Inappropriate Laughter Research Articles

Top of Basilar syndrome

Top of Basilar syndrome(BTS) is defined as ischemia of the areas fed by the distal basilar artery (brainstem, thalamus, cerebellum, temporal and occipital region). It shows many different symptoms. Usually visual and oculomotor disorders, changes of mind, speech disorders, seizures and hallucinations are observed. Beginning symptoms include epileptic seizures, decreased speech and inappropriate laughter, and hallucinations. This our article is that prensentation of neurological analysis in the management of cases with sudden behavioral disorders. A patient diagnosed with BTS with rare symptoms is presented. Clinicians need to be careful about BTS, which has symptoms such as sudden mood swings, personality changes and behavioral disorders

The Year’s Work in American Humor Studies, 2021

The Year’s Work in American Humor Studies, 2021

Neuropsychiatric symptoms related to agenesis of the corpus callosum. A case report

IntroductionWe present the case of a 41-year-old male patient with multiple psychiatric diagnoses, he was diagnosed with agenesis of the corpus callosum, which explains his clinical presentation.ObjectivesThe objective is to carry out a brief review of the symptoms associated with the agenesis of the corpus callosum.MethodsThe patient has been diagnosed with ADHD, cyclothymia, depressive anxiety disorder and social phobia. He has been treated with a multitude of drugs such as antidepressants, anxiolytics, stimulants and even low-dose antipsychotics. Despite the pharmacological treatments received, as well as the therapies, the patient’s functionality has progressively worsened, to the point of restricting going out of the home or maintaining a stable job.Biographical data were collected, including psychomotor retardation and inappropriate laughter. He showed mannerisms such as fluttering and low frustration tolerance. He was slow to respond to his name and showed little affective resonance with his sister and parents. Restrictive interests, especially with English culture, for which he later studied English philology. On the other hand, his mother explains that he had no symbolic play and that, from early childhood, he had difficulties in relationships with peers.Due to the aforementioned clinical manifestations, the functional worsening and the examination carried out in the consultation room, it was decided to extend the study with a brain MRI, where an agenesis of the corpus callosum was observed.ResultsAgenesis of the corpus callosum is a malformation of the central nervous system, which affects one in every 4000 births. It can be partial or complete, and occurs between the 7th-20th week of gestation.Agenesis of the corpus callosum presents with a triad of symptoms:-Reduced interhemispheric communication of sensory-motor information.-Increased information processing time-Difficulty in abstract thinking.This triad causes difficulties not only cognitively, but also socially. There is difficulty in integrating and learning new verbal and visual information. Tendency to literalism, with difficulty in understanding double meanings. They also have difficulty understanding non-verbal language and reading emotions, which makes interaction with peers difficult. All these symptoms can sometimes be confused with symptoms compatible with Autism Spectrum Disorder.Conclusions After the diagnosis and after focusing the patient’s treatment on the most limiting symptoms of his daily life, an integrated approach was initiated, not only at a pharmacological level, with the use of antidepressants and anxiolytics, but also from a psychotherapeutic point of view, working on those areas in which the patient is most dysfunctional. He was accompanied in the disability application process, as well as helped in the search for associations for adults with ASD, finding there the answer to his symptoms and difficulties.Disclosure of InterestNone Declared

Angelman Syndrome in a Filipino Child

Angelman syndrome (AS) is a distinct condition that presents with severe developmental delay. This condition also presents with speech impairment, ataxia/tremor, and inappropriate laughter. Some other features in most patients include microcephaly, seizures, tongue protrusion, wide mouth, and hypopigmentation. This case aims to emphasize the value of diagnosis in a patient with developmental delay. The diagnosis allows anticipation of the development of other possible problems and guides appropriate management. This report also aims to increase awareness regarding the condition. Here we present a 71-month-old Filipino male with developmental delay at six months, seizures at 10 months with a note of an overall happy demeanor and frequent inappropriate bouts of laughter at one year old. The patient also presented with severe stunting, microcephaly, wide mouth and ataxic gait. Through pattern recognition and the updated consensus of its diagnostic criteria, and confirmation via fluorescence in situ hybridization (FISH), which revealed a deletion in chromosome 15q11, the diagnosis of AS was made. This case re-emphasizes the role of clinical recognition of this condition and its confirmation via cytogenetic techniques like FISH, which will aid appropriate management and counseling for the patient and their families.

