephalitis. It is important to acknowledge that there are patients who are seronegative and that some of the diagnostic exams mentioned have sometimes limited availability. As acknowledged by Guasp et al, there are patients with first psychotic episodes that have an autoimmune etiology, but because of the lack of neurological signs, could potentially be missed of treatment. So, it is important to establish formal diagnostic guidelines for this disorder, namely orienting lines for first psychotic episodes, which is the most commson psychiatric manifestation. This also enlightens the need for neurologic and psychiatric cooperation for these patients.Disclosure of InterestNone Declared

Hyperbaric oxygen therapy in carbon monoxide poisoning in Moroccan patients.

Describe the epidemiological, clinical characteristics of acute carbon monoxide poisoning (COP), and the therapeutic effect of hyperbaric oxygen therapy on patient's clinical outcome. This is the first study in this field in Morocco. It studies retrospectively 309 victims of acute COP with major neurological signs. All patients have been treated with hyperbaric oxygen therapy, and have been admitted by the emergency department of the Mohammed V military training hospital in Rabat, between January 2015 and December 2018. All poisonings were accidental and occurred especially in winter (50%), with a predominance in urban areas (93%). The poisoning was often caused by a non-compliant water heater (91%), multi-causality was in half of the cases, and affected adults and women (mean age: 21 ± 17years, gender (male:female) 1:1.5. The average admission time to the hyperbaric chamber lasted 9h 04min (± 12h 32min). Clinical signs were mainly unconsciousness (151 cases), headache (85 cases), vomiting (63 cases), nausea (53 cases), and dizziness (51 cases). All the patients benefitted from hyperbaric oxygen therapy sessions. The clinical outcome was positive in the majority of cases, but 23% of the cases presented minor or major sequelae. Patients with sequelae had a longer length of admission than those who had not. COP is a major public health problem in Morocco. Through this study, we suggest the interest of hyperbaric oxygen therapy, especially when it is administered timely without delay.

PB1763 CNS INVOLVEMENT AT PRESENTATION IN AML: A RETROSPECTIVE CASE SERIES

Background:Central nervous system involvement at present in Acute Myeloid Leukemia is uncommon. When it occurs, the course is less predictable and prognostication and therapy guidelines are less lucid than with acute lymphoblastic leukemia.Aims:To review the clinical profile, presentation, imaging findings, CSF positivity, management, and outcomes of AML patients with CNS involvement at the time of initial presentation.Methods:We retrospectively collected the clinicopathological profile, management and outcome data of AML patients with CNS involvement over the past 5 years at our Institute. Patients with CNS involvement by CSF positivity or direct CNS or spinal cord involvement were included. It is our policy to perform CNS evaluation at presentation in all Pediatric AML or with symptoms suggestive of CNS involvement e.g. paraparesis, seizures, proptosis.Patients with paraspinal masses causing cord compressions, intra cranial bleed, intraretinal bleed, CNS involvement at relapse, or possible CNS involvement but without either CSF or imaging confirmation were excluded.Results:Eight patients fulfilled our inclusion criteria. Of these 4 were adults and 4 were pediatric patients (<18 years age). Only 1 (12.5%) amongst these was a female. Cytogenetics were t(8:21), t(8:21) +8, t(8:21) +8 +19, AML with t(9:22) in 1 patient each with rest 50% patients having normal cytogenetics.CSF analysis was positive for malignant cells in 3 patients, not done in 1 (because patient was very sick) and negative in the rest. Imaging was not done in 2 patients who were CSF positive with no focal neurological deficits, and was normal in 1 patient with 3rd cranial nerve palsy. Leptomeningeal involvement was present in 2 patients, cavernous sinus and falx cerebri involvement in 1, paravertebral deposits and cord enhancement in 1, and paravertebral and infratemporal deposits in another.All patients were treated with 3+7 induction followed by HIDAC with IT therapy with each cycle and 2 patients received RT as well. Ph positive AML was taken up for transplant after 2nd induction with HIDAC and is currently undergoing the same. 1 patient was not given chemotherapy because she had severe fungal pneumonia and expired.Six out of the 7 treated patients showed near complete resolution of CNS symptoms/imaging findings after chemotherapy. 1 patient with paraplegia had persisting spinal cord lesions despite 2 induction chemotherapies, IT and RT. Four patients relapsed – one CNS relapse, two CNS and BM relapse and 1 patient had BM relapse in the background of persisting CNS disease. Currently only 3 patients are alive, one of whom is undergoing Allogenic HSCT.Summary/Conclusion:CNS involvement in AML is rare at presentation. Presentation can vary from asymptomatic CSF positivity, to isolated cranial nerve involvement to paraplegia and recurrent seizures. Screening and Management protocols are not clearly defined. NCCN recommends screening before first consolidation in patients with monocytic differentiation, Mixed phenotypic acute leukemia, WBC >40000at diagnosis, extra medullary disease or high risk APML. For major neurological signs imaging should be performed. If no signs or symptoms then CSF should be done. Treatment with standard AML chemotherapy with IT/RT seems to have short term effects only. Overall prognosis in this group of patients seems poor with a high rate of relapse and mortality. Larger studies are required to address relevant questions such as high risk patients who should be screened, indications of performing CSF analysis and imaging, and adequate management.image

