Calcified cerebral embolism: a 9-case series and review of the literature.

OBJECTIVE: Calcified cerebral embolus (CCE), a rarely reported and underdiagnosed cause of stroke, may be the first manifestation of a vascular or cardiac disease. We describe the characteristics of CCE in a series of 9 cases and review the literature on the subject. PATIENTS AND METHODS: We included patients with CCE from 3 different hospitals. We describe the diagnostic approach, neuroimaging findings, origin of the embolism, treatment, and prognosis of these patients. RESULTS: We identified a total of 9 patients presenting spontaneous CCE as the cause of acute ischaemic stroke. In all cases, the middle cerebral artery was affected; all patients underwent CT. A possible calcific source was found in 6 patients (66.6%), originating in the carotid arteries in 3 (33.3%) and in the heart in the other 3 patients (33.3%). Only one patient was treated in the acute phase (trombectomy) and only 11% of patients had modified Ranking Scale scores ≤ 2 at 3 months. CONCLUSIONS: CCE is more frequent than previously thought and, although the condition continues to be underdiagnosed, it is of considerable prognostic relevance in the aetiological study of stroke.

Depression is Associated with Impulse-compulsive Behaviors in Parkinson's disease.

BACKGROUND: Depression and impulse control disorders (ICDs) are both common in Parkinson's disease (PD) patients and their coexistence is frequent. Our aim was to determine the relationship between depression and impulsive-compulsive behaviors (ICBs) in a large cohort of PD patients. METHODS: PD patients recruited from 35 centers of Spain from the COPPADIS cohort from January 2016 to November 2017 were included in the study. The QUIP-RS (Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease-Rating Scale) was used for screening ICDs (cutoff points: gambling ≥6, buying ≥8, sex≥8, eating≥7) and compulsive behaviors (CBs) (cutoff points: hobbyism-punding ≥7). Mood was assessed with the BDI-II (Beck Depression Inventory - II) and major, minor, and subthreshold depression were defined. RESULTS: Depression was more frequent in PD patients with ICBs than in those without: 66.3% (69/104) vs 47.5% (242/509); p<0.0001. Major depression was more frequent in this group as well: 22.1% [23/104] vs 14.5% [74/509]; p=0.041. Considering types of ICBs individually, depression was more frequent in patients with pathological gambling (88.9% [8/9] vs 50.2% [303/603]; p=0.021), compulsive eating behavior (65.9% [27/41] vs 49.7% [284/572]; p=0.032), and hobbyism-punding (69% [29/42] vs 49.4% [282/571]; p=0.010) than in those without, respectively. The presence of ICBs was also associated with depression (OR=1.831; 95%CI 1.048-3.201; p=0.034) after adjusting for age, sex, civil status, disease duration, equivalent daily levodopa dose, antidepressant treatment, Hoehn&Yahr stage, non-motor symptoms burden, autonomy for activities of daily living, and global perception of QoL. LIMITATIONS: Cross-sectional design. CONCLUSIONS: Depression is associated with ICBs in PD. Specifically, with pathological gambling, compulsive eating behavior, and hobbyism-punding.

Physiotherapy for freezing of gait in Parkinson's disease: a systematic review and meta-analysis. / Fisioterapia para la congelación de la marcha en la enfermedad de Parkinson: revisión sistemática y metaanálisis.

INTRODUCTION: Freezing of gait (FOG) is one of the most severe symptoms associated with Parkinson's disease (PD). Physiotherapy treatment could be an effective strategy for treating FOG, but no systematic review has been carried out in this regard. AIM: To identify the characteristics, methodological quality, and main outcomes of the studies that have analyzed the effects of physiotherapy interventions in FOG up to date, by performing a systematic review and a meta-analysis. PATIENTS AND METHODS: Four electronic databases were searched in order to find randomized controlled trials that provided information regarding the effects of any kind of physiotherapy treatment on FOG. The methodological quality of the included investigations was assessed by means of the PEDro scale. RESULTS: Twelve studies were identified for inclusion into the qualitative analysis, with four randomized controlled trials included in the final meta-analysis. The quality of the trials was generally good. Those physiotherapy modalities including cues were more effective for treating FOG than traditional physiotherapy approaches. The meta-analysis indicated that physiotherapy interventions had a significantly greater impact on FOG than control comparisons. CONCLUSIONS: Physiotherapy treatment, especially those modalities including visual and auditory cueing, should be prescribed to PD patients with FOG. Future studies including PD patients with cognitive impairment and FOG objective measurement tools are need to complete the existing scientific evidence.

