Paroxysmal dystonia and pathological laughter as a first manifestation of multiple Sclerosis
Paroxysmal dystonia is an uncommon but well-established feature of multiple sclerosis (MS). Attacks can occur in established MS and may even occasionally be the initial symptom of this disorder.
Pathological laughter is usually seen as a pseudobulbar palsy in some diffuse neurological diseases, but cases have been described, mostly in ischaemic attacks or tumours, where it is presented as bursts of laughter of variable duration.
The pathogenesis of neither of the two phenomena has been fully established but both have been reported as being positive phenomena resulting from ectopic activation with ephaptic spread.
Bonnie’s Research Results:
Definition:
Paroxysmal dystonias
Paroxysmal dystonias are a group of rare conditions where attacks of dystonia occur, in between which people are usually completely normal. Sometimes there are triggers to these attacks such as sudden movement, fatigue, coffee and alcohol.
In some types of paroxysmal dystonia the attacks are very brief (seconds to minutes), whereas in others the attacks can be much longer (minutes to hours). Some people have many attacks per day, whereas others have very infrequent attacks.
During an attack, people do not lose consciousness, and are completely aware of their surroundings. Paroxysmal dystonias do not affect the mind or the sense. Paroxysmal dystonias are not the same as epilepsy, and therefore do not necessarily disqualify one from driving (although advice should be sought from the driving licence authority, DVLA).
In most people the condition has no known cause and usually starts in teenage years. Sometimes the condition can be caused by damage to the brain, for example following a head injury. The condition can occasionally run in families.
How can paroxysmal dystonias be treated?
To date, no cure exists for people with paroxysmal dystonias, although a great deal of research is being undertaken around the world, with significant progress. The condition is not life-threatening but treatment is essential and usually available using drugs.
Although paroxysmal dystonia is not the same as epilepsy, many people can be very successfully treated using small doses of the same drugs used to treat epilepsy. In some people these drugs can stop attacks completely. Drugs are not universally effective, and some people do experience side effects.
How do I live with paroxysmal dystonia?
Paroxysmal dystonia can be a challenging condition to live with. The attacks can be quite unpredictable, and this can interrupt normal life. Paroxysmal dystonia is quite a rare condition, and therefore most people, including some doctors, will not have heard of it. People can become confused between paroxysmal dystonia and epilep
Pathological laughter and crying
Patients with pathological laughter and crying (PLC) are subject to relatively uncontrollable episodes of laughter, crying or both. The episodes occur either without an apparent triggering stimulus or following a stimulus that would not have led the subject to laugh or cry prior to the onset of the condition. PLC is a disorder of emotional expression rather than a primary disturbance of feelings, and is thus distinct from mood disorders in which laughter and crying are associated with feelings of happiness or sadness. The traditional and currently accepted view is that PLC is due to the damage of pathways that arise in the motor areas of the cerebral cortex and descend to the brainstem to inhibit a putative centre for laughter and crying. In that view, the lesions `disinhibit' or `release' the laughter and crying centre. The neuroanatomical findings in a recently studied patient with PLC, along with new knowledge on the neurobiology of emotion and feeling, gave us an opportunity to revisit the traditional view and propose an alternative. Here we suggest that the critical PLC lesions occur in the cerebro-ponto-cerebellar pathways and that, as a consequence, the cerebellar structures that automatically adjust the execution of laughter or crying to the cognitive and situational context of a potential stimulus, operate on the basis of incomplete information about that context, resulting in inadequate and even chaotic behaviour.
Definition:
Pseudobulbar palsy
Pseudobulbar palsy refers to a group of symptoms—including difficulty with chewing, swallowing, and speech, as well as inappropriate emotional outbursts—that accompany a variety of nervous system disorders.
Description
Pseudobulbar palsy refers to a cluster of symptoms that can affect individuals suffering from a number of nervous system conditions, such as amyotrophic lateral sclerosis, Parkinson's disease, stroke, multiple sclerosis, or brain damage due to overly rapid correction of low blood sodium levels.