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Neurosurgery Oct 2019Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these...
BACKGROUND
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses.
OBJECTIVE
To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation.
METHODS
Scopus, Web of Science, and PubMed were searched from database inception to May 1, 2018 for articles reporting skull base ECD. An institutional retrospective analysis of all patients treated at the authors' institution since January 1, 1996 was also performed to supplement these data.
RESULTS
Of 465 retrieved articles, 18 studies totaling 20 patients met inclusion criteria. Institutional review identified an additional 7 patients. Collectively, the median age at diagnosis was 49 yr (interquartile range, 42-58) with a 4:1 male-to-female ratio. Patients frequently presented with diplopia (48%), headache (30%), dysarthria (22%), and vertigo or imbalance (22%), though trigeminal hypesthesia (11%), facial nerve paresis (7%), hearing loss (7%), and trigeminal neuralgia (7%) were also observed. ECD commonly mimicked meningioma (33%), trigeminal schwannoma (8%), neurosarcoidosis (8%), and skull base lymphoma (8%).
CONCLUSION
Discrete skull base lesions frequently mimic more common pathology such as meningioma or cranial nerve schwannomas. Medical therapy comprises the initial treatment for symptomatic skull base disease. Surgical resection is not curative and the utility of surgical intervention is largely limited to biopsy to establish diagnosis and/or surgical debulking to relieve mass effect.
Topics: Adrenal Cortex Hormones; Adult; Erdheim-Chester Disease; Female; Humans; Male; Middle Aged; Radiosurgery; Retrospective Studies; Skull Base
PubMed: 30828728
DOI: 10.1093/neuros/nyz027 -
Medicine Dec 2018In this study, we aimed to review the literature on phenytoin intoxication induced by compound phenytoin sodium, ephedrine hydrochloride and theophylline tablets...
OBJECTIVE
In this study, we aimed to review the literature on phenytoin intoxication induced by compound phenytoin sodium, ephedrine hydrochloride and theophylline tablets (CPEHTT).
METHOD
A literature search was performed in the following databases: WANFANG DATA, HowNet, National Library Reference and Consultation Alliance, Full-text Database of Foreign Medical Journals, PubMed and Ovid. The search terms were "Compound Phenytoin Sodium, ephedrine Hydrochloride and Theophylline Tablets," and "poisoning," or "toxicity," in Chinese and in English.
RESULT
Ten articles including 104 patients with CPEHTT intoxication were identified. The ages of the patients ranged from 52 to 82 years. Sixty-seven patients were male and thirty-seven patients were female (the male/female ratio, approximately 2:1). The most common clinical manifestations were dizziness (85%) and ataxia (85%), followed by limb weakness (65%), diplopia (25%), binocular horizontal nystagmus (24%), limb numbness (13%), nausea and vomiting (12%), somnolence (10%), tremor and high muscle tension (7%), lag in response (5%), dysarthria (6%), choking cough (2%), auditory hallucination and visual fantasy (1%), and involuntary movement (1%). All patients had chronic lung disease, and the most common disease was chronic bronchitis. The dosage ranged 4 to 15 tablets per day with medication duration of more than 1 year for most patients.
CONCLUSION
The CPEHTT intoxication caused by phenytoin toxicity represents a drug safety problem in China. The common clinical manifestations, serum phenytoin concentrations, and associated factors of CPEHTT intoxication are important for diagnosis and prevention. These findings may help guide clinicians to correctly attend to the use of CPEHTT and avoid its toxicity.
Topics: Bronchodilator Agents; China; Drug Combinations; Ephedrine; Humans; Phenytoin; Tablets; Theophylline
PubMed: 30572493
DOI: 10.1097/MD.0000000000013689 -
Die Rehabilitation Oct 2019Evaluation of German-language guidelines for aphasia and dysarthria rehabilitation, and comparison with international guidelines.
AIM
Evaluation of German-language guidelines for aphasia and dysarthria rehabilitation, and comparison with international guidelines.
METHODS
The quality of the 6 included guidelines, as found through a comprehensive literature search, was sytematically evaluated using AGREE II. Quality ratings were compared to the results of a systematic review of 19 English-language guidelines.
RESULTS
The quality of German-language guidelines was heterogeneous. In 2 out of 6 evaluation domains and in the overall assessment, no German-language guideline reached the cut-off of 66,67% for good guideline quality. Apart from the domain Editorial Independence, results remained behind those of the international reference guidelines.
