Deep gluteal syndrome is defined as a non-discogenic sciatic nerve disorder with entrapment in the deep gluteal space: a systematic review.

PURPOSE: Clinicians are not confident in diagnosing deep gluteal syndrome (DGS) because of the ambiguity of the DGS disease definition and DGS diagnostic pathway. The purpose of this systematic review was to identify the DGS disease definition, and also to define a general DGS diagnostic pathway. METHODS: A systematic search was performed using four electronic databases: PubMed, MEDLINE, EMBASE, and Google Scholar. In eligibility criteria, studies in which cases were explicitly diagnosed with DGS were included, whereas review articles and commentary papers were excluded. Data are presented descriptively. RESULTS: The initial literature search yielded 359 articles, of which 14 studies met the eligibility criteria, pooling 853 patients with clinically diagnosed with DGS. In this review, it was discovered that the DGS disease definition was composed of three parts: (1) non-discogenic, (2) sciatic nerve disorder, and (3) nerve entrapment in the deep gluteal space. In the diagnosis of DGS, we found five diagnostic procedures: (1) history taking, (2) physical examination, (3) imaging tests, (4) response-to-injection, and (5) nerve-specific tests (electromyography). History taking (e.g. posterior hip pain, radicular pain, and difficulty sitting for 30 min), physical examination (e.g. tenderness in deep gluteal space, pertinent positive results with seated piriformis test, and positive Pace sign), and imaging tests (e.g. pelvic radiographs, spine and pelvic magnetic resonance imaging (MRI)) were generally performed in cases clinically diagnosed with DGS. CONCLUSION: Existing literature suggests the DGS disease definition as being a non-discogenic sciatic nerve disorder with entrapment in the deep gluteal space. Also, the general diagnostic pathway for DGS was composed of history taking (posterior hip pain, radicular pain, and difficulty sitting for 30 min), physical examination (tenderness in deep gluteal space, positive seated piriformis test, and positive Pace sign), and imaging tests (pelvic radiographs, pelvic MRI, and spine MRI). This review helps clinicians diagnose DGS with more confidence. LEVEL OF EVIDENCE: IV.

Deep Brain Stimulation in Patients With Mutations in Parkinson's Disease-Related Genes: A Systematic Review.

BACKGROUND: Deep brain stimulation (DBS) is an effective treatment for Parkinson's disease (PD), and careful selection of candidates is a key component of successful therapy. Although it is recognized that factors such as age, disease duration, and levodopa responsiveness can influence outcomes, it is unclear whether genetic background should also serve as a parameter. OBJECTIVES: The aim of this systematic review is to explore studies that have evaluated DBS in patients with mutations in PD-related genes. METHODS: We performed a selective literature search for articles regarding the effects of DBS in autosomal dominant or recessive forms of PD or in PD patients with genetic risk factors. Data regarding changes in motor and nonmotor scores and the presence of adverse events after the stimulation were collected. RESULTS: A total of 25 studies were included in the systematic review, comprising 135 patients. In the shorter term, most patients showed marked or satisfactory response to subthalamic DBS, although leucine rich repeat kinase 2 carriers of R114G mutations had higher rates of unsatisfactory outcome. Longer term follow-up data were scarce but suggested that motor benefit is sustained. Patients with the glucosidase beta acid (GBA) mutation showed higher rates of cognitive decline after surgery. Motor outcome was scarce for pallidal DBS. Few adverse events were reported. CONCLUSIONS: Subthalamic DBS results in positive outcomes in the short term in patients with Parkin, GBA, and leucine-rich repeat kinase 2 (non-R144G) mutations, although the small sample size limits the interpretation of our findings. Longer and larger cohorts of follow-up, with broader nonmotor symptom evaluations will be necessary to better customize DBS therapy in this population.

Presence or absence of cognitive complaints in Parkinson's disease: mood disorder or anosognosia?

We intended to evaluate whether non-demented Parkinsons's disease (PD) patients, with or without subjective cognitive complaint, demonstrate differences between them and in comparison to controls concerning cognitive performance and mood. We evaluated 77 subjects between 30 and 70 years, divided as follows: PD without cognitive complaints (n = 31), PD with cognitive complaints (n = 21) and controls (n = 25). We applied the following tests: SCOPA-Cog, Trail Making Test-B, Phonemic Fluency, Clock Drawing Test, Boston Naming Test, Neuropsychiatric Inventory, Hospital Anxiety and Depression Scale (HADS) and Beck Depression Inventory. PD without complaints presented lower total score on Scales for outcome of Parkinson's disease-cognition as compared to controls (p = 0.048). PD with complaints group showed higher scores on HADS (p = 0.011). PD without complaints group showed poorer cognitive performance compared to controls, but was similar to the PD with complaints group. Moreover, this group was different from the PD without complaints and control groups concerning mood.

