Striatal and thalamic automatic segmentation, morphology, and clinical correlates in Parkinsonism: Parkinson's disease, multiple system atrophy and progressive supranuclear palsy.

Parkinson's disease (PD), multisystem atrophy (MSA), and progressive supranuclear palsy (PSP) present similarly with bradykinesia, tremor, rigidity, and cognitive impairments. Neuroimaging studies have found differential changes in the nigrostriatal pathway in these disorders, however whether the volume and shape of specific regions within this pathway can distinguish between atypical Parkinsonian disorders remains to be determined. This paper investigates striatal and thalamic volume and morphology as distinguishing biomarkers, and their relationship to neuropsychiatric symptoms. Automatic segmentation to calculate volume and shape analysis of the caudate nucleus, putamen, and thalamus were performed in 18 PD patients, 12 MSA, 15 PSP, and 20 healthy controls, then correlated with clinical measures. PSP bilateral thalami and right putamen were significantly smaller than controls, but not MSA or PD. The left caudate and putamen significantly correlated with the Neuropsychiatric Inventory total score. Bilateral thalamus, caudate, and left putamen had significantly different morphology between groups, driven by differences between PSP and healthy controls. This study demonstrated that PSP patient striatal and thalamic volume and shape are significantly different when compared with controls. Parkinsonian disorders could not be differentiated on volumetry or morphology, however there are trends for volumetric and morphological changes associated with PD, MSA, and PSP.

Management of Parkinson's disease and other movement disorders in woman of childbearing age: Part 1. / Manejo de la enfermedad de Parkinson y otros trastornos del movimiento en mujeres en edad fértil: Parte 1.

INTRODUCTION: The main challenge of Parkinson's disease in women of childbearing age is managing symptoms and drugs during pregnancy and breastfeeding. The increase in the age at which women are having children makes it likely that these pregnancies will become more common in future. OBJECTIVES: This study aims to define the clinical characteristics of women of childbearing age with Parkinson's disease and the factors affecting their lives, and to establish a series of guidelines for managing pregnancy in these patients. RESULTS: This consensus document was developed through an exhaustive literature search and a discussion of the available evidence by a group of movement disorder experts from the Spanish Society of Neurology. CONCLUSIONS: Parkinson's disease affects all aspects of sexual and reproductive health in women of childbearing age. Pregnancy should be well planned to minimise teratogenic risk. A multidisciplinary approach should be adopted in the management of these patients in order to take all relevant considerations into account.

Management of Parkinson's disease and other movement disorders in women of childbearing age: Part 2. / Manejo de la enfermedad de Parkinson y otros trastornos del movimiento en mujeres en edad fértil: parte 2.

INTRODUCTION: Many diseases associated with hyperkinetic movement disorders manifest in women of childbearing age. It is important to understand the risks of these diseases during pregnancy, and the potential risks of treatment for the fetus. OBJECTIVES: This study aims to define the clinical characteristics and the factors affecting the lives of women of childbearing age with dystonia, chorea, Tourette syndrome, tremor, and restless legs syndrome, and to establish guidelines for management of pregnancy and breastfeeding in these patients. RESULTS: This consensus document was developed through an exhaustive literature search and a discussion of the content by a group of movement disorder experts from the Spanish Society of Neurology. CONCLUSIONS: We must evaluate the risks and benefits of treatment in all women with hyperkinetic movement disorders, whether pre-existing or with onset during pregnancy, and aim to reduce effective doses as much as possible or to administer drugs only when necessary. In hereditary diseases, families should be offered genetic counselling. It is important to recognise movement disorders triggered during pregnancy, such as certain types of chorea and restless legs syndrome.

Management of neurological care during the COVID-19 pandemic. / La gestión de la asistencia neurológica en tiempos de la pandemia de Covid-19.

INTRODUCTION: The COVID-19 epidemic has led to the need for unprecedented decisions to be made to maintain the provision of neurological care. This article addresses operational decision-making during the epidemic. DEVELOPMENT: We report the measures taken, including the preparation of a functional reorganisation plan, strategies for hospitalisation and emergency management, the use of telephone consultations to maintain neurological care, provision of care at a unit outside the hospital for priority patients, decisions about complementary testing and periodic in-hospital treatments, and the use of a specific telephone service to prioritise patients with epileptic seizures. CONCLUSION: Despite the situation of confinement, neurology departments must continue to provide patient care through different means of operation. Like all elements of management, these must be evaluated.