TITLE: Fisioterapia para la congelación de la marcha en la enfermedad de Parkinson: revisión sistemática y metaanálisis.Introducción. La congelación de la marcha (CDM) es uno de los síntomas más graves asociados con la enfermedad de Parkinson (EP). El tratamiento fisioterapéutico podría ser una estrategia efectiva para su tratamiento, pero no se ha realizado ninguna revisión sistemática al respecto. Objetivo. Identificar las características, la calidad metodológica y los principales resultados de los estudios que han analizado los efectos de las intervenciones fisioterapéuticas en CDM hasta la fecha, mediante la realización de una revisión sistemática y un metaanálisis. Pacientes y métodos. Se realizaron búsquedas en cuatro bases de datos electrónicas para encontrar ensayos controlados aleatorizados que proporcionaran información con respecto a los efectos de cualquier tipo de tratamiento fisioterapéutico sobre la CDM. La calidad metodológica de las investigaciones se evaluó mediante la escala PEDro. Resultados. Se identificaron 12 estudios para su inclusión en el análisis cualitativo y cuatro ensayos controlados aleatorizados se incluyeron en el metaanálisis final. La calidad de los ensayos fue generalmente buena. Las modalidades de fisioterapia que incluían señales fueron más efectivas para tratar la CDM que los enfoques de fisioterapia tradicionales. El meta­análisis indicó que las intervenciones fisioterapéuticas tuvieron un impacto significativamente mayor sobre la CDM que las comparaciones de control. Conclusiones. El tratamiento fisioterapéutico, especialmente las modalidades que incluyen señales visuales y auditivas, debe prescribirse a los pacientes con EP con CDM. Se necesitan estudios futuros que incluyan pacientes con EP con deterioro cognitivo y herramientas de medición objetiva de la CDM para completar la evidencia científica existente.

Calcified cerebral embolism: a 9 case series and review of the literature. / Embolia cerebral cálcica: presentación de una serie de 9 casos y revisión de la literatura.

OBJECTIVE: Calcified cerebral embolism (CCE), a rarely reported and underdiagnosed cause of stroke, may be the first manifestation of a vascular or cardiac disease. Our purpose is to describe the characteristics of CCE in a series of 9 cases and review the literature on the subject. PATIENTS AND METHODS: We included patients with CCE from 3 different hospitals. We described the diagnostic approach, neuroimaging findings, origin of the embolism, treatment, and prognosis of these patients. RESULTS: We identified a total of 9 patients presenting spontaneous CCE as the cause of acute ischaemic stroke. In all cases, the middle cerebral artery was affected; all patients underwent CT. A possible calcific source was found in 6 patients (66.6%), originated in the carotid arteries in 3 (33.3%) and in the heart in the other 3 patients (33.3%). Only one patient was treated in the acute phase (trombectomy) and only 11% of patients had modified ranking scale scores ≤ 2 at 3 months. CONCLUSIONS: CCE is more frequent than previously thought and, although the condition continues to be underdiagnosed, it is of considerable prognostic relevance in the aetiological study of stroke.

Influence of cognitive impairment on the freezing of gait in non demented people with Parkinson's disease. / Influencia del deterioro cognitivo en la congelacion de la marcha en pacientes con enfermedad de Parkinson sin demencia.

