Perseverative error in schizophrenia: correlation with cortical blood flow by SPECT.

INTRODUCTION: Perseverative error (PE) is a core symptom of schizophrenia which has been proposed as a phenotypic marker of the illness. Moreover, hypofrontality observed in functional neuroimaging studies while executing a cognitive task has also been suggested as a characteristic sign of schizophrenia. We propose combining symptom and sign to demonstrate the existence of a regional cortical blood flow (RCBF) pattern associated to PE that might constitute a biological marker of schizophrenia. MATERIAL AND METHOD: We used Single Photon Emission Computerized Tomography (SPECT), to study the RCBF associated to PE and to correct response (CR), during the execution of the Wisconsin Card Sorting Test (WCST), of 18 patients with schizophrenia and 13 controls. We focused on five well-defined bilateral brain regions, using the RCBF of the same regions at rest as a baseline. RESULTS: Patients made more PE than controls in the WCST. Among patients, we observed a correlation between PEs and right occipital RCBF. Among controls, we found a negative correlation between PEs and left temporal cortex RCBF and a positive correlation between CRs and left frontobasal and overall left frontal cortexes RCBF. CONCLUSIONS: The severity of PE is associated to higher right parietal-occipital activity in patients with schizophrenia. CR in the WCST are associated to higher left frontal activity in controls but not in patients. Probably, there is a RCBF redistribution pattern related to the typical perseveration of schizophrenia which might constitute a phenotypic marker of the illness observable by functional neuroimaging techniques.

El error perseverativo en la esquizofrenia: correlación con el flujo sanguíneo cortical mediante SPECT / Perseverative error in schizophrenia: correlation with cortical blood flow by SPECT

Introducción. El error perseverativo (perseverative error, PE) es un síntoma característico de la esquizofrenia que ha sido propuesto como marcador fenotípico de la enfermedad. Junto a ello, la hipofrontalidad observada mediante neuroimagen funcional durante la ejecución de una prueba cognitiva ha sido igualmente sugerida como signo característico de la esquizofrenia. Nos proponemos combinar síntoma y signo para demostrar la existencia de un patrón de flujo sanguíneo cortical relativo (relative cortical blood flow, RCBF) asociado al PE, lo que podría constituir un marcador biológico de la esquizofrenia. Material y métodos. Mediante tomografía computarizada por emisión de fotón único (SPECT) estudiamos el patrón de RCBF asociado al PE y a la respuesta correcta (Correct Response, CR) del Test de Ordenación de Cartas de Wisconsin (Wisconsin Card Sorting Test, WCST) en 18 pacientes con esquizofrenia y 13 controles. Nos centramos en cinco regiones cerebrales bien definidas bilateralmente, utilizando como línea de base el RCBF de dichas regiones en reposo. Resultados. Los pacientes cometieron más PE que los controles en el WCST. En los pacientes observamos una correlación entre PE y RCBF de la corteza occipital derecha. En los controles encontramos una correlación negativa entre PE y RCBF de la corteza temporal izquierda y una correlación positiva entre CR y RCBF de las cortezas frontobasal izquierda y frontal global izquierda. Conclusiones. La severidad del PE se asocia a una mayor actividad parietooccipital derecha en pacientes con esquizofrenia. La CR del WCST se asocia a mayor actividad frontal izquierda en controles, pero no en pacientes. Probablemente existe una redistribución del RCBF relacionada con la perseveración típica de la esquizofrenia, lo que podría constituir un marcador fenotípico de la enfermedad observable mediante técnicas de neuroimagen funcional

Introduction. Perseverative error (PE) is a core symptom of schizophrenia which has been proposed as a phenotypic marker of the illness. Moreover, hypofrontality observed in functional neuroimaging studies while executing a cognitive task has also been suggested as a characteristic sign of schizophrenia. We propose combining symptom and sign to demonstrate the existence of a regional cortical blood flow (RCBF) pattern associated to PE that might constitute a biological marker of schizophrenia. Material and method. We used Single Photon Emission Computerized Tomography (SPECT), to study the RCBF associated to PE and to correct response (CR), during the execution of the Wisconsin Card Sorting Test (WCST), of 18 patients with schizophrenia and 13 controls. We focused on five well-defined bilateral brain regions, using the RCBF of the same regions at rest as a baseline. Results. Patients made more PE than controls in the WCST. Among patients, we observed a correlation between PEs and right occipital RCBF. Among controls, we found a negative correlation between PEs and left temporal cortex RCBF and a positive correlation between CRs and left frontobasal and overall left frontal cortexes RCBF. Conclusions. The severity of PE is associated to higher right parietal-occipital activity in patients with schizophrenia. CR in the WCST are associated to higher left frontal activity in controls but not in patients. Probably, there is a RCBF redistribution pattern related to the typical perseveration of schizophrenia which might constitute a phenotypic marker of the illness observable by functional neuroimaging techniques

Basal ganglia volumetric studies in affective disorder: what did we learn in the last 15 years?