Inappropriate Laughter: Affective Homophily and the Unlikely Comedy of #MeToo

This article investigates the affective and ambiguous dynamics of feminist humor as an unexpected strategy of resistance in connection with #MeToo, asking what laughter may do to the sharpness of negative affect of shame and anger driving the movement. Our inquiry comes in three vignettes. First, we deploy Nanette—Hannah Gadsby’s 2018 Netflix success heralded as the comedy of the #MeToo era—arguing that the uniform viral warmth surrounding the show drives the emergence of networked feminisms through “affective homophily,” or a love of feeling the same. With Nanette, the contagious qualities of laughter are tamed by a networked logic of homophily, allowing for intensity while resisting dissent. Our second vignette zooms in on a less known feminist comedian, Lauren Maul, and her online #MeToo musical comedy riffing off on apologies made by male celebrities accused of sexual harassment, rendering the apologies and the men performing them objects of ridicule. Our third example opens up the door to the ambivalence of irony. In considering the unexpected pockets of humor within the #MeToo scandal that ripped apart the prestigious institution of the Swedish Academy, we explore the emergence of carnivalesque comedy and feminist uses of irony in the appropriation of the pussy-bow blouse as an ambiguous feminist symbol. Our examples allow us to argue for the political importance of affective ambiguity, difference, and dissent in contemporary social media feminisms, and to highlight the risk when a movement like #MeToo closes ranks around homogeneous feelings of not only shame and rage, but also love.

Pseudobulbar Affect in Parkinsonian Disorders: A Review.

Pseudobulbar affect (PBA) is a neurological symptom of inappropriate and uncontrollable laughter or crying that occurs secondary to a variety of neurological conditions, including parkinsonian disorders. PBA is a socially and emotionally debilitating symptom that has been estimated to affect 3.6% to 42.5% of the population with Parkinson’s disease. While indexing measures and treatment options for PBA have been extensively studied in neurological conditions such as amyotrophic lateral sclerosis and multiple sclerosis, there has been considerably less attention given in the literature to PBA in parkinsonian disorders. The purpose of this review is to discuss the pathophysiology of PBA, its prevalence and impact on quality of life in parkinsonian disorders, and the treatment options currently available. Areas requiring further study, including the development of standardized, cross-culturally validated methods of symptom assessment, and evidence-based studies exploring the efficacy of current treatment options in parkinsonian disorders, are also highlighted.

Pathological Laughter as a Symptom of Middle Cerebral Artery Stroke

BackgroundPathological laughter is defined as uncontrollable and inappropriate laughter unrelated to an emotion or a mood. This symptom can reveal a stroke. Case ReportWe described the case of a 57-year-old patient who presented to the emergency department 2 h after a sudden onset of left hemiparesis preceded by pathological laughter. The left motor weakness was very discrete and underestimated because of severe behavioral changes, that is, laughter, joviality, and motor restlessness. Despite abnormal brain imaging results, symptoms were considered as atypical to evoke a stroke. The patient did not receive intravenous thrombolysis. Brain magnetic resonance imaging performed 2 days after admission confirmed the diagnosis of stroke. Why Should an Emergency Physician Be Aware of This?Emergency physicians are at the forefront of stroke management. They should be aware that the initial symptom of a stroke can be atypical and lead to misunderstanding the diagnosis. Because the treatment of stroke requires the fastest care, it is important for emergency physicians to know that sudden behavioral troubles and pathological laughter can reveal strokes.

