Delayed complications of deep brain stimulation: 16-year experience in 249 patients.

BACKGROUND: Over the years, most of the deep brain stimulation (DBS) complications described have been mainly related to the surgery itself or the stimulation. Only a few authors have dealt with chronic complications or complications due to implanted material. METHODS: We retrospectively analyzed complications beyond the 1st month after surgery in 249 patients undergoing DBS at our site for 16 years, with 321 interventions overall. RESULTS: Our results show that infection is the most frequent delayed complication (12.5%), the pulse generator being the most common location. Lead breaks (9.3%) are the second most frequent complication. Symptomatic peri-lead edema and cyst formation were exceptional. CONCLUSIONS: The best knowledge about DBS complications allows for better solutions. In case of infection, conservative treatment or partial removal of the DBS system appears to be safe and reasonable. Intracranial complications related to DBS material such as peri-lead edema and cyst formation have a good prognosis. They may appear long after DBS implantation.

Bilateral pallidal deep brain stimulation in myoclonus-dystonia: our experience in three cases and their follow-up.

BACKGROUND: Myoclonus-dystonia syndrome (MDS) is an autosomal dominant movement disorder caused by mutations in the SGCE gene. MDS is characterized by mild dystonia and myoclonic jerks, and a constellation of psychiatric manifestations. Deep brain stimulation (DBS) of bilateral internal globus pallidus (GPi) has recently been introduced as a new and beneficial technique to improve motor symptoms in MDS. METHODS: We report three proven genetically MDS cases with successful response to DBS, and their clinical evolution over years. RESULTS: DBS improves significantly the Unified Myoclonus Rating Scale and Burke-Fahn-Marsden Dystonia Rating Scale in all three patients. This improvement is sustained over the years and no major adverse events were recorded. DBS stimulation parameters employed are justified and compared with cases reported throughout the literature. DISCUSSION: DBS of bilateral GPi is an effective and safe therapy to be considered in MDS refractory cases. Careful neuropsychological evaluation is essential inside the presurgery planning. Correct location of the DBS electrodes and individualized selection of stimulation parameters in each case are the main determinants of the best clinical response.

[Unilateral thalamotomy with high intensity focal ultrasound in patients with refractory essential tremor: a comparative study between patients under and over 70 years of age]. / Talamotomía unilateral con ultrasonidos focales de alta intensidad en pacientes con temblor esencial refractario: un estudio comparativo entre pacientes menores y mayores de 70 años.

INTRODUCTION: High intensity focal ultrasound (HIFU) thalamotomy is a novel treatment for refractory tremor. This study aims to compare the reduction in tremor intensity and adverse effects of treatment between patients younger and older than 70 years of age. PATIENTS AND METHODS: All the patients with refractory essential tremor treated with HIFU between March 2021 and March 2023 were included consecutively. Various demographic and clinical variables were analysed, including age and the items on the Clinical Rating Scale for Tremor (CRST). Cerebral vascular pathology was quantified using the Fazekas scale. Outcomes and adverse effects were compared between the patients aged 70 years or younger, and those older than 70 years. RESULTS: Ninety patients were included, and 50 of them were over 70 years old. Prior to treatment, the CRST A + B score was 20.4 ± 5.7 among those under 70 years of age, and 23.3 ± 5.1 in those older (p = 0.013). At six months after treatment, the mean was 3.8 ± 5.1 and 4.8 ± 4.5, respectively (p = 0.314). We found no significant differences in the CRST C score (2.8 ± 4.1 and 3.5 ± 4.8, p = 0.442). There were also no significant differences between the patients with vascular pathology (Fazekas = 1) and those without (4.6 ± 7.3 and 4.3 ± 4, p = 0.832). There were no differences in the presence of adverse effects between the groups based on age and vascular pathology. CONCLUSIONS: Contrary to traditional opinion, older patients do not have a poorer response or a higher rate of adverse effects after HIFU treatment.

