-
Jaw clonus in neuromyelitis optica spectrum disorder with subsequent osmotic demyelination syndrome.Journal of Family Medicine and Primary... Feb 2020Jaw clonus, a fascinating, yet uncommon clinical sign, is suggestive of supranuclear lesions of the trigeminal nerve. It has previously been reported in association with...
Jaw clonus, a fascinating, yet uncommon clinical sign, is suggestive of supranuclear lesions of the trigeminal nerve. It has previously been reported in association with amyotrophic lateral sclerosis. Hereby, we report an index case of jaw clonus in a patient of neuromyelitis optica spectrum disorder with subsequent osmotic demyelination syndrome with pseudobulbar palsy due to the involvement of pontine corticobulbar fibres.
PubMed: 32318496
DOI: 10.4103/jfmpc.jfmpc_1117_19 -
BMJ Case Reports Dec 2019
Topics: Aged, 80 and over; Deglutition Disorders; Humans; Ischemic Attack, Transient; Male; Mastication; Pseudobulbar Palsy; Toothbrushing; Vibration
PubMed: 31892622
DOI: 10.1136/bcr-2019-232061 -
Case Reports in Neurology 2019Foix-Chavany-Marie syndrome (FCMS) is a rare type of pseudobulbar palsy characterized by automatic-voluntary dissociation of movements of the face, tongue, pharynx, and...
Foix-Chavany-Marie syndrome (FCMS) is a rare type of pseudobulbar palsy characterized by automatic-voluntary dissociation of movements of the face, tongue, pharynx, and masticatory muscles. Most cases are due to bilateral ischemic lesions of the anterior operculum, but the syndrome has also been described after unilateral opercular damage, either isolated or associated with contralateral cortico-nuclear tract involvement. We report a patient with FCMS due to right anterior opercular lesion with contralateral infarction of the corona radiata. The patient presented with paralysis of the face and tongue with automatic and voluntary dissociation. To our knowledge, FCMS with this peculiar lesion topography has rarely been reported. We discuss the underlying mechanism with reference to MRI and diffusion tensor imaging.
PubMed: 31824287
DOI: 10.1159/000503856 -
Frontiers in Neurology 2019To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was...
To investigate the anatomical characteristics, clinical manifestations, and imaging features of bilateral cerebral peduncular infarction. A retrospective analysis was performed on 11 patients diagnosed with bilateral cerebral peduncular infarction in the Affiliated Hospital of Xuzhou Medical University from December 2014 to December 2018. Their clinical and imaging features were analyzed and summarized in combination with the relevant national and international literature. Among all the patients, there were eight cases with a history of hypertension, four cases with a history of diabetes mellitus, and four cases with a history of smoking. Conscious disturbance was observed in nine cases, quadriplegia in seven cases, pseudobulbar paralysis in three cases, and ataxia in one case. Brain magnetic resonance (MR) scans of bilateral cerebral peduncles showed patchy abnormal shadows with a hypointense signal on T1-weighted imaging (T1WI) and apparent diffusion coefficient (ADC) and hyperintense signal on T2-weighted imaging (T2WI), fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI). Computed tomography angiography (CTA) scans of head and neck showed severe stenosis or occlusion of vertebral artery, basilar artery, or posterior cerebral artery. All the patients received standardized treatment for cerebral infarction. Six patients died while five were left disabled. Bilateral cerebral peduncle infarction may be related to cerebral perfusion insufficiency caused by the stenosis or occlusion of vertebrobasilar artery and its branches. The main clinical manifestations are locked-in syndrome and persistent vegetative state. The specific imaging feature of "Mickey Mouse ear"-like infarction is associated with a poor prognosis.
PubMed: 31708855
DOI: 10.3389/fneur.2019.01107 -
PloS One 2019Congenital Zika Syndrome (CZS) is a unique pattern of congenital abnormalities found in fetuses and neonates infected with the Zika virus (ZIKV). Here, we clinically...