Clinical and genetic analysis of two unrelated patients with Angelman syndrome and novel UBE3A mutations

To explore the genetic cause for two familial Angelman syndrome cases and correlation between the clinical phenotypes and their genetic basis. Karyotyping analysis and microarray assay were carried out to exclude chromosome anomalies and uniparental disomy. The UBE3A gene was analyzed for potential point mutations, deletions, insertions and splice site mutations. Reverse transcription PCR was used to evaluate splicing mutation of the RNA transcripts. DNA sequencing showed the proband of family 1 has carried a novel maternal UBE3A splice acceptor site mutation, resulting in a guanine-to-cytosine transversion (IVS15-1G>C). Reverse transcription PCR revealed the proband and his mother both carried heterozygous mutant transcripts with loss of 54 and 59 nucleotides in exon 16, respectively. The proband displayed severe mental retardation, ataxia, seizures and inappropriate laughter. The siblings of family 2 has carried a novel maternal missense mutation in exon 16 of the UBE3A gene (c.2540C>T). She also presented with mental retardation, absent speech, mild ataxia and inappropriate laughter. The novel IVS15-1G>C and c.2540 C>T mutations of the UBE3A gene probably underlie the AS in the two families. Compared with small-scale mutations, larger fragments mutations can produce more severe phenotypes.

Atypical presentation of central pontine myelinolysis in hyperglycemia.

Central pontine myelinolysis (CPM) usually occurs with rapid correction of severe chronic hyponatremia. Despite the pronounced fluctuations in serum osmolality, CPM is rarely seen in diabetics. This is a case report of CPM associated with hyperglycemia. A 45-year-old non-smoking and non-alcoholic African American male with past medical history of type 2 diabetes, hypertension, stage V chronic kidney disease and hypothyroidism presented with a two-week history of intermittent episodes of gait imbalance, slurred speech and inappropriate laughter. Physical examination including complete neurological assessment and fundoscopic examination were unremarkable. Laboratory evaluation was significant for serum sodium: 140 mmol/L, potassium: 3.9 mmol/L, serum glucose: 178 mg/dL and serum osmolality: 317 mosmol/kg. His ambulatory blood sugars fluctuated between 100 and 600 mg/dL in the six weeks prior to presentation, without any significant or rapid changes in his corrected serum sodium or other electrolyte levels. MRI brain demonstrated a symmetric lesion in the central pons with increased signal intensity on T2- and diffusion-weighted images. After neurological consultation and MRI confirmation, the patient was diagnosed with CPM secondary to hyperosmolar hyperglycemia. Eight-week follow-up with neurology was notable for near-complete resolution of symptoms. This case report highlights the importance of adequate blood glucose control in diabetics. Physicians should be aware of complications like CPM, which can present atypically in diabetics and is only diagnosed in the presence of a high index of clinical suspicion.Learning points:Despite the pronounced fluctuations in serum osmolality, central pontine myelinolysis (CPM) is rarely seen in diabetics. This case report of CPM associated with hyperglycemia highlights the importance of adequate blood glucose control in diabetics.Physicians should be aware of complications like CPM in diabetics.CPM can present atypically in diabetics and is only diagnosed in the presence of a high index of clinical suspicion.

Amelioration of Aggression and Echolalia With Propranolol in Autism Spectrum Disorder

Amelioration of Aggression and Echolalia With Propranolol in Autism Spectrum Disorder

Neuro Wilson disease in an adolescent girl – early presentation- a case report

Wilson disease (WD) is an inborn error of copper metabolism caused by a mutation to the copper-transporting gene ATP7B. The disease has an autosomal recessive mode of inheritance, and is characterized by excessive copper deposition, predominantly in the liver, cornea, kidney and brain. There are varied clinical presentations for WD. The prognosis depends on various factors like age, sex, organ involvement, time of diagnosis, early initiation of de-coppering therapy and extent of involvement in case of neurowilson disease. In WD excessive copper accumulates in liver then gets redistributed to nervous system, cornea, kidneys and other organs. In first decade of life, hepatic involvement predominates but neurological manifestations occur in third or fourth decade. Studies showed Indian children with neurowilson disease present with behavior abnormality, speech and cognitive impairment, sub-clinical affection of visual pathway and autonomic function. Here we present a 12 years old girl with primary neurological manifestation of Wilson disease. She presented with abnormal gait, dysarthria and inappropriate laughter. On examination she also had Kayser- Fleischer (KF) ring in both eyes and MRI revealed extensive gray and white matter abnormalities, which suggest poor prognosis in the index case. In spite of good compliance with de-coppering therapy with D- penicillamine and zinc, she had progressive neurological deterioration in the form of progressive dystonia, dysarthria and difficulty in walking.