INTRODUCTION: Freezing of gait (FOG) is a motor disturbance usually appearing in advanced Parkinson's disease (PD). Cognitive and executive function seems to play an important role in this phenomenon. AIM: To investigate if cognitive and kinematic parameters correlate with FOG in PD patients without dementia. PATIENTS AND METHODS: We conducted an observational cross-sectional study. Participants were classified in two groups: freezers and non-freezers. Clinical information was obtained by Hoehn and Yahr scale, Unified Parkinson's Disease Rating Scale and balance test of Short Physical Performance Battery. Cognitive function was evaluated using Minimental Examination and the Fuld Object Memory Evaluation; executive function was assessed with the Frontal Assessment Battery test. Battery kinematic parameters were assessed by means of gait speed, cadence, stride length and stride time. RESULTS: Twenty-five participants with PD without dementia completed the evaluation. Statistical significant differences between freezers and non-freezers were found in global cognition (p = 0,02), memory (p = 0,04), executive function (p = 0,04), cadence (p = 0,02), stride length (p = 0,04) and stride time (p = 0,01). CONCLUSION: Cognitive parameters may have an important contribution to the manifestation of freezing of gait in PD. These results may have important clinical implications for developing future non-pharmacological and cognitive interventions strategies targeted to PD patients with FOG.

TITLE: Influencia del deterioro cognitivo en la congelacion de la marcha en pacientes con enfermedad de Parkinson sin demencia.Introduccion. La congelacion de la marcha (CDM) es una alteracion motora que suele aparecer en estadios avanzados de la enfermedad de Parkinson (EP). Las funciones cognitivas y ejecutivas parecen tener un papel importante en la aparicion de este fenomeno. Objetivo. Investigar si los parametros cognitivos y cinematicos se correlacionan con la CDM en pacientes con EP sin demencia. Pacientes y metodos. Estudio observacional y transversal. Los participantes se clasificaron en dos grupos: con y sin CDM. La informacion clinica se obtuvo mediante la escala de Hoehn y Yahr, la Unified Parkinson's Disease Rating Scale y la prueba de equilibrio de la Short Physical Performance Battery. La funcion cognitiva se valoro con el miniexamen cognitivo y la Fuld Object Memory Evaluation, y la funcion ejecutiva, con la Frontal Assessment Battery. Los parametros cinematicos se valoraron mediante la velocidad de la marcha, la cadencia, la longitud del paso y el tiempo del paso. Resultados. Veinticinco participantes con EP sin demencia completaron el programa. Se encontraron diferencias estadisticamente significativas entre individuos con y sin CDM en cognicion global (p = 0,02), memoria (p = 0,04), funcion ejecutiva (p = 0,04), cadencia (p = 0,02), longitud del paso (p = 0,04) y tiempo del paso (p = 0,01). Conclusion. Diversos parametros cognitivos pueden contribuir de forma importante en la aparicion de la CDM en la EP. Estos resultados pueden tener implicaciones clinicas relevantes para el desarrollo de estrategias e intervenciones no farmacologicas y cognitivas dirigidas a pacientes con EP y con CDM.

Influence of cognitive impairment on fall risk among elderly nursing home residents.

BACKGROUND: Information relating the severity of cognitive decline to the fall risk in institutionalized older adults is still scarce. This study aims to identify potential fall risk factors (medications, behavior, motor function, and neuropsychological disturbances) depending on the severity of cognitive impairment in nursing home residents. METHODS: A total of 1,167 nursing home residents (mean age 81.44 ± 8.26 years; 66.4% women) participated in the study. According to the MEC, (the Spanish version of the Mini-Mental State Examination) three levels of cognitive impairment were established: mild (20-24) "MCI", moderate (14-19) "MOCI", and severe (≤14) "SCI". Scores above 24 points indicated the absence cognitive impairment (NCI). Information regarding fall history and fall risk during the previous year was collected using standardized questionnaires and tests. RESULTS: Sixty falls (34%) were registered among NCI participants and 417 (43%) among people with cognitive impairment (MCI: 35%; MOCI: 40%; SCI: 50%). A different fall risk model was observed for MCI, MOCI, SCI, and NCI patients. The results imply that the higher the level of cognitive impairment, the greater the number of falls (F1,481 = 113.852; Sig = 0.015), although the level of significance was not maintained when MOCI and SCI participants were compared. Depression, neuropsychiatric disturbances, autonomy constraints in daily life activity performance, and low functional mobility were factors closely associated with fall risk. CONCLUSION: This study provides evidence indicating that fall risk factors do not hold a direct correlation with the level of cognitive impairment among elderly nursing home care residents.