TITLE: Talamotomía unilateral con ultrasonidos focales de alta intensidad en pacientes con temblor esencial refractario: un estudio comparativo entre pacientes menores y mayores de 70 años.Introducción. La talamotomía con ultrasonidos focales de alta intensidad (HIFU) es un tratamiento novedoso para el temblor refractario. El objetivo de este estudio es comparar la reducción en la intensidad del temblor y los efectos adversos del tratamiento entre pacientes menores y mayores de 70 años. Pacientes y métodos. Se incluyó consecutivamente a todos los pacientes con temblor esencial refractario tratados con HIFU entre marzo de 2021 y marzo de 2023. Se analizaron diferentes variables demográficas y clínicas, incluyendo la edad y los apartados de la Clinical Rating Scale for Tremor (CRST). Se cuantificó la patología vascular cerebral mediante la escala de Fazekas. Se compararon los resultados y los efectos adversos entre los grupos de edad de 70 años o menos y de más de 70 años. Resultados. Se incluyó a 90 pacientes, 50 de ellos de más de 70 años. Previamente al tratamiento, la CRST A + B era de 20,4 ± 5,7 en los menores de 70 años y de 23,3 ± 5,1 en los mayores (p = 0,013). A los seis meses tras el tratamiento, la media fue de 3,8 ± 5,1 y 4,8 ± 4,5, respectivamente (p = 0,314). No hallamos diferencias significativas en la CRST C (2,8 ± 4,1 y 3,5 ± 4,8, p = 0,442). Tampoco hubo diferencias significativas entre pacientes con patología vascular (Fazekas = 1) y sin ella (4,6 ± 7,3 y 4,3 ± 4, p = 0,832). No hubo diferencias en la presencia de efectos adversos entre los grupos de edad y de patología vascular. Conclusiones. En contra de lo tradicionalmente concebido, los pacientes de mayor edad no tienen una peor respuesta ni una mayor tasa de efectos adversos tras el tratamiento con HIFU.

[Advanced Parkinson's disease: clinical characteristics and treatment (part 1)]. / Enfermedad de Parkinson avanzada. Características clínicas y tratamiento (parte I).

INTRODUCTION: A large percentage of patients with Parkinson's disease (PD) develop motor fluctuations, dyskinesias, and severe non-motor symptoms within 3 to 5 years of starting dopaminergic therapy, and these motor complications are refractory to treatment. Several authors refer to this stage of the disease as advanced Parkinson's disease. OBJECTIVE: To define the clinical manifestations of advanced PD and the risk factors for reaching this stage of the disease. DEVELOPMENT: This consensus document has been prepared by using an exhaustive literature search and by discussion of the contents by an expert group on movement disorders of the Sociedad Española de Neurología (Spanish Neurology Society), coordinated by two of the authors (JK and MRL). CONCLUSIONS: Severe motor fluctuations and dyskinesias, axial motor symptoms resistant to levodopa, and cognitive decline are the main signs in the clinical phenotype of advanced PD.

Advanced Parkinson's disease: clinical characteristics and treatment. Part II.

INTRODUCTION: Many patients who have had Parkinson's disease (PD) for several years will present severe motor fluctuations and dyskinesias which require more aggressive therapies. The different approaches which are now available include deep brain stimulation of the subthalamic nucleus or medial globus pallidus, subcutaneous infusion of apomorphine, and intestinal infusion of levodopa-carbidopa. OBJECTIVE: To define the indications and results for the 3 available therapies for advanced PD. DEVELOPMENT: Exhaustive review of the literature concerning the indications and results of deep brain stimulation, subcutaneous apomorphine infusion and duodenal infusion of levodopa/carbidopa gel to treat patients with advanced Parkinson disease. CONCLUSIONS: Although numerous studies have confirmed the efficacy of the 3 different therapies in advanced PD, there are no comparative studies that would allow us to define the best candidate for each technique.