CONCLUSION
In some cases, quality can be improved by simple means. It is more resource-intensive, but crucial to document search strategies and how evidence was used in guideline development. A harmonization of the German-language guidelines seems desirable.
Topics: Aphasia; Dysarthria; Germany; Humans; Language; Practice Guidelines as Topic; Quality Assurance, Health Care; Stroke Rehabilitation
PubMed: 30273948
DOI: 10.1055/a-0651-9838 -
Infection Apr 2024Cladophialophora bantiana is a wonted melanized fungus causing brain abscess. In past many cases were reported from Asia, particularly from India. Of late, there is a... (Review)
Review
PURPOSE
Cladophialophora bantiana is a wonted melanized fungus causing brain abscess. In past many cases were reported from Asia, particularly from India. Of late, there is a rise in cases in places besides Asia and hence a review of the cases is warranted.
METHODS
We present a case of fatal cerebral phaeohyphomycosis caused by C. bantiana and conduct a systematic review of culture confirmed brain abscess due to C. bantiana reported between 2015 and 2022.
RESULTS
Of the 39 cases found, majority (68%) were immunocompromised. The various clinical presentations were headache (53%), hemiparesis (34%), visual disturbance (25%), altered sensorium (18%), aphasia/dysarthria (12%) and seizures (9%). Isolated lesion was observed in 18 (60%) patients. In the sequence of occurrence, the lesions were in frontal (30%), temporal (27%) and parietal (20%) region. There were five cases with coinfections such as concurrent detection of Nocardia pneumonia in two cases, toxoplasma DNA in brain abscess, coexisting pulmonary Cryptococcus neoformans infection and coexisting Candida in a case of brain abscess in one case each. Surgical intervention was performed in 84% cases. Antifungal therapy included voriconazole (80%), liposomal amphotericin B (76%), 5-fluorocytosine (30%), posaconazole (10%), and amphotericin B deoxycholate (6%). The overall mortality was 50% with lower mortality (42%) in regions outside Asia compared to Asia (63.6%) though not statistically significant.
CONCLUSIONS
C. bantiana brain abscess is an emerging infection worldwide. Next generation sequencing is an upcoming promising diagnostic test. Early complete excision of the lesion with effective antifungals may improve the outcome.
Topics: Humans; Cerebral Phaeohyphomycosis; Antifungal Agents; Voriconazole; Brain Abscess; Ascomycota
PubMed: 37979132
DOI: 10.1007/s15010-023-02126-4 -
International Journal of... Dec 2021Neuroimaging may provide clinical evidence for speech treatment-induced neuroplasticity. This review aimed to report the current scope of evidence relating to brain...
Neuroimaging may provide clinical evidence for speech treatment-induced neuroplasticity. This review aimed to report the current scope of evidence relating to brain changes identified using neuroimaging techniques, following effective speech intervention in adults and children with motor speech disorders (MSD). Studies were retrieved from five electronic databases (PubMed, CINAHL, EMBASE (Medline), SCOPUS, and Web of Science) and a general internet search. Seven studies met the inclusion criteria. Using structural or functional neuroimaging techniques, five studies reported on the effects of the Lee Silverman Voice Treatment for dysarthria in adults and children, one study on the outcome of rhythmic-melodic voice training in adults with apraxia of speech, and one study on the effects of Prompts for Restructuring Oral Muscular Phonetic Targets therapy in children with idiopathic apraxia of speech. Identified brain changes included: enhanced white matter tract integrity; normalisation of baseline cortical activity; right-hemisphere shifts in re-organisation; perilesional activations; and cortical thinning. The current review identified preliminary evidence for treatment-dependent brain changes in adults and children with MSD. Although important to interpret within the context of Phase I research, the identification of therapeutic effects across seven heterogeneous studies suggests that treatment-induced improvements in speech performance are underpinned by demonstrable alterations in brain structure and/or function. Future research is required to better define these mechanisms of neuronal re-organisation in individuals receiving treatment for MSD, including their prognostic potential.
Topics: Adult; Apraxias; Child; Humans; Neuroimaging; Neuronal Plasticity; Speech; Speech Disorders
PubMed: 34030526
DOI: 10.1080/17549507.2021.1908425 -
Journal of Huntington's Disease 2020Corticobulbar symptoms have been reported in all stages of Huntington's disease (HD); aspiration pneumonia associated with swallowing impairment has been identified as...