Until today, morphometric neuroimaging studies on affective disorders concentrate on the limbic system, especially the hippocampus, amygdala, and anterior cingulate. In most of the studies and reviews available today, the basal ganglia are of secondary interest. It seems that the basal ganglia are interest of neurologist, whereas the limbic system is reserved for psychiatric neuroimaging studies. We follow a different approach in this review, studying all available papers on MRI research of the basal ganglia in unipolar depression and bipolar disorder. We found a possibly larger neostriatum in bipolar and possibly smaller one in unipolar patients. None of the unipolar studies found any larger basal ganglion, and only one out of 12 bipolar studies found smaller basal ganglia. Both findings seemed to depend on age (tendency toward smaller volumes in unipolar and bipolar with older age), sex (men tending to pathology in both disorders) and bipolar patients show a possible influence of medication, which is not assessed so far in unipolar depression. We conclude that several methodological shortcomings in volumetric MRI research on the basal ganglia in affective disorders make it necessary to imply more research in this area. We suggest (a) better MRI methods (we do not have a single volumetric 3 Tesla study in this patient group); (b) studies of medication-naïve patients (thus ruling out the medication effect); (c) Studies that directly compare unipolar depressed and bipolar patients are needed to determine whether these apparent differences in morphometric abnormalities, as observed through the mediating comparison with healthy subjects, are real.

[Mild cognitive impairment: A review of the literature]. / Die leichte kognitive Störung. Vergesslichkeit im Alter--eine Vorstufe der Demenz?

People with MCI suffer from a moderate memory impairment, from deficits in attention and cognitive flexibility but do not fulfil the diagnostic criteria of dementia. These patients feel forgetful, and sometimes may have problems with their jobs and families because of their forgetfulness. In many cases (10-15% per year), MCI is leading to Alzheimer's disease. The sooner MCI is diagnosed and treated, the better the transition into Alzheimer's disease may perhaps be delayed. The diagnostic instruments are the same as for the diagnosis of Alzheimer's disease: biomarkers of the cerebrospinal fluid, neuroimaging and neuropsychological testing. Neuropsychological testing already shows deficits, even when neuroimaging findings still seem to be normal. Therapy of MCI is basically like the treatment of Alzheimer's disease: symptomatic treatment with cholinesterase-inhibitors or antioxidants, and compensatory psychological approaches.

Sexuality in multiple sclerosis.

Sexuality and partnership have an important influence on the quality of life of every person and also on people with chronic disorders such as multiple sclerosis. The findings in literature show high evidence that people with multiple sclerosis experience high levels of sexual dysfunction, most of them with hypoactive sexual behaviour often associated with dissatisfaction in relationship, and also the partners seem to show lower sexual and partnership satisfaction. The most common problems in women are lack of sexual interest and decreased libido, often with problems in orgasmic capacity, while men report erectile dysfunction and also lack of sexual interest. The impact of the level of disability and duration of the illness remains unclear. Positive familial support can often help the patient in coping with the illness, nonetheless problems with changing roles and multiple-sclerosis-minimizing can improve the need of contacts to outstanding persons.

Normal striatal D2 receptor binding in idiopathic restless legs syndrome with periodic leg movements in sleep.

Dopaminergic treatment is very effective in restless legs syndrome (RLS) and periodic leg movements in sleep (PLMS). However, neuroreceptor imaging studies that addressed altered striatal dopaminergic function have given controversial results. In this present study, 14 patients with idiopathic RLS (iRLS) and PLMS with a good response to dopaminergic and non-dopaminergic treatment and ten healthy sex- and age-matched controls were investigated off-medication by using 123I-IBZM and SPECT. RLS symptoms and sleep disturbances were evaluated using three nights of polysomnography, the Pittsburgh Sleep Quality Index, and the International RLS Study Group (IRLSSG) rating scale. The patients presented with sleep disturbances, a high PLMS index (56.2 +/- 33.1 per h), and severe RLS symptoms during SPECT (IRLSSG rating scale 23.1 +/- 8.0), and showed no significant differences in striatal to frontal IBZM binding to D2 receptors compared to controls (ratio striatum/frontal cortex, right side 1.60 +/- 0.10 vs 1.63 +/- 0.08, P = 0.35, NS; left side 1.61 +/- 0.11 vs 1.63 +/- 0.08, P = 0.51, NS). These findings show normal function of striatal D2 receptors in successfully treated patients with iRLS and PLMS. Dopaminergic and non-dopaminergic pretreatment does not appear to change striatal D2 receptor binding as compared to healthy controls. Structures other than striatal D2 receptors are discussed as possible causes of the treatment effects in RLS.