Progression and treatment of a series of patients with advanced LRRK2-associated Parkinson's disease.

INTRODUCTION: LRRK2 mutations have traditionally been associated with a benign phenotype of Parkinson's disease (PD). Favourable responses to deep brain stimulation (DBS) are reported in the advanced phase. METHODS: We performed a retrospective analysis of the clinical characteristics and progression of 13 patients with LRRK2-associated PD (13 with G2019S and 1 with I1371V). Nine patients were in the advanced phase, with a mean progression time of 7.2 years before reaching this phase. RESULTS: Seven patients underwent bilateral subthalamic DBS implantation, and 2 received infusion treatment. Patients with mutation G2019S responded excellently to DBS, with Unified Parkinson's Disease Rating Scale (UPDRS) II and III scores improving by 80% at 6 months. This response was sustained over time. The patient with mutation I1371V had a severe phenotype of the disease, and presented a moderate response to DBS. Patients with advanced LRRK2-associated PD showed predominantly frontal cognitive involvement, with significant language impairment. CONCLUSIONS: In these patients, progression was faster in the advanced stage of the disease. We emphasise the suitability of subthalamic DBS in the management of these patients.

[Subthalamic deep brain stimulation in a case of idiopathic Parkinson's disease and schizophrenia]. / Estimulación cerebral profunda subtalámica en un caso de enfermedad de Parkinson idiopática y esquizofrenia.

INTRODUCTION: Parkinson's disease (PD) and schizophrenia can coexist. Antipsychotics block striatal D2 receptors, which inevitably aggravates the manifestations of PD. CASE REPORT: We report the case of a male patient with idiopathic Parkinson's disease and schizophrenia, with poor tolerance to minimal doses of levodopa, who underwent a dramatic improvement after bilateral subthalamic deep brain stimulation (DBS-STN). DBS-STN was taken into consideration here, due to the severity of this particular case, as the only possible way to achieve motor improvement. CONCLUSIONS: The diagnosis of idiopathic PD was confirmed despite antidopaminergic treatment. DBS-STN can be considered a treatment option for disabling manifestations of PD, provided that a careful selection of patients is carried out..

TITLE: Estimulación cerebral profunda subtalámica en un caso de enfermedad de Parkinson idiopática y esquizofrenia.Introducción. La enfermedad de Parkinson (EP) y la esquizofrenia pueden coexistir. Los antipsicóticos bloquean los receptores D2 estriados, lo que inevitablemente agrava las manifestaciones de la EP. Caso clínico. Presentamos el caso de un paciente con enfermedad de Parkinson idiopática y esquizofrenia, con pobre tolerancia a dosis mínimas de levodopa, que presentó una gran mejoría tras la estimulación cerebral profunda subtalámica bilateral (ECP-NST). La ECP-NST se consideró aquí, debido a la gravedad de este caso particular, como la única posibilidad de lograr una mejoría motora. Conclusiones. El diagnóstico de EP idiopática se confirmó pese al tratamiento antidopaminérgico. La ECP-NST puede considerarse como una opción de tratamiento para las manifestaciones de la EP invalidantes, siempre y cuando la selección del paciente sea cuidadosa.

Progression and treatment of a series of patients with advanced LRRK2-associated Parkinson's disease. / Evolución y tratamiento de la fase avanzada de una serie de pacientes con enfermedad de Parkinson LRRK2.