BACKGROUND
Corticobulbar symptoms have been reported in all stages of Huntington's disease (HD); aspiration pneumonia associated with swallowing impairment has been identified as the most common cause of death. Whilst recent research has described positive effects of corticobulbar rehabilitation in other neurodegenerative conditions, it is unclear if this is similarly effective in HD. Preliminary evidence in corticospinal rehabilitation has revealed physical therapy and exercise could be beneficial for individuals with HD.
OBJECTIVE
This systematic review will explore the literature relative to rehabilitation of corticobulbar symptoms in adults with HD.
METHODS
Two investigators independently searched relevant electronic databases for literature related to corticobulbar rehabilitation in HD, published in English until October 2019. Included studies were critically appraised using the Oxford Centre for Evidence-based Medicine Levels of Evidence, Cochrane Risk of Bias Tool and Scottish Intercollegiate Guidelines Network checklists. Study outcomes included measurements of function, quality of life or neuromuscular physiology.
RESULTS
Seventy-seven publications were screened with eight studies meeting the inclusion criteria - two randomised control trials and six intervention studies. Validated and objective outcome measures of corticobulbar symptoms were infrequently used. There was a high risk of bias identified in 7/8 studies. The data suggested positive clinical outcomes, no adverse effects and no deterioration observed across longitudinal studies.
CONCLUSIONS
This systematic review documented a lack of high-quality evidence to support the use of rehabilitation to treat corticobulbar symptoms in HD. However, the suggestion of potential positive effects based on available, albeit limited, studies provides justification for further research in this area.
Topics: Deglutition Disorders; Dysarthria; Humans; Huntington Disease; Outcome Assessment, Health Care; Pyramidal Tracts
PubMed: 31744013
DOI: 10.3233/JHD-190384 -
Journal of Alzheimer's Disease : JAD 2021Anti-IgLON5 disease is a rare neurodegenerative tauopathy that displays heterogeneity in clinical spectrum, disease course, cerebrospinal fluid (CSF) findings, and...
BACKGROUND
Anti-IgLON5 disease is a rare neurodegenerative tauopathy that displays heterogeneity in clinical spectrum, disease course, cerebrospinal fluid (CSF) findings, and variable response to immunotherapy. Sleep disorders, bulbar dysfunction, and gait abnormalities are common presenting symptoms, and conventional brain MRI scanning is often unrevealing.
OBJECTIVE
To provide a comprehensive overview of the literature and to assess the frequency of symptoms, MRI findings, and treatment response in patients with IgLON5 autoimmunity in the serum and CSF or restricted to serum.
METHODS
We examined a 65-year-old woman with bulbar-onset IgLON5 disease with serum-restricted antibodies, and we also performed a systematic review of all confirmed cases reported in the English literature.
RESULTS
We identified 93 patients, included our case. Clinical data were obtained in 58 subjects, in whom the most frequent symptoms were sleep-disordered breathing, dysphagia, parasomnias, dysarthria, limb or gait ataxia, stridor or vocal cord paresis, movement disorders, and postural instability. Distinct MRI alterations were identified in 12.5% of cases, as opposed to unspecific or unremarkable changes in the remaining patients. T2-hyperintense non-enhancing signal alterations involving the hypothalamus and the brainstem tegmentum were observed only in the present case. Inflammatory CSF was found in half of the cases and serum-restricted antibodies in 4 patients. Treatment with immunosuppressant or immunomodulatory drugs led to sustained clinical response in 19/52 patients.
CONCLUSION
Anti-IgLON5 autoimmunity should be considered in patients with sleep disorders, bulbar syndrome, autonomic involvement, and movement disorders, and high-field brain MRI can be of diagnostic help.
Topics: Aged; Autoimmune Diseases; Bulbar Palsy, Progressive; Cell Adhesion Molecules, Neuronal; Female; Humans; Hypothalamic Diseases; Neurodegenerative Diseases; Tauopathies
PubMed: 33337376
DOI: 10.3233/JAD-201105 -
Therapeutic Advances in Neurological... 2022The safety and efficacy of hyperacute reperfusion therapies in childhood stroke due to focal cerebral arteriopathy (FCA) with an infectious and inflammatory component is...