Congenital Zika Syndrome (CZS) is a unique pattern of congenital abnormalities found in fetuses and neonates infected with the Zika virus (ZIKV). Here, we clinically identify and characterize infants with CZS between 2015 and 2018 in Mato Grosso do Sul, Brazil-a border area with Paraguay and Bolivia. This cross-sectional study, based on primary and secondary data, tracks the cases registered in the Brazilian Public Health Reporting System through the following stages: (1) preliminary data analysis, (2) identification of the congenital syndrome cases, (3) etiologic classification of the cases, (4) active search, and (5) clinical assessment. Of the 72 investigated cases, 16 were probable cases of CZS. Of these, it was only possible to clinically assess 11 infants. Considering the 16 probable cases of CZS, nine were classified as confirmed cases, and five as potential cases of the syndrome. Regarding clinical features, brain palsy was identified in all analyzed infants. Moreover, microcephaly and pseudobulbar syndrome were found in eight infants, and hydrocephalus was found in three individuals. In addition to these conditions, seven children were malnourished. Our study may provide significant insights for other researches that aim to elucidate CZS and its clinical and populational consequences.
PubMed: 31584972
DOI: 10.1371/journal.pone.0223408 -
Child's Nervous System : ChNS :... Jun 2020Cerebellar mutism (CM), pseudobulbar palsy, posterior fossa syndrome (PFS), and cerebellar cognitive affective syndrome (CCAS) are terms that have been used, sometimes... (Review)
Review
Pediatric post-operative cerebellar mutism syndrome, cerebellar cognitive affective syndrome, and posterior fossa syndrome: historical review and proposed resolution to guide future study.
BACKGROUND
Cerebellar mutism (CM), pseudobulbar palsy, posterior fossa syndrome (PFS), and cerebellar cognitive affective syndrome (CCAS) are terms that have been used, sometimes interchangeably, to refer to the complex neurological constellation that occurs following surgical removal of cerebellar and fourth ventricular tumors, mostly in children, but also sometimes in adults.
METHODS
This paper reviews the origins of what is now regarded as pediatric post-operative cerebellar mutism, the cerebellar cognitive affective syndrome, and the neurological manifestations of injury to or disruption of brainstem and cerebellar structures. It examines the specific components of each of these phenomena in the context of the evolving understanding of the role of the cerebellum in nervous system function.
RESULTS
Children undergoing surgical management of tumors in the posterior cranial fossa are at risk of experiencing cranial neuropathies, corticospinal damage, cerebellar ataxia and related motor disorders, neuropsychiatric and cognitive changes, and in some patients, mutism. These clinical presentations are differentiated from each other and examined in the context of the relevant anatomical structures and distributed neural circuits. The term posterior fossa syndrome is not sufficiently helpful in distinguishing the different elements of the clinical phenomena from each other, and because of this lack of precision and specificity, there is consensus among investigators in the international Posterior Fossa Society that the designation be retired.
CONCLUSIONS
Using contemporary brain imaging methods and guided by careful clinical observation and meticulous definition of clinical phenomenology, it is now feasible to perform detailed structure function correlation analyses to achieve two critical goals in the care of children with tumors in the posterior cranial fossa. The first goal is to identify and understand the neural circuits responsible for the different manifestations-arousal, cranial neuropathies, long tract signs, cerebellar motor syndrome, cerebellar vestibular syndrome, cerebellar cognitive affective syndrome including emotional dyscontrol, and mutism. The second goal is to transform this knowledge into practical clinical intervention, preventing the complications inherent in the necessary surgery whenever possible, and develop new approaches to treatment with methods including brain modulation targeting interconnected nodes of the damaged neural circuits.
Topics: Adult; Cerebellar Diseases; Cerebellar Neoplasms; Cerebellum; Child; Cognition; Cranial Fossa, Posterior; Humans; Mutism; Postoperative Complications
PubMed: 31240391
DOI: 10.1007/s00381-019-04253-6 -
BMC Neurology Mar 2019We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite...
BACKGROUND
We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP).
METHODS
Pyramidal motor system pathologies were analyzed in 18 cases with neuropathologically confirmed PSP. Based on a retrospective clinical analysis, cases were subtyped according to Movement Disorder Society criteria for clinical diagnosis of PSP as probable, possible or suggestive of PSP with Richardson's syndrome (n = 10), PSP with predominant corticobasal syndrome (n = 3), PSP with predominant parkinsonism (n = 3), PSP with predominant speech/language disorder (n = 1), and PSP with progressive gait freezing (n = 1). Clinical manifestations of motor neuron involvement (pseudobulbar or bulbar signs and spasticity) were retrospectively assessed semiquantitatively. Neuropathologically, hyperphosphorylated tau-related pyramidal motor system neuronal, neuritic, and glial pathology using anti-tau AT8 clone immunohistochemistry, was also evaluated.