INTRODUCTION: LRRK2 mutations have traditionally been associated with a benign phenotype of Parkinson's disease (PD). Favourable responses to deep brain stimulation (DBS) are reported in the advanced phase. METHODS: We performed a retrospective analysis of the clinical characteristics and progression of 13 patients with LRRK2-associated PD (13 with G2019S and one with I1371 V). Nine patients were in the advanced phase, with a mean progression time of 7.2 years before reaching this phase. RESULTS: Seven patients underwent bilateral subthalamic DBS implantation, and two received infusion treatment. Patients with mutation G2019S responded excellently to DBS, with Unified Parkinson's disease rating scale (UPDRS) II and III scores improving by 80% at six months. This response was sustained over time. The patient with mutation I1371 V had a severe phenotype of the disease, and presented a moderate response to DBS. Patients with advanced LRRK2-associated PD showed predominantly frontal cognitive involvement, with significant language impairment. CONCLUSIONS: In these patients, progression was faster in the advanced stage of the disease. We emphasise the suitability of subthalamic DBS in the management of these patients.

A prospective study of alterations in balance among patients with Parkinson's Disease. Protocol of the postural evaluation.

The aim of this study is to determine whether the balance problems experienced by Parkinson's disease (PD) patients may in part be due to dysfunctional processing of vestibular information, and to search for factors that may help predict the risk of falls. We evaluated the balance of 45 idiopathic PD patients and 20 healthy subjects by means of computerized dynamic posturography using sensory organization tests (SOT), rhythmic weight shift (RWS) tests and limits of stability (LOS) tests; and by the timed up-and-go (TUG) test. PD patients had poorer scores in the SOT than controls for overall balance and vestibular and visual inputs. They also performed worse in RWS and LOS tests, and were slower in performing the TUG test. Hoehn-Yahr stage did not correlate with vestibular input. Balance impairment in PD patients involves deteriorated processing of vestibular input, but this deterioration is independent of disease progression. Falls are related to PD patients' reduced limits of stability.

[Outpatient establishment and initial management of treatment with intraduodenal levodopa-carbidopa infusion in advanced Parkinson's disease]. / Instauracion ambulatoria y manejo inicial del tratamiento con infusion intraduodenal de levodopa-carbidopa en la enfermedad de Parkinson avanzada.

INTRODUCTION: Treatment with intraduodenal levodopa-carbidopa infusion is one of the three therapies currently available for advanced Parkinson's disease. It optimizes the benefit of antiparkinsonian treatment by counteracting the negative effect of erratic gastric emptying on the absorption of oral levodopa. The purpose is to describe our outpatient protocol of treatment establishment. PATIENTS AND METHODS: In our unit we have implemented a protocol for the treatment with intradoudenal levodopa-carbidopa infusion without admission based on the development of a multidisciplinary circuit among the Neurology Service, the Digestive Endoscopy Unit and the Home Hospitalization Unit. RESULTS: Over one and a half year, we treated five patients with advanced Parkinson's disease. All of them remain on the medication and no significant side effect has taken place. CONCLUSION: The outpatient onset install of this treatment saves costs and avoids the negative impact of admission on the patient with advanced Parkinson's disease, in the same way that favors their adaptation and tolerability to it.

TITLE: Instauracion ambulatoria y manejo inicial del tratamiento con infusion intraduodenal de levodopa-carbidopa en la enfermedad de Parkinson avanzada.Introduccion. El tratamiento con infusion intraduodenal de levodopa-carbidopa es una de las tres terapias de que disponemos en la actualidad en la enfermedad de Parkinson avanzada. Optimiza el beneficio del tratamiento antiparkinsoniano al contrarrestar el efecto negativo que provoca el vaciado gastrico erratico sobre la absorcion de la levodopa oral. El objetivo es describir nuestro protocolo de instauracion del tratamiento de modo ambulatorio. Pacientes y metodos. En nuestra unidad hemos implementado un protocolo de instauracion del tratamiento con infusion continua de levodopa-carbidopa intestinal sin necesidad de ingreso hospitalario gracias al desarrollo de un circuito multidisciplinar entre el propio servicio de neurologia, la unidad de endoscopia digestiva y la unidad de hospitalizacion a domicilio. Resultados. En año y medio se trato a cinco pacientes con enfermedad de Parkinson avanzada. Todos ellos continuan con el tratamiento y no han tenido complicaciones significativas. Conclusion. La instauracion ambulatoria del tratamiento ahorra costes y evita el impacto negativo del ingreso en el paciente con enfermedad de Parkinson avanzada, de la misma manera que favorece su adaptacion y tolerancia a aquel.