Brain morphometry of preschool age children affected by autism spectrum disorder: Correlation with clinical findings.

The aim of our study was to use a combined imaging and clinical approach to identify possible patterns of clinical and imaging findings in a cohort of preschool age autism spectrum disorder (ASD) patients. In order to identify imaging patterns that could be related to specific clinical features, a selected group of ASD patients (age range 3-6 years) without dysmorphic features, epilepsy or other major neurological signs, malformations or other lesions at MRI was subjected to brain volumetric analysis using semiautomatic brain segmentation. An age-matched group of typically developing children was subjected to the same analysis. Our results were consistent with previous literature: Total gray matter volume, total cortical gray matter volume and amygdalar volumes were significantly greater in the ASD group than the control group. When we divided the study group into subgroups on the basis of clinical findings such as high- or low-functioning, or verbal and nonverbal, the only significant difference between verbal and nonverbal subjects was in cerebellar hemispheric size. In conclusions, our results confirm that newer brain MRI techniques using semiautomatic brain segmentation can provide information useful for defining the differences between ASD patients and controls, particularly if they form part of an integrated approach between MRI and cognitive-behavioral and genetic data. Clin. Anat. 32:143-150, 2019. © 2018 Wiley Periodicals, Inc. HIGHLIGHTS: Combined imaging and clinical approach in autism spectrum disorders Semiautomatic brain segmentation in a selected preschool age ASD group Reduced total cerebellar white matter volume in non-verbal ASD patients.

Corpus callosum agenesis and interhemispheric cysts: Epileptic evaluation and long-term outcome in 9 children

Objective: To describe perinatal history, clinical phenotype, MR features, neurological outcome, and EEG patterns of pediatric patients with Corpus Callosum Agenesis (CCAg) and Interhemispheric Cysts. Methods: We selected 9 patients (8 males, mean age 7,3 +/−5,7) with CCAg (partial or complete) and interemipheric cysts from our imaging database of 378 patients with midline brain anomalies scanned at Gaslini Children Hospital from Jan 2005 to Jan 2017. Interemispheric cysts were grouped according to the 2001 Barkovich classification. Presence and location of gray matter anomalies such as polymichrogyria (PMG) or nodular heterotopia (NH) were recorded. Perinatal, clinical, genetic testing (when available), EEG pattern, age of onset of seizure (if present) with drug responsivity were reported. Results: Complete CCAg was present in 6 individuals, while the remaining cases had partial CCAg. Type1 cysts were present in 1 patient (subtype1a). Type 2 cysts were 8 (2 subtype2a, 2 subtype2b, 1 subtype2c and 1 subtype2d). Of interest, 2 patients had an MRI pattern overlapping between subtype2b and 2c (transmanterllar NH, diffuse PMG, macrocephaly, developmental delay). EEG epileptic activity was present in all but epilepsy onset was observed in 3/9 cases, with drug response in all. Major neurological defects were excluded. Developmental delay was observed in all cases but cognitive outcome, registered for 6/9 patients, was milder: 3/6 normal IQ, 2/6 bordeline and 1 with mild Intellectual Disability. Psychiatric data were recorded for 7/9 patients: hyperactivity and anxiety were frequent comorbidities (5/7). Conclusions: CCAg associated with Intehemispheric Cysts may be associated with a good neurological outcome with borderline or normal cognition and no major neurological signs in the majority of patients. Despite the presence of EEG anomalies, the occurrence of epilepsy in these cases is rare and usually responsive to antiepileptic drugs.

