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From the philosophy auditorium to the neurophysiology laboratory and back: from Bergson to Damasio.

Henri Bergson (1859-1941) was probably the most influential French philosopher at the turn of the twentieth century. In 1927 he was awarded the Nobel Prize for literature. Far beyond the restricted academic philosophical milieu, the impact of his thinking reached personalities as diverse as Claude Debussy, Marcel Proust, George Bemard Shaw, and the impressionists. His essay The Laughter (Le Rire) is one of the most profound and original ever written on the sense of humor. Bergson's opinions, with their emphasis on life, instinct and intuition, represented a deviation from the rationalist mainstream of western philosophical tradition. In some circles he was received with skepticism and irony, as in Bertrand Russel's History of Western Philosophy. Today, unbiased by theoretical "bergsonism," neurophysiologic research--as undertaken mainly by Antonio Damasio's team at Iowa University--confirms many of his hypotheses and elucidates their mechanisms. In this new light, intuition and "recognition by the body" should not be seen as the personal fantasy of an original thinker but as fundamental cognitive tools.

Agnosia↗

A joke in a dream. A note on the complex aesthetics of disguise.

It was Freud's belief that while jokes and dreams have much in common, the comic elements that appear in dreams are not truly funny: unlike "real" jokes, they leave us cold. What, then, are we to make of the appearance in a dream of a well constructed joke that would produce laughter in and of itself even beyond the context of the dream? Such an example is cited and the reasons for the rarity of the phenomenon, as well as its multiple meanings, are explored.

Dreams↗

Age-related occurrence of signs and symptoms in the Rett syndrome.

The occurrence of signs and symptoms in the Rett syndrome (RS) was analyzed in a series of females born 1945-87 (median age 17 1/2 years) and fulfilling the diagnostic criteria for classic RS. For general information, data from 91 girls and women were used (group A), while the more detailed analyses were based on three age related subgroups: the youngest 20, born 1980-87 (group B); the 34 girls born 1970-79 (group C); and the oldest 37, born 1945-69 (group D). Data from group A indicated a developmental stagnation (stage I) at median age 11 (5-24) months and loss of ability to use acquired skills (stage II) at 19 (12-36) months. Group B displayed subtle prodromes in the first months of life, and later in infancy gross motor delay with insufficient equilibrium control. Development invariably came to a definite break at a crucial stage of maturation and was followed by a remarkable "awakening" and return of interest to act and interact (stage III). Loss of skills belonged to the triad contact/communication, hand use/skill, and babble/words. By age 2 1/2 years, apraxia and involuntary and stereotyped movements, were found in all. Seizures, hyperventilation and spells of screams and laughter were more frequent in group C (94%, 65% and 71%, respectively), and breathholding, bloating and drooling in group D (73%, 43% and 81%, respectively). Plantar flexion and abiotrophy of feet, as well as peroneal weakness and scoliosis, increased with age and was found in 89%, 86%, and 89%, respectively. The sequence of events described, emerged as subtle insufficiency and more or less abruptly turned into loss. Conversely to known progressive encephalopathies, the deterioration was followed by excess of activity, only years later to turn into restriction.

Adolescent↗

The use of the supra-pubic fibrous complex in isthmic hysteropexies.

This study forwards a new surgical approach we have performed in the treatment of genital prolapse, associated or not with stress urinary incontinence. The study used 150 patients with genital prolapse and stress urinary incontinence in various stages, and lasted from 1985 until 2001. Four-five threads of silk were passed through the uterine muscle in the isthmus area, just below the bladder-uterus recess, and fastened to the supra-pubic ligament complex. Although the mechanical basis for this surgical approach is not entirely clear, no relapse, incident or post-surgery complications (bladder voiding problems, enteroceles, detrusor instability, urethra or bladder bottom lesions, incomplete urinary retention or clearance, hematoma or abscess of the Retzius space) were noted in any of the studied cases. The suspension of the uterine isthmus on to the supra-symphyseal fibrous complex leads to an increase in urethral closure pressure during the occasional increase of intra-abdominal pressure (cough, laughter, spontaneous movements, walking), the results being superior to the other methods used for treating genital prolapse.