[Pseudomigraine as a symptom of carbon monoxide intoxication]. / Pseudomigraña como manifestación de intoxicación por monóxido de carbono.

INTRODUCTION: Carbon monoxide poisoning (CO) is a common intoxication. Diagnosis is sometimes difficult since the symptoms may be very varied. We report a case of repeated intoxication which led to several consultations and admission to hospital, since there were headaches and loss of consciousness suggesting basilar migraine. CASE REPORT: A 30 year old woman consulted for repeated headaches over the previous six months, normally occurring in the morning, accompanied by vertigo, nausea and vomiting and loss of consciousness on two occasions. Clinical examination and the complementary tests done (laboratory analysis, cerebral MR, ECG, ECG Holter, EEG) were normal. Finally CO intoxication was diagnosed when levels of carboxyhemaglobin of over 30% were found in both the patient and her sister. The cause was a faulty water heater, confirmed on technical inspection. CONCLUSIONS: CO intoxication may present as repeated headaches, with or without alteration in consciousness. Finding similar symptoms in other people living in the same place should increase suspicion. In certain case, anamnesis should include details regarding apparatus used for heating, cooking and bathrooms. More carboxyhaemaglobin estimations should be done in Emergency Departments.

[Experience with pupil tropicamide test in Alzheimer's disease]. / Experiencia con el test pupilar de tropicamida en la enfermedad de Alzheimer.

Scinto et al (Science 1994; 266: 1051-4) observed that a single drop of 0.01% tropicamide elicited more than a 13% pupil dilatation in 19 individuals with probable or possible Alzheimer's disease (AD) and 3 cognitive abnormal elderly without dementia, but not in 30 out of 32 normal elderly and 3 out of 4 patients suffering from other dementias. It could be an easy and bloodless test to help in diagnosing AD. We have administered the same test to 10 patients with probable AD (NINCDS-ADRDA criteria) and twenty 40-90 year-old control subjects (10 of whom were sons or daughters of AD patients and 10 without a family history of dementia). The researcher does not know which group the subject belongs to. He administers a single drop of 0.01% tropicamide in eye and one drop of 0.45% normal saline solution in the other (without knowing the contents of either vial) and measures the pupil diameter by means of a Goldmann pupilometer in basal condition and 10, 15, 25, 30, 35, 45, and 55 minutes after. The results show that it is necessary to measure the pupils at least between the minutes 25 and 55 to detect tha maximum pupil dilation in every case. The cutoff point to consider the result positive must be located between 43 and 50%. If we establish the cutoff point in 50% of pupil dilation, 90% of AD patients and 35% of control subjects show a positive response. There was not a statistically significant difference between both control groups. Our results from this test show a sensitivity of 90% and a specificity of 65%. The positive responses of some control subjects may express a weak specificity, or perhaps they mean that we have a marker of the pre-clinical stage of the disease before us.

[The prognostic value and evolution of blood pressure during an acute phase of stroke]. / Valor pronóstico y evolución de la tensión arterial en la fase aguda de la enfermedad cerebrovascular.

We studied the prognostic influence and evolution of blood pressure during the acute phase of stroke in 89 patients (50 men and 39 women) with an average age of 69.4 +/- 10.8 years. Seventy-two were diagnosed as having ischaemic infarct and 17 as having spontaneous intracerebral haemorrhage. Blood pressure was taken every four hours for twelve days. Clinical situation was evaluated using the Rankin scale. Systolic and diastolic blood pressure progressively decreased without needing any medication in the first two weeks of evolution. The decrease was greatest in hypertense patients and in those with left ventricle hypertrophy. We found the initial figures for systolic and diastolic blood pressure significantly higher in those patients with brain infarct who had not died and in those in a better functional position the second week of evolution. Blood pressure did not influence the prognosis of intracerebral haemorrhage patients.