Presence or absence of cognitive complaints in Parkinson's disease: mood disorder or anosognosia? / Presença ou ausência de queixas cognitivas na doença de Parkinson: transtorno de humor ou anosognosia?

ABSTRACT We intended to evaluate whether non-demented Parkinsons’s disease (PD) patients, with or without subjective cognitive complaint, demonstrate differences between them and in comparison to controls concerning cognitive performance and mood. We evaluated 77 subjects between 30 and 70 years, divided as follows: PD without cognitive complaints (n = 31), PD with cognitive complaints (n = 21) and controls (n = 25). We applied the following tests: SCOPA-Cog, Trail Making Test-B, Phonemic Fluency, Clock Drawing Test, Boston Naming Test, Neuropsychiatric Inventory, Hospital Anxiety and Depression Scale (HADS) and Beck Depression Inventory. PD without complaints presented lower total score on Scales for outcome of Parkinson’s disease-cognition as compared to controls (p = 0.048). PD with complaints group showed higher scores on HADS (p = 0.011). PD without complaints group showed poorer cognitive performance compared to controls, but was similar to the PD with complaints group. Moreover, this group was different from the PD without complaints and control groups concerning mood.

RESUMO Avaliar se pacientes com doença de Parkinson (DP) sem demência, com ou sem queixa cognitiva subjetiva, demonstram diferenças entre eles e comparativamente aos controles relativos a desempenho cognitivo e humor. Avaliados 77 indivíduos entre 30 e 70 anos: PD sem queixas cognitivas (n = 31), PD com queixas cognitivas (n = 21) e controles (n = 25). Testes aplicados: SCOPA-cognição, Trail Making Test-B, Fluência Fonêmica, Teste do Relógio, Teste Nominativo de Boston, Inventário Neuropsiquiátrico, Escala Hospitalar de Depressão e Ansiedade (HADS)e Inventário de Depressão de Beck. PD sem queixas apresentaram menor pontuação total na SCOPA-cognição, comparativamente aos controles (p = 0,048). Por outro lado, PD com queixas apresentaram maior pontuação no HADS (p = 0,011) em comparação aos controles. O grupo PD sem queixas mostrou pior desempenho cognitivo em comparação aos controles, mas foi semelhante ao PD com queixas. Este grupo foi diferente dos grupos PD sem queixas e controle em relação ao humor.

Clinical spectrum of levodopa-induced complications.

The first years of Parkinson disease (PD) treatment are marked by good and sustained responses to dopaminergic therapy. With disease progression and longer exposure to levodopa (l-dopa), patients develop a range of l-dopa-induced complications that include motor and non-motor symptoms. Motor complications include motor fluctuations, characterized by periods of reduced benefit from the medication, and l-dopa-induced dyskinesia, characterized by emergence of hyperkinetic involuntary movements. Dyskinesia can occur at peak effect of l-dopa, at the beginning and end of dose, or between doses. These motor complications are often associated with fluctuations in non-motor symptoms, particularly fluctuations in neuropsychiatric, autonomic, and sensory symptoms. Recognizing such complications and understanding their relationship with the timing of l-dopa doses is essential for adequate diagnosis and management. Society.

Tardive dyskinesia syndromes: current concepts.

Tardive syndromes (TS) encompass a broad spectrum of abnormal movements due to chronic exposure to dopamine receptor blocking agents. This review provides a compiled update on TS, including phenomenology, epidemiology, pathophysiology, genetic correlations and therapeutics, highlighting the emerging experience with atypical antipsychotics. The advent of atypical antipsychotics, which have lower affinity for dopamine receptors and act on 5-HT2A and 5-HT2C serotonin receptors, was expected to dramatically reduce the prevalence and incidence of this iatrogenic problem. Recent studies have shown that the reduction has been more modest than expected and TS remains an important challenge. Recent insights on pathophysiology, risk factors and genetic correlations have raised the hope for further individualized treatment for schizophrenic patients, and more strict use of antipsychotics. Up to now, there is no definite treatment for TS, but options range from relatively innocuous low doses of propranolol to more invasive procedures such as deep brain stimulation.

Clinical features and treatment with botulinum toxin in blepharospasm: a 17-year experience.

OBJECTIVE: It was to analyze clinical aspects of patients with blepharospasm, including outcomes of botulinum toxin treatment. Additionally, clinical characteristics of isolated blepharospasm were compared to those of blepharospasm plus other movement disorders. METHODS: Clinical data recorded during 17 years were reviewed. The variables included age, gender, age of onset, past medical history, head trauma, smoking history, family history of dystonia, severity, duration of botulinum toxin relief and adverse effects. RESULTS: A total of 125 patients were included and 75.2% were female. The mean age of onset was 54.3 years; 89.6% of the individuals started with contractions in eye region, and 39.2% of them spread to lower face or neck. Isolated blepharospasm group was compared with blepharospasm-plus group for demographic and clinical features, and therapeutic outcomes, without significant differences. Botulinum toxin treatment improved the severity of contractions (p=0.01) with low rate of side effects (14%). CONCLUSIONS: Both groups - isolated blepharospasm and blepharospasm-plus - shared similar results concerning epidemiology, clinical features and therapeutic response to botulinum toxin.