Is There an Association Between Magnetic Resonance Imaging and Neurological Signs in Patients With Vertebral Osteomyelitis?: A Retrospective Observational Study on 121 Patients.

Neurological complications can occur in up to 51% of vertebral osteomyelitis (VO) in surgical series. The aim of our study was to estimate the frequency of neurological signs in a nonselected population of patients with VO and to assess clinical and MRI changes associated with these complications.We reviewed medical charts of patients with VO from 2007 to 2014 in our University Hospital and their MRIs were analyzed by a radiologist blinded from clinical data. Neurological status was defined as follow: normal, minor signs (radiculalgia or sensory loss), and major signs (motor deficit and/or sphincter dysfunction).A total of 121 patients were included. Mean age was 64.3 years. Overall, 50 patients (40%) had neurological signs, 26 were major signs (21.5%). Neurological signs were present at the time of admission in 37 patients and happened secondarily in 13 cases. MRI changes associated with major neurological signs were: Cervical involvement (P = 0.011), dural sac compression (P = 0.0012), ventral effacement of the subarachnoidal space (P < 0.001), compressive myelopathy (P = 0.006). More than 50% of the vertebral body destruction (P = 0.017), angular kyphosis (P = 0.016) partial or complete destruction of posterior arch (P = 0.032) were also associated with these signs. Neither epidural abscesses, multifocal lesions, loss of disk height, nor nerve roots compression were associated with major neurological signs.Neurological signs occurred in 40% of our patients with one half being major signs. Cervical involvement, vertebral destruction, angular kyphosis, dural compression, effacement of subarachnoid space and compressive myelopathy on MRI were risk factors associated with neurological complications.

Late-onset multiple sclerosis presenting with cognitive dysfunction and severe cortical/infratentorial atrophy

Objective: Although cognitive dysfunction is a relevant aspect of multiple sclerosis (MS) from the earliest disease phase, cognitive onset is unusual thus jeopardizing early and accurate diagnosis. Here we describe 12 patients presenting with cognitive dysfunction as primary manifestation of MS with either mild or no impairment in non-cognitive neurological domains. Methods: Twelve patients with cognitive onset who were subsequently diagnosed with MS (CI-MS) were included in this retrospective study. Twelve cognitively normal MS patients (CN-MS), 12 healthy controls and four patients having progressive supranuclear palsy (PSP) served as the reference population. Results: Ten CI-MS patients had progressive clinical course and all patients had late disease onset (median age = 49 years; range = 40–58 years). Among cognitive functions, frontal domains were the most involved. Compared to CN-MS and healthy controls, significant cortical and infratentorial atrophy characterized CI-MS patients. Selective atrophy of midbrain tegmentum with relative sparing of pons, known as “The Hummingbird sign,” was observed in eight CI-MS and in three PSP patients. Discussion: Our observation suggests that MS diagnosis should be taken into consideration in case of cognitive dysfunction, particularly when associated with slowly progressive disease course and severe cortical, cerebellar and brainstem atrophy even in the absence of other major neurological symptoms and signs.