Δ9-THC Intoxication by Cannabidiol-Enriched Cannabis Extract in Two Children with Refractory Epilepsy: Full Remission after Switching to Purified Cannabidiol.

Animal studies and preliminary clinical trials have shown that cannabidiol (CBD)-enriched extracts may have beneficial effects for children with treatment-resistant epilepsy. However, these compounds are not yet registered as medicines by regulatory agencies. We describe the cases of two children with treatment-resistant epilepsy (Case A with left frontal dysplasia and Case B with Dravet Syndrome) with initial symptom improvement after the introduction of CBD extracts followed by seizure worsening after a short time. The children presented typical signs of intoxication by Δ9-THC (inappropriate laughter, ataxia, reduced attention, and eye redness) after using a CBD-enriched extract. The extract was replaced by the same dose of purified CBD with no Δ9-THC in both cases, which led to improvement in intoxication signs and seizure remission. These cases support pre-clinical and preliminary clinical evidence suggesting that CBD may be effective for some patients with epilepsy. Moreover, the cases highlight the need for randomized clinical trials using high-quality and reliable substances to ascertain the safety and efficacy of cannabinoids as medicines.

Ten-year updated meta-analysis of the clinical characteristics of pediatric mania and hypomania.

The phenomenology and diagnosis of pediatric bipolar disorder has been controversial. We aimed to update a 2005 meta analysis of the prevalence of manic symptoms in youth, in order to determine whether the picture of pediatric mania has changed as research on pediatric bipolar disorder has grown. We conducted literature reviews in PsycINFO and PubMed; studies with the prevalence of manic symptoms in youth were included. Two raters coded each study; kappa was 0.86-1.0. Twenty studies were meta-analyzed (N = 2,226 youths). The most common symptoms across bipolar subtypes, using a random-effects model, were: increased energy 79%, irritability 77%, mood lability 76%, distractibility 74%, goal-directed activity 72%, euphoric/elated mood 64%, pressured speech 63%, hyperactive 62%, racing thoughts 61%, poor judgment 61%, grandiosity 57%, inappropriate laughter 57%, decreased need for sleep 56%, and flight of ideas 54%. Symptom rates were heterogeneous across samples; potential predictors were explored but no clear patterns were found. Debate continues about the definitions of pediatric bipolar disorder; the results of this meta-analysis suggest that there is significant heterogeneity of symptom prevalence between studies, and that symptoms vary widely across individuals. Understanding the roots of this heterogeneity could broaden understanding of the complex clinical presentation of pediatric mania, and aid in diagnosis.

RETT SYNDROME: DIFFICULTIES OF DIAGNOSTICS (CLINICAL AND PSYCHOPATHOLOGICAL ASPECTS)

Showed a rare case of atypical forms of Rett syndrome in girl adolescent. The peculiarity of the disease was in the late manifestation of clinical symptoms (6 years old), when there was autistic behavior and regression in development, as well as rare stereotypical hand movements, with characteristic changes in the EEG as benign epileptiform patterns of childhood. In adolescence (12 years) there were episodes of hyperventilation and arrest breathing, deformation of the back, inappropriate laughter and screams. Was found mutation in the heterozygous state (s.674>G / N) in the gene MECP2 (exons 1–4) by the method of direct automatic sequencing.

Subacute encephalitis in a child seropositive for alpha-3 subunit of neuronal nicotinic acetylcholine receptors antibody

A previously healthy and developmentally normal 5-year-old boy from Saudi Arabia presented with a 3 mo history of abnormal behavior in the form of hyperactivity, decreased social engagement, episodic aggressiveness, decreased cognitive performance in school, inappropriate laughter and talking to himself, insomnia, tics and other stereotyped behaviors. His clinical neurological examination did not reveal any focal neurological deficits, but a prolonged electroencephalogram showed non-specific slowing over the right hemisphere. A diagnosis of encephalopathy was entertained, and he underwent testing for infectious, toxic, metabolic and mitochondrial disorders. Magnetic resonance imaging of the brain and cerebrospinal fluid studies were all normal. However, a comprehensive evaluation of paraneoplastic antibodies from cerebrospinal fluid demonstrated the presence of antibodies against the alpha-3 subunit of the neuronal nicotinic acetylcholine receptor, a finding also documented by tests from two different laboratories. An extensive diagnostic investigation that included a fludeoxyglucose-positron emission tomography scan for malignancies showed negative results. The patient was treated with plasmapheresis treatments (five ex- changes) that resulted in a significant improvement in his clinical picture. Following his initial treatments, the patient continued to receive monthly infusions of intravenous immunoglobulin. An evaluation at 5.5 mo after initiation of first treatment revealed a considerable improvement of his behavior and language abilities. The patient returned to his school where he has performed satisfactorily. To our knowledge, this is the first report of a child who developed sub-acute cognitive and neurobehavioral re- gression in association with the presence of a serum antibody to a neuronal ganglionic nicotinic acetylcholine receptor and who improved after immunotherapy.