The high prevalence of impulse control behaviors in patients with early-onset Parkinson's disease: A cross-sectional multicenter study.

INTRODUCTION: In Parkinson's disease patients, impulse control disorders (ICDs) have been associated with younger age and early disease onset, yet the prevalence of ICDs in early-onset Parkinson's disease (EOPD) patients has yet to be studied. Thus, we set out to compare the prevalence of impulse control behaviors (ICBs) in a cohort of EOPD patients with that in age and gender matched healthy controls (HCs), as well as to analyze the association of these symptoms with the use of dopaminergic drugs and other clinical or demographic factors. METHODS: A cross-sectional, multicenter study was carried out on patients recruited from outpatient Movement Disorder Clinics, assessing ICBs using the short form of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease (QUIP). In addition, depression and quality of life (QoL) were measured, along with other demographic and clinical variables. RESULTS: Of the 87 EOPD patients, 49 (58.3%) displayed an ICB, as did 28 of the 87 HCs (32.9%; p=0.001). Most of the EOPD patients that displayed an ICB (91.8%) were medicated with a dopamine agonist (DA) and accordingly, DA treatment was associated with a 7-fold increased risk of developing an ICB. Patients with ICBs had a higher depression score and a worse QoL. CONCLUSIONS: ICBs are much more prevalent in EOPD patients than in HCs and they are associated with DA intake, depression and a worse QoL.

The social impact of Parkinson's disease in Spain: Report by the Spanish Foundation for the Brain. / Informe de la Fundación del Cerebro sobre el impacto social de la enfermedad de Parkinson en España.

INTRODUCTION: Understanding the social and economic impact of Parkinson's disease is essential for resource planning and raising social awareness. DEVELOPMENT: Researchers reviewed the data published to date on epidemiology, morbidity and mortality, dependency, and economic impact of Parkinson's disease in Spain. In addition, a study has been carried out in order to define the public and private health care resources of Spanish patients affected by Parkinson's disease by means of an e-mail survey of all neurologists specialising in this disease and belonging to the Spanish Society of Neurology's study group for movement disorders. CONCLUSIONS: The incidence and prevalence rates of Parkinson's disease in Spain are similar to those in the rest of Europe. According to current population estimates, there are at least 300.000 patients with Parkinson's disease and one new case per 10.000 habitants per year in Spain. This has a major impact on the patient's quality of life and nearly doubles patient mortality. In addition, the disease generates sizeable costs for the country that may exceed 17.000€ per year per patient; costs will rise due to the ageing of the population and the new therapies employed. Healthcare professionals and administrators dedicate their efforts to providing quality care to patients. Despite the above, we still have a long way to go in order to provide quality, efficient, multidisciplinary, and universal healthcare.

[Galantamine therapy in dementia associated with CADASIL]. / Tratamiento con galantamina en la demencia asociada a CADASIL.

INTRODUCTION: Cholinergic neuronal impairment has been suggested in cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencefalopathy (CADASIL). Cholinomimetic therapy could be useful. CASE REPORTS: Four patients with CADASIL and dementia were treated with the acetylcholinesterase inhibitor galantamine and we assessed cognitive, behavioral, functional and the caregiver burden aspects. Three patients showed either mild improvement or stabilization in the behavior and caregiver burden. CONCLUSION: Our results suggest some benefit from galantamine treatment and they could support the existence of a cholinergic deficit in CADASIL.

[Lymphomatosis cerebri as the cause of leukoencephalopathy]. / Linfomatosis cerebral como causa de leucoencefalopatía.