Female↗

The anatomy and embryology of the hypothalamus in relation to hypothalamic hamartomas.

The hypothalamus is involved in a variety of autonomic, endocrine, neurological and behavioural functions including temperature, osmostatic and autonomic nervous system regulation, pituitary, thyroid, adrenal and gonadal control, thirst, appetite and weight control, memory and emotional behaviour including aggression and laughter, and biological (circadian) rhythms. The functional anatomy of the hypothalamus and its major afferent and efferent neurological connections are described, with particular reference to hypothalamic hamartomas (HH), gelastic seizures, MRI of the hypothalamus, and potential effects of surgery for HH. Normal development of the hypothalamus is reviewed in relation to models of forebrain development, descriptive hypothalamic embryology and the importance of known transcription factors. Potential environmental antecedents to HH development are discussed, and the significance for sporadic, isolated HH of several syndromes associated with HH is explored.

Animals↗

[The symptomatic treatment of amyotrophic lateral sclerosis].

Patients with amyotrophic lateral sclerosis (ALS) have symptoms of progressive muscle weakness, of disturbed speech and swallowing, and in the terminal phase those of respiratory weakness. Treatment options, in particular those for excessive weight loss and respiratory weakness, should be introduced to the patients and their families when the patient is emotionally capable and before dysarthria severely hampers communication. Special equipment for keeping the patient as mobile as possible should be made available much earlier than in the case of other diseases of the muscles as in ALS progression is much faster. Cramps, pathological crying or laughter, spasms, and spasticity can all be treated by medication. When speech can no longer be understood, adaptive strategies such as sign language, mime, posture and communication apparatus varying from a note pad to advanced computer systems can be used. Sialorrhoea, caused by difficulty swallowing with its accompanying danger of aspiration can be halted by the use of medication, by radiotherapy and by the injection into the salivary glands of botulin A toxin. Weight loss, also a result of dysphagia, can be avoided by eating frequent small meals or if necessary performing a percutaneous endoscopic or radiological gastroscopy. Excess mucus in the respiratory tract can be treated with anticholinergics. Difficulty in coughing up thick and sticky mucus cannot always be adequately helped. Respiratory weakness is treatable by external respiratory supportive therapy using a nasal mask, as well as invasive respiratory support via a trachcostoma and by treating the symptoms of respiratory weakness. The latter form of treatment is palliative and forms part of terminal care. During the terminal phase restlessness, anxiety, pain, and dyspnoea require the most attention. Treatment requires careful multidisciplinary cooperation.

Amyotrophic Lateral Sclerosis↗

Primary progressive aphasia as the initial manifestation of corticobasal degeneration. A "three in one " syndrome?

In 1994, the term "Pick complex" was proposed to indicate significant clinical and pathological overlapping between primary progressive aphasia, frontal lobe dementia and corticobasal degeneration. We report the case of a 60-year-old man, who initially presented progressive non-fluent aphasia with orofacial apraxia, and subsequently, over a period of 3 years, developed mutism, pathological laughter, extrapyramidal rigidity, dystonia, alien hand syndrome and bulbar signs. An extensive haematological, immunological and biochemical work up was normal. The results of neuroimaging studies and neuropsychological tests, along with the clinical evolution, finally led us to the ?three in one? diagnosis, supporting the concept of Pick complex.

Aphasia, Primary Progressive↗

Selective serotonine reuptake inhibitors prevents emotional lability in healthy subjects.