Brain Tumor Presenting With Psychiatric Symptoms

Back to table of contents Previous article Next article LettersFull AccessBrain Tumor Presenting With Psychiatric SymptomsOzdilek Betul, M.D., and Midi Ipek, M.D.Ozdilek BetulSearch for more papers by this author, M.D., and Midi IpekSearch for more papers by this author, M.D.Published Online:1 Oct 2011https://doi.org/10.1176/jnp.23.4.jnpe43AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Brain tumors may present with multiple psychiatric symptoms, such as depression, anxiety disorders, panic attacks, personality change, abulia, auditory and visual hallucinations, mania, or memory difficulties. In the present study, we discuss a case of a 50-year-old man who was treated for 7 years for anxiety disorder and depression but did not respond to treatment. At that period, he did not develop any major neurological signs or symptoms. When he complained of headache, he was examined by a neurologist, and MRI scans (MRIs) were performed. MRIs of the brain revealed a glial tumor in the left temporal lobe, and he underwent surgical treatment and subsequently received chemotherapy and radiotherapy.We conclude that brain tumors can be neurologically silent and only present with psychiatric symptoms. Therefore, we emphasize the need for brain imaging studies in a patient with resistance to psychiatric drugs and without neurological signs or symptoms.Case ReportDuring the previous 7 years, a 50-year-old man had been treated in various psychiatric departments for recurrent depressive and anxiety disorders several times. He continued individual psychotherapy with his psychologist and was tried on several psychotropic agents, with poor response. His symptoms did not improve. He was prescribed paroxetine 30 mg/day and mirtazapine 30 mg /day after his last visit. When he complained of persistent headache, he was referred to a neurologist, and his neurological examination did not show any abnormal signs; MRIs were then performed. A contrast-enhanced MRI of the brain revealed a glial tumor in the left temporal lobe involving limbic structures and the anterior part of the corpus callosum (Figure 1). The patient underwent osteoplastic craniotomy, with partial resection of the mass. Histopathology confirmed an anaplastic astrocytoma Grade III. He underwent chemotherapy and radiotherapy, and his condition improved.FIGURE 1. Tumor in the Left Temporal Lobe Involving Limbic Structures and Anterior Part of the Corpus Callosum Seen in the Axial and Coronal Sections of Brain MRI With and Without ContrastDiscussionBrain tumors can be associated with a variety of psychiatric symptoms, such as anxiety disorders, panic attacks, personality changes, mania, psychosis, apathy/abulia and cognitive deterioration, without any physical or neurological signs.1,2This case illustrates the need for prompt assessment for brain imaging when patients present with any atypical psychiatric symptoms or changes in the mental state. Our patient was treated in psychiatric services for 7 years before the brain tumor was diagnosed. It is unclear in our case whether his psychiatric symptoms were of functional origin or were caused by the brain tumor. It is possible that an MRI/computerized tomography scan with contrast may have detected the mass.3 A thorough neurological evaluation is important to assist in the diagnosis. Atypicality of presentation, poor response to treatment, or waxing and waning of symptoms should lead to suspicion of organic etiology.When brain tumors develop in patients with established psychiatric disorders, detailed history, neurological examination, and brain imaging become essential, as psychiatric patients are known to have difficulties in reporting and describing their own symptoms.4 It is recommended that any patient over 40 years of age with a change in neurobehavioral status should have neuroimaging of the brain.4 The same applies to patients with psychiatric presentations accompanied by specific neurologic or neurobehavioral changes or with poor response to psychopharmacologic treatment. We recommend that any change in the clinical presentation of patients with well-established psychiatric histories or the emergence of atypical psychiatric symptoms should be further explored through neuroimaging.Marmara University Hospital Department of Neurology Istanbul-Turkeye-mail: [email protected]com1. Moise D , Madhusoodanan S : Psychiatric symptoms associated with brain tumors: a clinical enigma. CNS Spectr 2006; 11:28–31Crossref, Medline, Google Scholar2. Madhusoodanan S , Danan D , Moise D : Psychiatric manifestations of brain tumors: diagnostic implications. Expert Rev Neurother 2007; 7:343–349Crossref, Medline, Google Scholar3. Hollister LE , Boutros N : Clinical use of CT and MR scans in psychiatric patients. J Psychiatry Neurosci 1991; 16:194–198Medline, Google Scholar4. Madhusoodanan S , Danan D , Brenner R , et al.: Brain tumor and psychiatric manifestations: a case report and brief review. Ann Clin Psychiatry 2004; 16:111–113Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited ByCascading handcrafted features and Convolutional Neural Network for IoT-enabled brain tumor segmentationComputer Communications, Vol. 153The Dorsolateral Prefrontal Cortex, the Apathetic Syndrome, and Free Will21 May 2019 | Activitas Nervosa Superior, Vol. 61, No. 3 Volume 23Issue 4 Fall 2011Pages E43-E44 Metrics PDF download History Published online 1 October 2011 Published in print 1 October 2011

Brain Tumor Presenting With Psychiatric Symptoms

Brain Tumor Presenting With Psychiatric Symptoms

Evidence of a non-progressive course of alternating hemiplegia of childhood: study of a large cohort of children and adults