Prevalence of parkinsonism and Parkinson's disease in the Arosa Island (Spain): a community-based door-to-door survey.

There are scarce epidemiological data on parkinsonism in Spain. Since the Arosa Island community (Northwestern Spain) has been for centuries relatively isolated, it represents a potentially useful setting to undercover genetic factors with a founder effect, as well as local environmental influences. We performed a one-stage door-to-door survey in the Arosa Island in 2004 to determine the prevalence of parkinsonism and Parkinson's disease (PD) in the population aged 65 years or over. Out of 41 individuals detected with parkinsonism, 15 were diagnosed with PD (36.6%), 13 with drug induced parkinsonism (31.7%), seven with vascular parkinsonism (17.1%), four patients had parkinsonism with associated features (9.8%) and two had unspecified parkinsonism (4.9%). We obtained a crude prevalence rate of parkinsonism of 5.44% (adjusted rate: 4.73%) and a crude prevalence rate of PD of 1.99% (adjusted rate 1.7%); both prevalence rates increased with advancing age. The prevalence rate for parkinsonism was higher than that in similar populations of Spain and other European countries while that for PD was in the range obtained from these populations. Among PD cases, 26.7% (n=4) were undiagnosed before the survey. Most cases of drug-induced parkinsonism were secondary to neuroleptics and had not been previously diagnosed. The relatively high proportion of undiagnosed drug-induced parkinsonisms and PD is surprising in a public health system which is free and universal. The lack of excess of late-onset PD among Arosans does not support the existence of specific genetic or environmental factors contributing to PD in this particular geographical area.

Prevalence and European comparison of dementia in a ≥75-year-old composite population in Spain.

OBJECTIVES: To estimate dementia prevalence in Spain. MATERIALS AND METHODS: Nine probabilistic and geographically defined samples participated. A screening design based on the MMSE was implemented. Positively screened individuals underwent clinical evaluation. The total number of cases in Spain was estimated. Prevalence was confronted to that of other European countries. RESULTS: Five hundred and forty-six persons aged ≥75 participated, 49 had dementia (35 with Alzheimer's disease [AD], 10 with vascular dementia [VD], 4 other; 25 first diagnosed in the study). Age- and sex-adjusted prevalence and estimated nationwide cases were 7.5% (95% CI 5.4-9.7), 5.6 (95% CI 3.7-7.5) and 1.4 (95% CI 0.5-2.3), and 290,000 (95% CI 208,000-372,000), 214,000 (95% CI 141,000-288,000) and 54,000 (95% CI 20,000-88,000) for dementia, AD and VD, respectively. CONCLUSIONS: Dementia prevalence in Spain is comparable to other European populations, while a high number of undiagnosed cases live in the community. The potential impact of Mediterranean diet, hypertension control and decreasing vascular risk factors is discussed.

Ischemic stroke and epilepsy in a patient with Tourette's syndrome: association with the antiphospholipid syndrome and good response to levetiracetam.

The role played by different humoral factors, including antiphospholipid antibodies, in the pathogenesis of Tourette syndrome (TS) is still presently unclear. We present a patient with chronic and severe TS who, at the age of 16 years, presented an ischemic stroke in the left posterior cerebral artery and/or postero-inferior temporal branch of the left medial cerebral artery. A complete study was negative with the exception of a positive lupus anticoagulant. The stroke was related with the primary antiphospholipid syndrome (APS). The stroke manifested visual abnormalities and thereafter by secondary generalized complex partial seizures. The epileptic syndrome was initially difficult to control but responded dramatically to levetiracetam. With this therapy, the manifestations of TS, especially the tics, improved. We conclude that some TS cases may present APS. In addition, levetiracetam may be useful in the management of TS. Further investigations should pursue both these facts.