Clinical features and treatment with botulinum toxin in blepharospasm: a 17-year experience / Característica clínicas e tratamento com toxina botulínica em blefaroespasmo: experiência de 17 anos

OBJECTIVE: It was to analyze clinical aspects of patients with blepharospasm, including outcomes of botulinum toxin treatment. Additionally, clinical characteristics of isolated blepharospasm were compared to those of blepharospasm plus other movement disorders. METHODS: Clinical data recorded during 17 years were reviewed. The variables included age, gender, age of onset, past medical history, head trauma, smoking history, family history of dystonia, severity, duration of botulinum toxin relief and adverse effects. RESULTS: A total of 125 patients were included and 75.2% were female. The mean age of onset was 54.3 years; 89.6% of the individuals started with contractions in eye region, and 39.2% of them spread to lower face or neck. Isolated blepharospasm group was compared with blepharospasm-plus group for demographic and clinical features, and therapeutic outcomes, without significant differences. Botulinum toxin treatment improved the severity of contractions (p=0.01) with low rate of side effects (14%). CONCLUSIONS: Both groups - isolated blepharospasm and blepharospasm-plus - shared similar results concerning epidemiology, clinical features and therapeutic response to botulinum toxin.

OBJETIVOS: Analisar as características clínicas de pacientes com blefaroespasmo e os resultados do tratamento com toxina botulínica. Além disso, os pacientes foram divididos em dois grupos, blefaroespasmo isolado e blefaroespasmo associado a outros distúrbios do movimento, os quais foram comparados quanto a características clínicas e terapêuticas. MÉTODOS: Foram revisados prontuários dos últimos 17 anos. As variáveis consideradas foram idade, sexo, idade de início dos sintomas, antecedentes pessoais, história prévia de trauma crânio-encefálico, tabagismo, história familiar de distonia, gravidade do blefaroespasmo, duração do efeito da toxina botulínica e efeitos adversos. RESULTADOS: Foram incluídos 125 pacientes, dos quais 75,2% eram do sexo feminino. A média de idade do início dos sintomas era 54,3 anos; 89,6% dos pacientes tiveram início dos sintomas na região ocular e em 39,2% dos casos houve disseminação para face ou pescoço. Não houve diferenças significativas entre os grupos blefaroespasmo isolado versus blefaroespasmo-plus. O tratamento com toxina botulínica proporcionou melhora dos sintomas (p=0,01) com baixa incidência de efeitos adversos (14%). CONCLUSÕES: Ambos os grupos - blefaroespasmo isolado e blefaroespasmo-plus - apresentaram as mesmas características clínicas, epidemiológicas e resposta terapêutica à toxina botulínica.

Gradenigo's Syndrome: Beyond the Classical Triad of Diplopia, Facial Pain and Otorrhea.

We report a case of a non-Hodgkin's lymphoma in a young woman presenting with an abdominal mass and an unusual instance of cranial nerve palsies mimicking Gradenigo's syndrome. This condition is characterized by a triad of otorrhea, facial pain and diplopia, related to otitis media in the pre-antibiotic era. Incomplete and atypical clinical features of Gradenigo's syndrome have been described and noninfectious causes may mimic this condition. Careful clinical history and physical examination, including neuroimaging, are necessary to make a differential diagnosis.

Ginkgo biloba and cerebral bleeding: a case report and critical review.

Ginkgo biloba is a herbal medication that is often used worldwide. Although side effects are uncommon, G. biloba has been associated with serious bleeding complications, especially intracranial hemorrhage. We report the case of a young woman who made chronic use of G. biloba and suffered from cerebral bleeding without any structural abnormalities. Several studies have pointed to the association between G. biloba and intracranial hemorrhage.

Hepatitis C virus: a rare manifestation--remitting relapsing central and peripheral demyelination.

The most frequent neurologic manifestations of hepatitis C virus infection include peripheral neuropathy axonal type and central nervous system (CNS) vasculitis. Affected patients usually have cryoglobulinemia and other signs of vasculitis. Demyelinating lesions, both central and peripheral are rarely described. We present a case of simultaneous peripheral nervous system and CNS demyelination that comes in relapsing episodes, with negative cryoglobulins.