[Primary high-grade B-cell lymphoma of the CNS. Case report and review of the literature]. / Primäres hochmalignes B-Zell-Lymphom des ZNS. Fallpräsentation und Literaturübersicht.

We report the case of a 69-year-old patient referred to our clinic because of mania. When examined by neuroradiological imaging, there were lesions seen appearing and disappearing in different regions of the brain during a period of 2 months. Differential diagnosis of these changing lesions, progressive severe illness, and the role of glucocorticoid therapy concerning these lesions are discussed. The diagnosis of primary CNS lymphoma of the B-cell type could not made sure until autopsy.

Apraxia of eyelid closure in Huntington's disease.

We report a patient with genetically confirmed Huntington's disease (HD) presenting apraxia of eyelid closure (AEC). She was unable to close her eyes at command but was able to blink. Chorea and AEC ameliorated significantly during treatment with olanzapine and riluzole, an inhibitor of glutamate release. AEC is reported in progressive supranuclear palsy, Creutzfeldt-Jakob's disease, amyotrophic lateral sclerosis, and as post-stroke AEC. No report on HD is available so far, although oculomotor disturbances are quite common in this disease.

High-dose olanzapine in Huntington's disease.

The few reports available on olanzapine in Huntington's disease (HD) are insufficiently documented and/or insufficiently dosed. We describe a 30-year-old woman with genetically confirmed HD who presented with severe chorea. She was not able to eat or dress without help and did not respond to haloperidol; the motor scale of the Unified HD Rating Scale (UHDRS-I) revealed 65 of a possible 124 points. After admission, we treated the patient with a high dose of olanzapine (30 mg daily). The chorea almost ceased in the next 2 days, she was able to eat and walk without assistance (UHDRS-I of 21 points), and fine motor tasks improved, as well as gait and eye movements. This effect lasted for 5 months. We conclude that high-dose olanzapine appears to be useful in grave choreatic attacks.

[Adrenoleukodystrophy mimicking multiple sclerosis]. / Adrenoleukodystrophie kann Multiple Sklerose imitieren.

The article describes the development of symptoms in a 40-year-old female patient who is a symptomatic carrier of X-linked adrenoleucodystrophy (ALD). ALD is characterized by impaired peroxisomal beta-oxidation of very long chain fatty acids and is associated with mutations of the ALD gene, resulting in a defective peroxisomal membrane-transport protein. Our patient's symptoms are identical to those found in multiple sclerosis, showing spastic paraparesis of the lower limbs with marked sensory deficits, visual disturbances in the right eye, and bladder difficulties. Visual and auditory evoked potentials were pathological, and a cranial MRI revealed multiple periventrical white-matter lesions. We found increased intrathecal immunoglobulin production. Diagnosis was established by high concentrations of very long chain fatty acids in serum and in dermal fibroblasts after the same was found in our patient's son. In familial multiple sclerosis, ALD should be excluded in male and female patients.

Compactotomy in Huntington's chorea.

Advances in neuroradiological and neurosurgical techniques have lead to a growing interest in functional neurosurgical interventions for medically intractable movement disorders. The majority of these procedures are performed in patients with hypokinetic movement disorders, especially Parkinson's disease. However, relatively few interventions were done in hyperkinetic disorders such as Huntington's disease (HD), mainly owing to the lack of an adequate target nucleus. We have recently described the case of a reversible chorea in a genetically confirmed HD patient. We subsequently identified a marked bilateral degeneration of the substantia nigra as the probable reason for choreatic cessation. We therefore suggest that primary striatal atrophy causing hyperkinesia and secondary substantia nigra atrophy favouring hypokinesia were balanced in this patient, thus resulting in a close-to-physiologic GABAergic basal ganglia output. We postulate that deep brain stimulation of the substantia nigra pars compacta may ameliorate hyperkinesia in choreatic movement disorders, thus representing the first effective therapy in Huntington's chorea. Several lines of evidence in recent neurophysiological research support our hypothesis and are discussed below.