Sneeze related area in the medulla: localisation of the human sneezing centre?

Sneezing is a rarely explored symptom in neurological practice. In the cat, a sneeze evoking centre is located in the medulla. The existence of a sneezing centre has not been confirmed in humans. A case with abnormal sneezing secondary to a strategic infarct in the right latero-medullary region is presented. A 66 year old man suddenly presented paroxysmal sneezing followed by ataxia, right sided motor and sensory symptoms, and hoarseness. The application of stimuli to the right nasal fossa did not evoke sneezing nor the wish to sneeze. The same stimuli to the contralateral nasal fossa evoked normal sneezing. The preservation of the superficial sensitivity of the nasal fossa indicates that the lesion was localised in the hypothetical human sneezing centre, very close to the spinal trigeminal tract and nucleus. This centre appears to be bilateral and functionally independent on both sides.

Autosomal dominant congenital fibre type disproportion: a clinicopathological and imaging study of a large family.

Congenital fibre type disproportion (CFTD) is considered a non-progressive or slowly progressive muscle disease with relative smallness of type 1 fibres on pathological examination. Although generally benign, CFTD has a variable natural course and severe progression has been observed in some patients. The pathogenesis of the disorder is unknown and many authors consider CFTD a syndrome with multiple aetiologies rather than a separate clinical entity. A positive family history has been reported in about 40% of cases, but the inheritance pattern is not clear. Both autosomal recessive and dominant modes of inheritance have been suggested. The present paper describes a large, multigenerational kindred that has an inherited myopathy fulfilling the histological criteria of CFTD, with autosomal dominant transmission and high penetrance. The clinical picture, remarkably similar in all affected family members, started in early infancy with mild limb muscle weakness. There was slow progression of symptoms into adulthood, with moderate to severe, mainly proximal, muscle weakness without loss of ambulation. Muscle biopsy from two affected individuals demonstrated predominance of small type 1 muscle fibres without other significant findings. Nerve conduction studies were normal and needle electromyography showed a myopathic pattern. MRI examination performed on three patients from successive generations showed involvement of proximal limb and paraspinal muscles. The clinical and pathological homogeneity in the present family, together with the lack of additional histological abnormalities after decades of disease progression in two affected individuals, supports this being a distinct myopathy with fibre type disproportion. Whether the disease in this family can be regarded as a form of the congenital myopathy known as CFTD or rather a unique condition sharing histological features with CFTD needs further investigation. This is, to our knowledge, the largest kindred with muscle fibre type disproportion reported to date. Our data confirm autosomal dominant inheritance, and this is the first MRI document of this disorder.

[Superficial siderosis of the central nervous system: report of two new cases]. / Siderosis superficial del sistema nervioso central: descripción de dos nuevos casos.

INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is a rare disease characterized by deafness, ataxia and pyramidal dysfunction. It is due to hemosiderin deposition in the subpial membranes of the brain, spinal cord and cranial nerves. Most cases are secondary to chronic or recurrent bleeding into the subarachnoid space. Diagnosis is permitted by magnetic resonance imaging (MRI). CASE REPORTS: We report two patients with a chronic, slowly progressive cerebellar ataxia and hearing loss. MRI showed T2 hypointense signals in the brain, cerebellum and spinal cord diagnostic of superficial siderosis of the CNS. Xanthochromia was present in one patient. Evoked potentials showed retrochoclear hearing loss. Extensive vascular studies were negative for bleeding sources. One patient, treated with oral anticoagulants, benefited from reduction of the International Normalized Ratio. CONCLUSION: Due to its rarity, clinical suspicion is essential for diagnosis of superficial siderosis of the CNS.