Progressive supranuclear palsy: new concepts / Paralisia supranuclear progressiva: conceitos atuais

Progressive supranuclear palsy (PSP) is a distinctive form of neurodegenerative disease which affects the brainstem and basal ganglia. Patients present supranuclear ophthalmoplegia, postural instability and mild dementia. PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. Over the last decade many lines of investigations have helped refine PSP in many aspects and it is the purpose of this review to help neurologists identify PSP, to better understand its pathophysiology and to provide a more focused, symptom-based treatment approach.

A paralisia supranuclear progressiva (PSP) é uma doença neurodegenerativa, que afeta principalmente o tronco cerebral e os núcleos da base. O quadro clínico se caracteriza por oftalmoparesia supranuclear, instabilidade postural e demência . Do ponto de vista anátomo-patológico, a PSP se caracteriza por acúmulo de emaranhados neurofibrilares no núcleo subtalâmico, globo pálido, núcleo rubro, substância negra, estriado, tegumento da ponte, núcleos oculomotores, bulbo e núcleo denteado. Nas últimas décadas, muitas linhas de pesquisa têm colaborado para redefinir a PSP em muitos aspectos. Os objetivos dessa revisão são auxiliar o neurologista geral na identificação da doença, compreensão da sua fisiopatologia, além de apresentar alternativas para seu tratamento sintomático.

Tic Disorder: An Unusual Presentation of Neurotoxoplasmosis in a Patient with AIDS.

Movement disorders have been increasingly recognized in patients with HIV infection and may be due to distinct causes, as opportunistic infections or medication side effects for example. Parkinsonism, tremor and hemichorea have been more frequently noted in association with HIV and opportunistic infections. However, a variety of involuntary movements have already been described. We report a case of neurotoxoplasmosis in a patient with HIV infection who presented with a dystonic tic involving ocular, oral and cervical movements.

Progressive supranuclear palsy: new concepts.

Progressive supranuclear palsy (PSP) is a distinctive form of neurodegenerative disease which affects the brainstem and basal ganglia. Patients present supranuclear ophthalmoplegia, postural instability and mild dementia. PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. Over the last decade many lines of investigations have helped refine PSP in many aspects and it is the purpose of this review to help neurologists identify PSP, to better understand its pathophysiology and to provide a more focused, symptom-based treatment approach.

A double-blind randomized controlled trial of low doses of propranolol, nortriptyline, and the combination of propranolol and nortriptyline for the preventive treatment of migraine / Estudo controlado, randomizado e duplo cego do uso de baixas doses de propranolol, nortriptilina e a combinação destas duas drogas no tratamento preventivo da migrânea

Few trials have evaluated combination of two or more drugs in the preventive treatment of migraine. In this study three therapeutic regimens were compared: (a) propranolol, at a dose of 40 mg per day, (b) nortriptyline, at a dose of 20 mg per day, and (c) the combination of these two drugs in these dosages. The groups were matched according to age, gender, and frequency of migraine attacks prior to treatment. The period of treatment was two months and the frequency and intensity of headache attacks of the 30 days pre-treatment period were compared with the frequency of headaches in the treatment period. Fourteen patients in groups A and B and sixteen patients in group C have completed the study. Treatment with propranolol, alone or in combination, was shown to be effective. Treatment with nortriptyline alone was not effective. All three therapeutic regimens were safe and side effects were minimal. The frequency of discontinuation of the study was the same in the 3 groups but no patient left the study due to adverse reactions. The combined therapy proved to be as safe as the monotherapy. Further studies evaluating this and other possible combinations of drugs in higher doses and for longer periods, should more clearly elucidate the role of combined therapy in the treatment of migraine.

Poucos ensaios clínicos têm avaliado o tratamento preventivo da migrânea através da combinação de drogas. Neste estudo, três regimes terapêuticos foram comparados: (a) popranolol, na dose de 40 mg por dia, (b) nortriptilina, na dose de 20 mg por dia e (c) combinação destas duas drogas nestas dosagens. Os grupos foram pareados de acordo com idade, sexo e freqüência de crises previamente ao tratamento. O período de tratamento foi de dois meses e a frequência e a intensidade das crises de cefaléia do período pré-tratamento foram comparadas com as do período de tratamento. Concluíram o estudo 14 pacientes do grupo A, 14 do grupo B e 16 do grupo C. Os tratamentos com propranolol, isolado ou em associação mostraram-se eficazes. O tratamento com nortriptilina isolada não se mostrou eficaz para a redução do número de dias com cefaléia. Todos os três regimes terapêuticos foram seguros e os efeitos colaterais foram mínimos. A freqüência de abandono do estudo foi a mesma nos 3 grupos e nenhum paciente abandonou o estudo devido a reações adversas. A terapia combinada mostrou-se tão segura quanto a monoterapia. Estudos futuros avaliando esta e outras possíveis combinações de drogas, em doses maiores e por períodos mais longos, deverão elucidar mais claramente o papel da terapia combinada no tratamento da migrânea.