Alternating hemiplegia of childhood is a neurological disorder characterized by episodes of hemiplegia, various non-epileptic paroxysmal events and global neurological impairment. Characterization of the evolution and outcome into adulthood has not been sufficiently investigated. The goal of this study was to elucidate the natural history of alternating hemiplegia within a large cohort of 157 patients, as part of the European Network for Research on Alternating Hemiplegia project. A questionnaire was formulated to determine the severity of both paroxysmal and global neurological impairment and address progression of the disorder by allocating data to specific age epochs up to and over 24 years of age. Patients in early age groups were consistently present in subsequent later age groups and for each patient, data were collected for each corresponding age epoch. The study was based on predominantly retrospective and, for a period of 2 years, prospective data. At inclusion, patients were aged from 9 months to 52 years. The median age at diagnosis was 20 months. All patients experienced hemiplegic attacks; 86.5% reported episodes of bilateral weakness, 88% dystonic attacks, 53% epileptic seizures, 72% developed chorea and/or dystonia and 92% mental retardation. When data over the course of the illness were examined for the whole cohort, the severity of symptoms did not appear to change, with the exception of abnormal ocular movements and hypotonia that regressed, but did not disappear into adulthood (from 86 to 36% and 76 to 36%, respectively). No statistically significant correlation between a history of severe paroxysmal hemiplegic/dystonic episodes and a worse neurological outcome was identified. Seven patients died, some of whom experienced severe plegic attacks or epileptic seizures at the time of death. History of severe plegic/dystonic attacks was not found to be an aggravating factor for deceased patients. Our results provide evidence that the natural history of alternating hemiplegia is highly variable and unpredictable for individual patients. However, we did not find evidence to support a steadily progressive and degenerative course of the disorder when patients were analysed as a group. For a minority of patients, a risk of sudden death was associated with more severe neurological impairment. The European Network for Research on Alternating Hemiplegia Registry, validated by our study, includes all major neurological signs and symptoms of alternating hemiplegia and may thus be used as a precedent for the progressive inclusion and follow-up of patients as well as a reference for genetic studies and treatment trials.

Experiences of Borna Disease Virus infection in Sweden

In the early 1970s a fatal neurological disorder in cats was reported in the areas around Lake Mälaren in central Sweden. The major signs were hind-leg ataxia, as well as absence or marked decrease in postural reactions and in some cases behavioural changes. The pathology of the disorder was characterized as a non-suppurative meningoencephalomyelitis, but the etiology was not determined. Almost twenty years later, the disorder now known as staggering disease (SD), was further characterized both clinically and pathologically. The same histopathological picture was seen as in the previous study, with inflammatory nodules, neuronal degeneration and perivascular cuffs mainly consisting of lymphocytes. The most severe inflammatory changes were seen in the grey matter of the brain stem, basal ganglia and hippocampus. Clinically the same major neurological signs were seen. Although the cats were examined for several known infectious agents causing central nervous system (CNS) disturbances, no etiological cause of SD was determined.

Evolution of Neurologic Features in Williams Syndrome

As a part of a large multidisciplinary clinical and research follow-up study, 47 Williams syndrome patients underwent detailed neurologic testing. Because previous studies have documented the absence of major neurologic signs in Williams syndrome, the neurologic testing focused on soft signs. Previous findings of impairment of both gross and fine motor coordination were confirmed, and the presence of mild cerebellar and extrapyramidal signs was documented. In a 4-year follow-up study, an age-related pattern was revealed: soft extrapyramidal signs became more evident from 8 years of age and increased in the 14+ age group. The results are discussed according to a hypothesis related to the dopaminergic system involvement in Williams syndrome: anomalous organization or accelerated ageing process.

Influence of tumor site on cognitive deficits in children with cerebellar tumors at diagnosis