INTRODUCTION: Lymphomatosis cerebri (LC) is an infrequent type of primary lymphoma of the central nervous system that is characterised by diffuse, infiltrating involvement of the white matter of the brain without the formation of a mass. AIM: To report the case of a patient with LC in order to draw attention to this disease, which is rarely diagnosed, and to its initial presentation in magnetic resonance imaging (MRI) as leukoencephalopathy. CASE REPORT: Our patient was a 56-year-old female who had clinical signs and symptoms of sub-acute dementia. Computerised axial tomography and MRI of the head revealed extensive, diffuse and bilateral involvement of the white matter, basal nuclei, mesencephalon and pons, with no mass effect or contrast enhancement. A stereotactic biopsy of the white matter (which was not conclusive) showed a perivascular mixed mononuclear-cell inflammatory infiltrate of B and T cells. No cytologic atypia was observed. Treatment was established with corticoids, which produced a clinical and radiological improvement in the first two months. During the next month the patient underwent rapid clinical deterioration with sleepiness and a worsening of the ability to walk. In an MRI scan the lesion had a more heterogeneous appearance with mass effect on adjacent structures and patchy contrast enhancement. A wedge biopsy of brain tissue led to a diagnosis of high-grade B-cell lymphoma. CONCLUSIONS: The imaging and histological appearance of LC may not be the one typically found in primary lymphomas of the central nervous system, and its clinical presentation may be similar to that of other diffuse processes involving compromise of the white matter (cerebral gliomatosis, inflammatory diseases of the white matter, such as Behçet's disease, Sjögren's disease or systemic lupus erythematosus).

Time window for clinical effectiveness of mass evacuation in a rat balloon model mimicking an intraparenchymatous hematoma.

The purpose of this study is to evaluate in a rat model if the early removal of an experimental intracerebral mass mimicking an extensive subcortical hematoma improves neurological outcome. Fifty six male Wistar rats were studied. A balloon was placed sterotactically at the level of the striatum. The balloon was inflated to 100 microl for periods of 10, 60 or 120 min (with 10 animals in each group). In 10 animals the balloon was not deflated and there were four sham operated cases. Neurological deficit was evaluated by a blinded observer by means of a clinical scale from 0 to 8 points at 24 and 72 h after inflation. Three additional animals at each inflation period were sacrificed after 6 h for pathological study with hematoxylin-eosin staining. Death rate was 9/10 animals who had permanent inflation, 4/10 in those with 2 h inflation, 2/10 for 1 h inflation and 0/10 for 10 min inflation (P<0.01 in chi square test). Many animals developed a particular clinical syndrome not previously described. Mean 72 h clinical scores (0-8 points) were 7.6 (S.D.: 1. 2) for the permanent inflation group, 4.4 (S.D.: 3.2) for 2 h of inflation, 2.3 (S.D.: 3.2) for 1 h and 0.4 (0.9) for 10 min of inflation (P<0.01 in Kruskal Wallis test). In the pathological study the rate of damaged neurons was significantly higher in the permanent than in transient inflation groups. In conclusion, in this balloon model evacuation of an extensive acute expanding subcortical (hematoma-like) mass must be performed within a limited time window to prevent the development of irreversible neurological deficits or death.

[HTLV-I myelopathy: presentation of a new case]. / Mielopatía por HTLV-I: presentación de un nuevo caso.

INTRODUCTION: HTLV-I is a human retrovirus which has been implicated in the genesis of tropical spastic paraparesis (HTLV-I-associated myelopathy). So far five cases of this illness have been detected in Spain, five of them in immigrants. We present a new case in Spain, with a characteristic chronic clinical picture. CASE REPORT: A 36-year-old black woman native of Ecuatorial Guinea, developed along 10 years a progressive paraparesis of asymmetric onset with important back pain, that arrives to paraplegic spastic phase at the present time. She presents distal amyotrophies, ulcers of decubitus and loss of control of sphincters, with normal mental status. Laboratory tests: blood, biochemistry and microbiologic studies: normal, or negative. She presented positive Western Blot serology for HTLV-I, confirmed by means of PCR technique. Cranial MRI: small and hyperintense subcortical lesions on T2 weighted images; spinal MRI: local atrophy at high thoracic level. A lumbar puncture was performed, with no cells, and with presence of oligoclonal bands, and a high IgG index. Urodynamic study: neurogenic spastic bladder. EMG: mild axonal polyneuropathy with prevalence in legs. CONCLUSIONS: In the differential diagnosis of progressive paraperesis, and mainly with epidemic antecedents, it is necessary to include a determination of HTLV-I between the diagnostic tests.

[Clinical manifestations associated with antiphospholipid antibodies]. / Manifestaciones clínicas asociadas a anticuerpos antifosfolípido.