BACKGROUND: Many subjects with depression and with brain lesions can poorly control their emotions with fits of weeping and tearfulness; neurological patients present outbursts of laughter as well. This condition is called Emotional Lability (EL). The antidepressant drugs of the family of selective serotonine reuptake inhibitors (SSRI) improve EL within a few days in both depressive and neurological disorders. EL can be present in healthy subjects as well, in whom it is considered as normal, although often embarrassing. METHODS: Two healthy subjects with EL, were treated with 20 mgs of Paroxetine or placebo for cycles of 5 days. Moreover the effect was observed of either Paroxetine or Fluoxetine on the emotion control of three patients with mood disorders both when they were depressed and after recovering from the depression. RESULTS: In all subjects, after few days of treatment, EL disappeared, and their emotion control and behaviour were both modified. CONCLUSIONS: (1) In healthy subjects EL is often embarrassing; the possibility is interesting of preventing it on selected occasions with a brief treatment with no side effects and a cheap cost. (2) SSRI are among the most used drugs in the word and every day they are assumed by millions of people including politicians, business man, soldiers, army commanders, policemen and criminals. The idea is very stimulating and highly worrying that the control of the emotions and behaviour of these million of people can be quickly modified by the assumption of one pill of SSRI for a few days or by its discontinuation.

Adult↗

Life enrichment for long-stay patients in acute care: an interdisciplinary program.

Mr. T's stay on a medical unit became much more meaningful and enjoyable for him as a result of the LEP. Patient Focus sessions, which included input from his family, elicited several changes. Reassessments revealed that he could walk short distances with assistance, hence, he was placed on a toileting regime and he remained continent. His volunteer devoted at least two visits a week to him, and as the communication patterns improved, he was able to be taken to the coffee shop regularly. Furthermore, his hidden talents were discovered in recreation sessions and he soon started bringing samples of artwork to his room to be posted on his bulletin board. Smiles and laughter, instead of anger, became the norm when people greeted him in his chair by the nursing station. The team felt that Mr. T had reached nursing home level of care but, unfortunately, his history of aggressive behaviour tended to make nursing home administrators wary and non-receptive. To date, the consensus of the health care teams which are involved with the LEP is that 45 minutes every two weeks for discussion and planning for a selected long-stay patient has been time well spent. The teams are proud of their collaborative efforts and achievements. Most of all, the patients and their families are pleased with the improved quality of life that has emerged.

Aged↗

Diversity in the imaging workforce.

Culture is the coherent, learned, shared view a group of people has about life's concerns that ranks what is important, and instills attitudes about those things which are considered appropriate as well as prescribed behavior. It notes that some things have more significance than others. "Culture shock" is what happens when a person suddenly finds himself or herself in a place where "yes may mean "no," where a fixed price is negotiable, where to be kept in an outer office is not a cause for insult, and where laughter may signify anger Effective leaders should be able to vary their styles of management, such as leading, planning, organizing, motivating, decision making, staffing, and more importantly, communicating with groups of workers from different cultural backgrounds. Leaders need to perceive conflict as both a challenge and an opportunity. Awareness about team members' cultural similarities and differences is key to foster effective conflict resolution. Synergistic organizations transcend the distinct cultures of the team members. They use these differences as a resource in designing and developing organizational systems. Effective leaders must become a champion and capable of acting in many ways, not experts rigidly adhering to a single approach.

Conflict, Psychological↗

Hamartomas of the tuber cinereum: CT, MR, and pathologic findings.

The neuroimaging studies, clinical evaluations, and surgical and pathologic findings in five children with biopsy-proved hamartomas of the tuber cinereum were reviewed. Surgical and/or MR findings showed that patients with precocious puberty had pedunculated lesions while those with seizures had tumors that were sessile with respect to the hypothalamus. The radiologic studies included six MR examinations in four patients and CT studies in all five patients. Three children presented with precocious puberty and two with seizures, one of which was a gelastic (spasmodic or hysteric laughter) type of epilepsy. MR studies were obtained both before and after surgery in two patients, only preoperatively in a third patient, and only postoperatively in the fourth child. MR was superior to CT in displaying the exact size and anatomic location of the hamartomas in all cases. The mass was isointense with gray matter on sagittal and coronal T1-weighted images, which best displayed the relationship of the hamartoma to the third ventricle, infundibulum, and mammillary bodies. Intermediate- or T2-weighted images showed signal characteristics of the hamartoma to be isointense (one case) or hyperintense (two cases) relative to gray matter. The difference in T2 signal intensity did not correlate with any obvious differences in histopathology. CT showed attenuation isodense with gray matter, and no calcium. There was no enhancement on CT. There was no enhancement on MR in the one case in which contrast medium was administered. Preservation of the posterior pituitary bright spot was noted on all pre- and postoperative T1-weighted MR scans.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

Magnetic resonance imaging of hypothalamic hamartomas causing gelastic epilepsy.