9045 Background: Children with cerebellar tumor are at risk for cognitive deficits (CD) depending on surgery and radiotherapy. Only few studies analyze the role of tumor itself in mental functioning (Ellenberg et al., ‘87). Data about neuropsychological organization before any treatment are essential to understand the effect of tumor and have a baseline for analyzing the negative impact of the different treatments in the CD. Aim of this study is to prospectively analyze cognitive functions before treatment in patients (pts) with cerebellar tumors. Methods: Twenty-five pts with cerebellar tumor were assessed at diagnosis.Children with previous and severe neurological disturbances neurological were excluded. Intelligence quotient (IQ) and sectorial cognitive abilities (memory, attention, language, visuospatial and executive functions) were evaluated. Neurological examination (BUSPAR) and magnetic resonance imaging (MRI) were performed in the same period of cognitive assessment. Neurological deficits were classified as major, mild or absent in relation with the results of BUSPAR. Results: Twenty pts were selected; males/females: 12/8; age: 7.6 years (range: 18 m-14.8 y); histhology: pilocytic astrocytoma (9 pts), medulloblastoma (9), ependymoma (1) and atypical teratoid-rabdoid (1); tumor location: right cerebellar hemisphere (4), left (4), vermal (12); neurological examination: major neurological signs (2 pts), mild (10), absent (6); hydrocephalus: 50% of pts. Three pts had IQ values below the average level, although mean IQ values were normal (mean: 99.6; range: 78–118). Sixteen/20 pts had selective CD mainly involving working memory, executive functions, attention, and visual motor integration. Language processing was defective in 6 pts (2/4 right-sides lesions, 4/12 vermal lesion). Conclusions: Sectorial CD are present before treatment in about 80% of pts, mainly related to the location of tumor. Preliminar data suggest a correlation between specific sites inside cerebellum and selective CD, with language problems mainly in right hemispheric tumor. Complex cognitive impairment was present in 15% of pts before treatment. These data will represent the baseline for further analysis about the impact of treatment on cognitive outcome. No significant financial relationships to disclose.

Psychiatric Symptoms Associated with Brain Tumors: A Clinical Enigma

Patients in psychiatric settings may present with medical conditions, such as brain tumors, which may or may not be associated with neurological symptoms. In some cases, patients may only have psychiatric symptoms, such as mood changes (depression or mania), psychotic symptoms, panic attacks, changes in personality, or memory difficulties. Brain tumors may be detected in patients at their first presentation to mental health services or sometimes in patients with well-established psychiatric diagnoses. This article presents the case of a 29-year-old woman who was treated for >4 years for posttraumatic stress disorder and borderline personality traits, who developed depressive symptoms and memory difficulties. However, she did not develop any major neurological signs or symptoms. Brain imaging showed the presence of a left thalamic tumor, later confirmed as glioblastoma multiforme. She underwent surgical treatment and radiation therapy. With this we show that in some cases, brain tumors can be neurologically silent and only present atypical psychiatric symptoms. We emphasize the need for neuroimaging studies in a patient with atypical changes in mental status, even without neurological signs or symptoms.

Restricted pain and thermal sensory loss in a patient with pontine lacunar infarction: a clinical MRI study

Pure sensory syndrome (PSS) is characterized by hemisensory symptoms without other major neurological signs. It was initially attributed to thalamic lacunar infarction, but several reports have shown the PSS can be due to small infarcts involving the posterior part of the internal capsula, the cerebral cortex and the brainstem. Paramedian and lateral pontine infarctions are associated respectively with lemniscal and spinothalmic (ST) sensory impairment. We describe a patient with an isolated impairment of the ST modalities caused by a segmental paramedian pontine infarction.

Cognitive development in low risk preterm infants at 3–4 years of life

Background: Major neurological handicaps and neuropsychological disturbances are more common in ex-preterm children than their counterparts born at term. Objective: To establish in a prospective study whether a characteristic neuropsychological...

Neuropsychological deficits in children with brain tumors at diagnosis: influence of tumor site and tumor-related factors

1571 Background Children with Central Nervous System (CNS) tumors are frequently affected by neurocognitive sequelae which deeply influence the quality of life. Surgery, radiotherapy and chemotherapy have been indicated as causes of neurocognitive impairment. We have investigated the role of tumor location and tumor-related factors on neuropsychological deficits before any therapy or surgical intervention in order to obtain baseline values useful to evaluate possible neuropsychological sequelae of subsequent treatments. Methods 54 children with brain tumors were evaluated early after radiological diagnosis. Children with previous neurological disturbances and with severe neurological conditions were excluded. Age at diagnosis: 8,3 y (range: 5m - 18 y); Males/ females: 3/2; Tumor location: posterior fossa: 38,6%; supratentorial: 15,9%; cerebral hemispheres: 45,5%; hydrocephalus: 31,8%. Early signs or symptoms of the tumor and the duration of clinical manifestations at diagnosis were investigated. We measured: total intelligence quotient (TIQ), verbal intelligence quotient (VIQ) and performance intelligence quotient (PIQ); language comprehension and production; short and long term verbal and visual memory; visual motor integration abilities; attention; frontal functions; lateralization. Results Mean IQ values were within the average (TIQ: 97,71 +/- 17,02; VIQ: 103,15 +/- 16,69; PIQ: 95,54+/-15,62). However 22,7% of children had IQ values lower than 85 and half of them were mentally retarded. 80% of them had cortical tumors involving temporo-mesial structures. Lower values of IQ were associated with long lasting clinical signs. Specific cognitive deficits were present in 65,8%, frequently mild and coherent with tumor site. Major neurological signs were related to deficits in memory span; no correlation was found between neurocognitive performances and hydrocephalus. Conclusions Cognitive deficits are already present at the time of diagnosis in a relatevely high percentage and are mainly related to tumor site; children with cortical tumor, mainly in temporal lobes, are at higher risk for severe alterations of cognitive development. (Supported by FOP; Ali Di Scorta) No significant financial relationships to disclose.