Fahr's Disease: A Rare Neurological Disease

Abstract A 40-year-old unmarried female presented with abnormal involuntary choreo-athetoid movements involving both upper limbs for 5 years along with features, such as bouts of disorientation, anxiety, personality changes, reckless behaviour, inappropriate laughter and progressive decline in the neurological status. On neurological examination, her speech was dysarthric with mild choreiform movements involving both upper limbs. She had MMSE score of 20/30. MRI scans of the brain plain T1- and T2-weighted axial and flair coronal images were obtained. It showed calcifications as hyper-intense lesions on T1W and hypo-intense T2W lesions in bilateral basal ganglion and bilateral dentate nuclei of cerebellum, that is consistent with Fahr's syndrome.

Sarcoidosis Presenting As Late‐Onset Dementia: Are Cerebrospinal Fluid Biomarkers Analyses Helpful?

To the Editor: Neurosarcoidosis is uncommon in elderly adults1 and is strongly associated with dementia.2 Biopsy of the central nervous system (CNS) is usually required to confirm the diagnosis.3 The case of a 71-year-old man with rapidly progressive onset of memory deficit and finally with signs of dementia syndrome, apathetic state, and abrupt weight loss (20 kg) is reported. His medical history was positive for high blood pressure and depression. Initial physical examination demonstrated bilateral Babinski signs, and initial cognitive assessment revealed a Mini-Mental State Examination (MMSE) score of 23 that rapidly declined to 9. Executive disorder and storage memory deficit were also initially noticed. Magnetic resonance imaging (MRI) of the brain showed minor hyperintense white matter lesions at T2, with an irregular aspect of the cerebral gyri and microbleeding next to the frontal horn of the lateral ventricle. Ethylcysteinate dimer enhanced single-photon emission computed tomography of the brain showed hypoperfusion in frontal lobes. With subsequent imaging and biological findings (Table 1) indicating systemic sarcoidosis, bronchial biopsy revealed an epithelial and giant cell inflammatory granuloma (EGG) without caseous necrosis.1 Rapid improvement of cognitive function and general health status (weight gain and partial regression of the apathetic state) was observed with cortiosteroid theraphy (1 mg/kg per day). The cortiosteroid theraphy dose was reduced to 0.5 mg/kg per day and maintained. The diagnosis of neurosarcoidosis was made based on the above-mentioned findings, which matched the diagnostic criteria for probable neurosarcoidosis.3 Thus, clinical findings, cognitive profile, laboratory test results supporting CNS inflammation (high cerebrospinal fluid (CSF) protein levels, presence of oligoclonal band), and pathology-confirmed systemic sarcoidosis (EGG), were compatible with the diagnosis of neurosarcoidosis. With no leptomeningeal or cerebral parenchyma damage, which would have been biopsied, there was no indication for brain biopsy. Unspecific brain imaging results do not exclude a diagnosis of neurosarcoidosis.4 Abnormalities of the white matter detected using computed tomography and degenerative changes affecting the perivascular spaces or the brain parenchyma in the form of small granulomas have been described in biopsy material.5 The microbleeding might also have been related to neurosarcoidosis in the present case.6 Additional support for the diagnosis of neurosarcoidosis was discovered. Over the following 44 months, the situation continued to improve while cortiosteroid theraphy was progressively reduced and finally stopped. Although he returned to independence in activities of daily living with stimulation (but not in instrumental activities of daily living), his cognitive deficit persisted (MMSE score 15). He was able to perform a description task from the Boston Diagnostic Aphasia Examination 7 and to read a text and the time on a clock, but his verbal fluency was poor, as well as his results on the Frontal Assessment Battery (5/18). In addition, some verbal (utterances repeated multiple times such as inappropriate laughter) and gestural (rubbing hands together; rubbing the face) stereotypies appeared and become almost permanent. He no longer exhibited disinhibited or aggressive behavior, but apathy persisted. The combination of a dementia syndrome of degenerative origin such as frontotemporal dementia (FTD) and pulmonary sarcoidosis could not be excluded.8 Normal CSF cytology does not exclude neurosarcoidosis, and positive oligoclonal bands are seen in at least 19% of neurosarcoidosis cases.9 Although FTD, which is a neurodegenerative disease with different behavioral subtypes, might easily explain the apathy and hypoperfusion in the frontal lobes, the inflammation and continuing improvement with corticotherapy are not consistent with this diagnosis. Moreover, during follow-up, as shown in Table 1, repeated measurements of CSF biomarkers at Months 3, 17, 21, and 27 showed variations in Tau and PhosphoTau-181 protein levels with subsequent normalization, whereas Aβ1–40 and Aβ1–42 levels remained abnormal. Although these CSF biomarker levels demonstrated acute cerebral stress and alteration of β-amyloid clearance, normalization of Tau and PhosphoTau-181 were not indicative of Alzheimer's disease and even contributed to the exclusion of this diagnosis10 and of FTD.11 Variations in CSF biomarkers were relevant for determining the differential diagnosis between FTD with pulmonary sarcoidosis and sequelae of neurosarcoidosis with symptoms mimicking, which was the case here. In the present clinical case, the variation in CSF biomarkers levels probably indicates brain tissue damage associated with the inflammatory process. If this raises interest in measuring CSF biomarkers for the diagnosis of rapidly progressing non-Alzheimer's dementia syndromes, further studies are necessary to better interpret value variations in Aβ1–40, Aβ1–42, Tau and PhosphoTau-181 proteins, although the measurement of CSF biomarkers would never replace pathological analysis of the brain tissue to confirm the origin of cognitive disorders. Conflict of Interest: None of the authors has any conflict of interest to declare. Author Contributions: MK, POL, RC, OB, TV, and GK wrote the manuscript. Sponsor's Role: There was no sponsor for this manuscript.