[Progressive degenerative aphasia: clinical and neuroradiological observations in 18 cases]. / Afasia progresiva degenerativa: observaciones clínicas y neurorradiológicas en 18 casos.

INTRODUCTION: In some types of degenerative dementia aphasia is the main disorder. In primary progressive aphasia. (PPA) atrophy is limited to the dominant peri-sylvan region. We present 18 cases of progressive aphasia of degenerative origin, with or without dementia. MATERIAL AND METHODS: We describe the clinical and neuro-radiological findings in 3 patients with 'aphasic dementia and motor neuron disease (ADMND)', 7 with 'semantic dementia' (DS), and 4 with 'fronto-temporal dementia' with 'marked non-fluent aphasia' (AFTD). Criteria published in recent years were used. RESULTS: In patients with ADMND non-fluent aphasia progressed to global aphasia, with dementia occurring after 2-9 months, and death after an average of 17 months. In cases with SD, initial anomic aphasia progressed to transcortical sensory or global aphasia, and in patients with AFTD, Broca's aphasia or motor transcortical aphasia progressed to global aphasia. Seven of these patients had been initially diagnosed as having PPA and became demented after two years or more. In most of the cases the cognitive disorder had the characteristics of fronto-temporal dementia. All cases had cortical atrophy or asymmetrical cortical or cortico-subcortical atrophy. The 4 cases of non-fluent PPA were not demented after 21 months-6 years of illness, and showed perisylvan and left fronto-temporopolar atrophy. CONCLUSIONS: The PPA may correspond to the initial form of at least three varieties of dementia, usually the fronto-temporal type. Dementia occurs after two years or more, except in patients with motor neurone disease, when there is a latent period of less than one year.

[Chronic inflammatory demyelinating polyradiculoneuropathy associated with treatment using intraduodenal infusion of levodopa-carbidopa]. / Polirradiculoneuropatia desmielinizante inflamatoria cronica asociada al tratamiento con infusion intraduodenal de levodopa-carbidopa.

TITLE: Polirradiculoneuropatia desmielinizante inflamatoria cronica asociada al tratamiento con infusion intraduodenal de levodopa-carbidopa.

[Isolated paralysis of the hypoglossal nerve due to atlanto-occipital joint cysts]. / Paralisis aislada del nervio hipogloso ocasionada por quistes occipitoatloideos.

TITLE: Paralisis aislada del nervio hipogloso ocasionada por quistes occipitoatloideos.

[Neuroprotection in Parkinson's disease: analysis though group of experts' methodology]. / Neuroprotección en la enfermedad de Parkinson: análisis a través de la metodología de informadores clave.

INTRODUCTION: Currently used antiparkinsonian drugs neither stop nor slow-down the progressive nature of the disease. The final phase of PD is characterized by the presence of symptoms and signs resistant to dopaminergic agents, such as depression, dementia, freezing and falls. Therefore, it is urgent to develop therapies able to positively modify this outcome. Despite neuroprotection is a research priority in PD, no effective strategies have been found so far. METHOD: A key informants study was conducted. A group of experts in PD fulfilled a questionnaire of 10 questions to explore the most important topics related to neuroprotection. Afterwards a consensus about the current situation of neuroprotection in PD was established and future directions of development were suggested. RESULTS: Most of the answers emphasized the need of new concepts, the limitations of animal models and the difficulties in the difficulties in demonstrating a neuroprotective effects in humans owing to a lack of biomarkers. Some of the experts believe that we are already exerting a disease modifying effect. CONCLUSIONS: The concept of neuroprotection should be widened. Animal models should be improved. A reliable biomarker to start neuroprotective therapies long before the appearance of motor symptoms and to evaluate the neuroprotective effect of any therapy should be urgently developed.