The antiphospholipid antibodies are immunoglobulins able to join negative charge phospholipids. The have been related to a great variety of conditions, specially among connective tissue illness although the idiopathic form seems to be the most frequent. Their presence must be ruled out in cases of young patients with stroke, deep veins thrombosis, acute heart attack and woman suffer multiple abortions and foetal death. These antibodies appear to be related to different clinical entities like Sneddon syndrome. Evans syndrome, "chorea gestationis", migraine. The laboratory determinations are based in direct methods (ELISA, RIA, ...) as well as in indirect ones (activated partial thromboplastin time, reptilase time, ...). The appropriate management and treatment may be based upon clinical expression, in case of arterial thrombosis (type II APS), or deep vein thrombosis (Type II) long term anticoagulation is indicated; Association with pentoxifylline in the case of retinal thrombosis (type IIIa), Stroke (type IIIb) cases may require long term anticoagulation as well as aspirin. Type IV cases are better managed with an individualised treatment.

[Chronic disequilibrium in the older patient: investigation of reversible cases in a 22-patient series]. / Inestabilidad crónica en el paciente mayor. investigación de causas reversibles en una serie de 22 casos.

Chronic instability is a frequent complaint in elderly patients. As literature on the subject is scarce, we investigated reversible causes, evaluating patients over 65 years of age referred to a regional neurological practice for disequilibrium lasting longer than three months. Clinical features were analyzed. Computerized tomography (CT) of the brain, and thyroid hormone and cyanocobalamin (Cc) serum levels were studied for all patients, as was clinical course. No CT abnormalities were found. Low Cc levels were observed in 6 patients, and 2 were diagnosed of previously undetected hypothyroidism. During the mean follow-up period of 12 months, improvement was seen in 3 specifically-treated patients, one with hypothyroidism and two with Cc deficit. We conclude that chronic instability in the older patient cannot usually be traced to a specific cause, but that it is important to protocolize the assessment of such patients, including routine tests for thyroid hormone and Cc levels, in order to rule out treatable causes.

[Treatment of refractory infantile epilepsy with vigabatrin in a series of 55 patients]. / Tratamiento con vigabatrina en la epilepsia refractaria infantil: experiencia en una serie de 55 pacientes.

We present the results of treatment with vigabatrin in the polytherapy of resistant infantile epilepsy. A retrospective review of 55 children with resistant epilepsy aged between 2 months and 15 years was carried out between January 1992 and January 1995. Semiologically, the crises treated were simple partial crises (CPS), complex partial crises (CPC), West's syndrome, the Lennox-Gastaut syndrome and polymorphic crises. The efficacy of the drug (defined as a 50% or more reduction in crises), secondary effects and the reduction in the number of drugs necessary were evaluated. 60% of the children studied improved. 20% became completely free of crises. 34% remained unchanged and 3% became worse. In West's syndrome 100% responded satisfactorily, as did 80% of the cases of partial crises, but only 40% of those with Lennox's syndrome responded. Generalized tonic-clonic crises did not respond, and myoclonic crises became worse. Aetiologically, there was greater improvement in the symptomic cases than in the essential cases, the improvement being 70% while 5 patients with tuberose sclerosis responded particularly well. Medication was stopped in one case because of side-effects, due to a psychiatric disturbance, and in another case vigabatrin caused transitory side-effects. In 10% of the subjects the initial efficacy wore off around 6 months later. We have shown the usefulness of vigabatrin in the polytherapy of infantile epilepsy, which is resistant to conventional epileptic drugs, mainly in simple and complex partial crises and in West's syndrome. Its minimal side-effects and the favourable response in over 50% of cases make it an extremely useful drug.

Benign Valsalva's maneuver-related headache: an MRI study of six cases.

Cough headache is not infrequent, but there have not been any series studied with current neuroimaging techniques, and effective therapy has seldom been reported. In a large series from an outpatient clinic of a general hospital, we have studied, with MRI, eight cases of headache related to situations provoking sudden increase of intrathoracic pressure (cough, straining, stooping), similar to that elicited by a Valsalva's maneuver. One case showed hindbrain herniation and another showed isolated hydrocephalus. Symptoms did not differ between these two cases and the six cases without MRI abnormality. Initial symptoms presented between 49 and 67 years of age, and headache was of variable location and duration, mostly global and short-lasting. During a mean follow-up of 13.3 months, one patient became spontaneously asymptomatic, one improved on indomethacin, and two improved after treatment with propranolol. We propose the eponym, benign Valsalva's maneuver-related headache (as more appropriate than the equivalent "cough headache"), for cases in which headache is related to such situations and structural lesions are excluded by MRI or similar tests.