[Experience in the treatment of multiple sclerosis with interferon beta in Galicia]. / Experiencia del tratamiento con interferón beta en la esclerosis múltiple en Galicia.

OBJECTIVE: To analyze the experience in daily clinical practice of interferon-beta (IFN-beta) treatment in relapsing-remitting (RR) and secondary progressive (SP) multiple sclerosis (MS) in Galicia (Spain). PATIENTS AND METHODS: Patients with RR-MS and SP-MS treated with IFN-beta1a and 1b between 1995 and December/2000, analyzing demographic and clinical data. RESULTS: 313 patients were included, with a mean age of 38.2 years. A total of 296 patients (94.6%) were clinically defined MS and 17 (5.4%) were laboratory supported (Poser criteria); 84.6% of the patients were RR and 15.4% were SP. The mean duration of the disease prior to treatment was 7.06 years. Betaferon was used in 52.4% patients (115 RR-MS and 47 SP-MS), Avonex in 26% and Rebif in 21.6%. Relapse rate was reduced in 68.8% for the RR-MS for Betaferon-treated patients, 73.3% for Avonex treated and 35.7% for Rebif-treated patients. Betaferon reduced relapse rate in 50% for SP-MS. The global EDSS remained stable during IFN-beta treatment. During treatment, 33% of Betaferon, 60.5% of Avonex and 54.5% of Rebif-treated patients remained relapse-free. Treatment was suspended in 12.9% of Betaferon, 6.2% of Avonex, and 3% Rebif-treated patients. The most frequent causes of treatment suspension were increase in disability and in relapse count. CONCLUSIONS: The present study supports the benefits of IFN-beta treatment in RR MS and SP MS in daily clinical practice, with reduction in relapses count and incapacity, good over-all tolerance and low incidence of serious adverse side-effects.

[Topiramate therapy in patients with refractory epilepsy]. / Tratamiento con topiramato en pacientes con epilepsia refractaria.

AIMS: The purpose of this study was to examine the result of administrating topiramate (TPM) to patients with epilepsy that is refractory to treatment with two or more antiepileptic agents. PATIENTS AND METHODS: A total of 50 patients were evaluated, 90% of which had partial seizures (simple partial, complex partial and partial with secondary generalisation) and the remaining 10% suffered from generalised seizures. The most frequent aetiology was that which corresponded to the symptoms (52% of the cases). 98% of the patients were treated with two or three drugs. TPM was added with a dosage interval between 75 and 550 mg and follow-up visits were carried out throughout a period of nine months. RESULTS: 12 patients remained seizure-free for at least the first three months of the study; in 20 patients the number of seizures decreased by 50% or more; in 14 patients no changes were observed, and in the other four there was an increase in the number of seizures. The best results were obtained in cases of seizures with an idiopathic aetiology, while complex partial seizures offered the worst results. We also analysed the factors that might exert an influence on the different degrees of response. CONCLUSIONS: TPM was effective in all kinds of seizures. 24% of the patients became seizure free, and the total percentage of respondents rose to 64%. No patients dropped out of the study because of intolerance to the drugs.

Acute femoral neuropathy secondary to an iliacus muscle hematoma.

We present a patient with a spontaneous iliacus muscle hematoma, appearing immediately after a minor physical maneuver, presenting with pain and femoral neuropathy initially evidenced by massive quadriceps muscle fasciculations. A magnetic resonance imaging (MRI) study of the pelvic area confirmed the diagnosis, showing a hematoma secondary to a partial muscle tear. The patient was managed conservatively, and the continuous muscle activity ceased in 3 days, with progressive improvement of the pain and weakness. The recovery was complete. Femoral neuropathy is uncommon and usually due to compression from psoas muscle mass lesions of diverse nature, including hematomas. Usually subacute, femoral neuropathy may present acutely in cases of large or strategically placed compressive femoral nerve lesions, and may require surgical evacuation. The case presented herein is remarkable since the muscle hematoma appeared after a nonviolent maneuver, fasciculations were present at onset, and conservative management was sufficient for a full recovery.