Laughter and collective awareness: The cinema auditorium as public space

This article looks at how the collective experience of laughter in the movie theater is related to the idea of the cinema as a public space. Through the non-verbal expression of laughter the audience ‘constructs’ a public space the viewers may not have been aware of to the same degree prior to the collective public expression. Moreover, the public space created through laughter allows for an expedient type of monitoring: inappropriate laughter may be exposed in front of others. With viewers who laugh approvingly about racist violence or misogynist jokes, we can easily lay bare the ethical implications.

'Face of giant panda': a rare imaging sign in Wilson's disease

A 26-year-old girl presented with fever and jaundice at 17 years of age followed by generalized tonic clonic seizures, right hand postural tremor, reduced school performance, inappropriate laughter and slowness of activities 2 years later. She also reported dystonic posturing of the right upper and lower limb with drooling of saliva. She had a positive Kayser–Fleischer …

Screening of UBE3A gene in patients referred for Angelman Syndrome

Angelman Syndrome (AS) is a neurodevelopmental disorder characterized by severe developmental delay, speech impairment and unique behaviors including inappropriate laughter and happy disposition. AS is related to deficient maternal UBE3A gene expression caused either by chromosomal deletions, uniparental disomy, molecular defects of the imprinted 15q11-q13 critical region or by loss of function mutations in the maternally inherited UBE3A. In the present study, screening UBE3A was performed in 43 patients who were referred for AS but whom previous molecular diagnostic tests failed to provide a diagnosis. Two causative mutations – one of them novel – and four polymorphic variants one of which is also novel were revealed. Further investigation of 7 patients disclosed defects in other genes involved in clinical phenotypes mimicking AS. A typical EEG pattern and microcephaly in patients with developmental delay prompt for AS investigation while wide genetic screening should be applied to help resolution of the complex phenotypes characterized by developmental delay.