Hypothalamic hamartomas may cause a peculiar epileptic syndrome characterized by seizures of laughter and precocious puberty. Four mentally handicapped patients suffering from gelastic epilepsy were referred to our institution for investigation; three of them also presented with precocious puberty. In all four cases magnetic resonance imaging (MRI) revealed a space-occupying lesion of the hypothalamus that was considered to be a hamartoma. Biopsies were not performed. Hamartomas appear isodense in plain computed tomography scans, and they do not enhance. Such lesions display an isointense signal in T1-weighted magnetic resonance images and a hyperintense signal in proton density and T2-weighted images. MRI is the procedure of choice for detecting such lesions at the base of the brain.

Adolescent↗

[Angelman's happy puppet syndrome].

The Angelman ('happy puppet') syndrome is clinically characterized by severe mental retardation without any development of speech, a happy disposition with paroxysms of laughter, a stiff-atactic gait with arms in flexion and abduction, epileptic seizures, EEG-abnormalities, and some dysmorphic features like prognathism, macrosomia, tongue protrusion and brachycephaly and/or microcephaly. The genetic background is still obscure, but in some patients with this syndrome small deletions in the long arm of chromosome 15 have been found. So further investigation of this probably rather frequent syndrome seems appropriate.

Child↗

Opening doors for the child "inside".

Music, storytelling, and humor are increasingly recognized as appropriate and effective interventions to help children cope with illness, hospitalization, and pain. Two pediatric nurses created an opportunity to collaborate on a project that offers songs, stories, and laughter to help children talk about the many emotional issues raised during hospitalization.

Child↗

[Clinical study of butane gas abuse: in comparison with toluene-based solvent and marihuana].

We reported 2 cases of patients who abused butane gas, toluene-based solvent and marihuana. They showed different signs in the each substance, respectively. Butane gas was easier to make visual hallucinations and distorted perception of body form, and was less potent and addictive than toluene-based solvent. Spontaneous laughter and the most amotivational state were characterized by marihuana intoxication. Alteration of auditory perception that simple music sounded wonderful was also experienced. Furthermore, the above symptoms were thought to change by the order of taking the substance. Therefore, it is needed to examine the order of the use of drugs and clarify differences of symptoms in abuse among drugs, respectively.

Adolescent↗

[Gelastic and cursive epilepsy].

When the most prominent ictal symptom in an epileptic seizure is laughing or running the condition has been termed respectively gelastic or cursive epilepsy. The article presents five patients with these ictal symptoms and discusses their clinical and EEG-features, etiology, and localisation of lesions responsible for the seizures. Laughter and running as ictal symptoms are usually part of complex partial seizures involving limbic structures or their efferent or afferent pathways.

Adult↗

The development of mania and organic euphoria during ECT.

Mania that develops in depressed patients who are undergoing ECT is relatively uncommon and may occur more frequently in bipolar patients. The authors' review of three cases illustrates the importance of the clinician distinguishing mania from organic euphoric states that can also develop during ECT. Severity of cognitive impairment and the presence of silly or inappropriate laughter are useful in making this distinction. Capacity to clinically evaluate the persistence of depressive symptoms should determine whether ECT can continue when an organic euphoric state develops.

Adult↗

Lithium carbonate in the treatment of two patients with infantile autism and atypical bipolar symptomatology.

Two patients with infantile autism by DSM-III criteria and with atypical bipolar symptomatology were treated with lithium carbonate. Both children demonstrated a significant response with levels above 1.0mEq/liter. Factors that may be useful in discovering patients with autism who may respond to lithium treatment include a family history of bipolar illness; extreme hyperactivity not responsive to a stimulant; a definite cyclic component to symptomatic behaviors; sustained laughter, irritability, or giddiness that is not stereotypic; and/or the presence of many or all of the symptom criteria for bipolar disorder.

Autistic Disorder↗