Antibodies directed against nitrosylated neoepitopes in sera of patients with human African trypanosomiasis.

Antibodies directed against nitrosylated epitopes have been found in sera from patients suffering from human African trypanosomiasis (HAT) but not in sera from control subjects living in the same endemic area or African control subjects living in France. We conjugated amino acids to albumin by glutaraldehyde (conjugates) and then nitrosylated the conjugates. Both conjugates and nitrosylated conjugates were analysed by enzyme-linked immunosorbent assay (ELISA). We detected antibodies directed against nitrosylated L-cysteine and L-tyrosine conjugates; antibody levels were higher in stage II patients than in stage I. Patients with severe clinical signs had higher antibody levels, and antibody levels were highest in patients with major neurological signs. Antibody response was only associated with the IgM isotype. We evaluated antibody specificity and avidity by competition experiments using conjugates and nitrosylated conjugates. Avidity was around 2 x10(-6) m for the S-nitroso-cysteine epitope and 2 x 10(-8) m for the S-nitroso-tyrosine epitope. Detection of circulating antibodies to S-nitroso-cysteine and S-nitroso-tyrosine epitopes provides indirect evidence for nitric oxide (NO) involvement in HAT and their levels are correlated with disease severity.

Intradiploic epidermoid cysts of the skull: a report of four cases

Epidermoid cysts are uncommon, benign and slow-growing lesions. They may often reach an enormous size without producing neurological symptoms. Intradiploic epidermoid cysts are nearly 25% of all epidermoids. They are derived from ectodermal remnants that stay within the cranial bones during embryonic development. Intradiploic epidermoid cysts can be located in any part of the skull. Two of our four cases were located in left occipital bone and the others were in the frontal bone. These tumours can occur at any age from the first to the seventh decade of life. Our cases had a mean age of 38;75 years (19–55 years). They may reach great sizes before the initial diagnosis and may produce major neurological signs. For good long-term prognosis, correct radiological assessment and complete removal of the tumour with its capsule are essential. We report four cases of intradiploic epidermoid cysts of the skull and analyse the clinical, radiological features and treatment of these lesions in the light of relevant literature.

Neurodevelopmental Investigations among Methylmercury-Exposed Children in French Guiana

French Guiana, like its neighbors, suffers from environmental pollution with methylmercury from gold mining activities, and Amerindian communities are particularly affected. A neurological and a neurospsychological evaluation were carried out in children of three Amerindian communities with various levels of pollution: 156 children from the Upper Maroni (high exposure), 69 from Camopi on the Oyapock river (median exposure), and 153 from Awala on the Atlantic coast (low exposure). Exposure to methylmercury was measured by determination of total mercury in the hair of the children and their mothers (geometric mean, 12.7 μg/g in Upper Maroni). No major neurologic signs were observed in the children examined. After adjustment for potential confounders, we found a dose-dependent association between maternal hair mercury level and increased deep tendon reflexes, poorer coordination of the legs, and decreased performance in the Stanford–Binet Copying score, which measures visuospatial organization. In this last test, the frequency of rotation errors was high in the 5–6 years age group and increased with mercury exposure. These associations depended on the sex of child and were stronger among boys. The interpretation of these results is limited mainly by the cross-sectional design of the study. It identifies specific neurological and neuropsychological deficits, in some cases modulated by sex, which are consistent with known targets of mercury neurotoxicity.