The safety and efficacy of hyperacute reperfusion therapies in childhood stroke due to focal cerebral arteriopathy (FCA) with an infectious and inflammatory component is unknown. Lyme neuroborreliosis (LNB) is reported as a rare cause of childhood stroke. Intravenous thrombolysis (IVT) and endovascular therapy (EVT) have not been reported in LNB-associated stroke in children. We report two children with acute stroke associated with LNB who underwent hyperacute stroke treatment. A systematic review of the literature was performed to identify case reports of LNB-associated childhood stroke over the last 20 years. Patient 1 received IVT within 73 min after onset of acute hemiparesis and dysarthria; medulla oblongata infarctions were diagnosed on magnetic resonance imaging (MRI). Patient 2 received successful EVT 6.5 hr after onset of progressive tetraparesis, coma, and decerebrate posturing caused by basilar artery occlusion with bilateral pontomesencephalic infarctions. Both patients exhibited a lymphocytic cerebrospinal fluid (CSF) pleocytosis and elevated antibody index (AI) to . Antibiotic treatment, steroids, and platelet inhibitors including tirofiban infusion in patient 2 were administered. No side effects were observed. On follow-up, patient 1 showed good recovery and patient 2 was asymptomatic. In the literature, 12 cases of LNB-associated childhood stroke were reported. LNB-associated infectious and inflammatory FCA is not a medical contraindication for reperfusion therapies in acute childhood stroke. Steroids are discussed controversially in inflammatory FCA due to LNB. Intensified antiplatelet regimes may be considered; secondary prophylaxis with acetyl-salicylic acid (ASA) is recommended because of a high risk of early stroke recurrence.
PubMed: 36061261
DOI: 10.1177/17562864221102842 -
Folia Phoniatrica Et Logopaedica :... 2015To systematically review randomized controlled trials that evaluate the effects of repetitive transcranial magnetic stimulation (rTMS) on rehabilitation aspects related...
Effects of Repetitive Transcranial Magnetic Stimulation in the Rehabilitation of Communication and Deglutition Disorders: Systematic Review of Randomized Controlled Trials.
OBJECTIVE
To systematically review randomized controlled trials that evaluate the effects of repetitive transcranial magnetic stimulation (rTMS) on rehabilitation aspects related to communication and swallowing functions.
METHODS
A search was conducted on PubMed, Clinical Trials, Cochrane Library, and ASHA electronic databases. Studies were judged according to the eligibility criteria and analyzed by 2 independent and blinded researchers.
RESULTS
We analyzed 9 studies: 4 about aphasia, 3 about dysphagia, 1 about dysarthria in Parkinson's disease and 1 about linguistic deficits in Alzheimer's disease. All aphasia studies used low-frequency rTMS to stimulate Broca's homologous area. High-frequency rTMS was applied over the pharyngoesophageal cortex from the left and/or right hemisphere in the dysphagia studies and over the left dorsolateral prefrontal cortex in the Parkinson's and Alzheimer's studies. Two aphasia and all dysphagia studies showed a significant improvement of the disorder, compared to the sham group. The other 2 studies related to aphasia found a benefit restricted to subgroups with a severe case or injury on the anterior portion of the language cortical area, respectively, whereas the Alzheimer's study demonstrated positive effects specific to auditory comprehension. There were no changes for vocal function in the Parkinson's study.
CONCLUSION
The benefits of the technique and its applicability in neurogenic disorders related to communication and deglutition are still uncertain. Therefore, other randomized controlled trials are needed to clarify the optimal stimulation protocol for each disorder studied and its real effects.
Topics: Aged; Aged, 80 and over; Humans; Male; Communication Disorders; Deglutition Disorders; Reference Values; Sound Spectrography; Speech Acoustics; Statistics as Topic; Transcranial Magnetic Stimulation; Voice Quality; Randomized Controlled Trials as Topic
PubMed: 26580744
DOI: 10.1159/000439128 -
Acute Medicine 2021A 51-year-old man presented with mild headache followed by sudden onset of right sided weakness and dysarthria on the background of one week history of right sided...
A 51-year-old man presented with mild headache followed by sudden onset of right sided weakness and dysarthria on the background of one week history of right sided sciatica and malaise. He was apparently immunocompetent with only past medical history being hypertension. There was no consumption of tobacco or excess alcohol and he previously had normal liver and renal function.
Topics: Adult; Brain Ischemia; Humans; Ischemic Stroke; Male; Meningoencephalitis; Middle Aged; Streptococcus; Stroke
PubMed: 35072388
DOI: 10.52964/AMJA.0881