RESULTS
Clinical manifestations of pyramidal motor system involvement were found in patients with different PSP subtypes. A statistically significant higher load of tau pathology was found in the pyramidal system in PSP-Richardson's syndrome compared to other PSP subtypes (p = 0.016); however, there was no significant correlation between pyramidal system tau pathology and related motor clinical symptoms.
CONCLUSIONS
Tau pathology in the spinal cord and pyramidal motor system structures is very common in progressive supranuclear palsy and may neuropathologically supplement the distinction between classic Richardson's syndrome from other progressive supranuclear palsy subtypes.
Topics: Aged; Aged, 80 and over; Cerebral Cortex; Female; Humans; Immunohistochemistry; Male; Middle Aged; Movement Disorders; Parkinsonian Disorders; Pyramidal Tracts; Retrospective Studies; Supranuclear Palsy, Progressive; tau Proteins
PubMed: 30894142
DOI: 10.1186/s12883-019-1270-1 -
JAMA Internal Medicine Feb 2019In 2010, the US Food and Drug Administration (FDA) approved a combination of dextromethorphan hydrobromide and quinidine sulfate for the treatment of pseudobulbar affect...
Assessment of Use of Combined Dextromethorphan and Quinidine in Patients With Dementia or Parkinson Disease After US Food and Drug Administration Approval for Pseudobulbar Affect.
IMPORTANCE
In 2010, the US Food and Drug Administration (FDA) approved a combination of dextromethorphan hydrobromide and quinidine sulfate for the treatment of pseudobulbar affect after studies in patients with amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS). This medication, however, may be commonly prescribed in patients with dementia and/or Parkinson disease (PD).
OBJECTIVE
To investigate the prescribing patterns of dextromethorphan-quinidine, including trends in associated costs.
DESIGN, SETTING, AND PARTICIPANTS
This population-based cohort study of patients prescribed dextromethorphan-quinidine used data from 2 commercial insurance databases, Optum Clinformatics Data Mart and Truven Health MarketScan. The Medicare Part D Prescription Drug Program data set was used to evaluate numbers of prescriptions and total reported spending by the Centers for Medicare & Medicaid Services. Patients were included if they were prescribed dextromethorphan-quinidine from October 29, 2010, when the drug was approved, through March 1, 2017, for Optum and December 31, 2015, for Truven. Data were analyzed from December 1, 2017, through August 1, 2018.
MAIN OUTCOMES AND MEASURES
The proportion of patients prescribed dextromethorphan-quinidine with a diagnosis of MS, ALS, or dementia and/or PD, as well as the number of patients with a history of heart failure (a contraindication for the drug).
RESULTS
In the commercial health care databases, 12 858 patients filled a prescription for dextromethorphan-quinidine during the study period. Mean (SD) age was 66.0 (18.5) years, 66.7% were women, and 13.3% had a history of heart failure. Combining results from both databases, few patients had a diagnosis of MS (8.4%) or ALS (6.8%); most (57.0%) had a diagnosis of dementia and/or PD. In the Medicare Part D database, the number of patients prescribed dextromethorphan-quinidine increased 15.3-fold, from 3296 in 2011 to 50 402 in 2016. Reported spending by Centers for Medicare & Medicaid Services on this medication increased from $3.9 million in 2011 to $200.4 million in 2016.
CONCLUSIONS AND RELEVANCE
Despite approval by the FDA for pseudobulbar affect based on studies of patients with ALS or MS, dextromethorphan-quinidine appears to be primarily prescribed for patients with dementia and/or PD.
Topics: Aged; Amyotrophic Lateral Sclerosis; Dementia; Dextromethorphan; Drug Combinations; Excitatory Amino Acid Antagonists; Female; Humans; Male; Middle Aged; Multiple Sclerosis; Parkinson Disease; Pseudobulbar Palsy; Quinidine; United States; United States Food and Drug Administration
PubMed: 30615021
DOI: 10.1001/jamainternmed.2018.6112