Klazomania

Klazomania (from the Greek "klazo"-to scream) refers to compulsive shouting; it has features resembling complex tics, similar to echolalia, palilalia and coprolalia, although this classification is somewhat difficult because tic disorders "may be challenging to differentiate from compulsions".

Klazomania has features similar to tic disorders; it has been seen in people with encephalitis lethargica, alcohol abuse and carbon monoxide poisoning. It was first reported by L. Benedek in 1925 in a patient with postencephalitic parkinsonism.

Classification

Klazomania shares some features with tics, with similar neural activity to other tic disorders including Tourette syndrome. Compulsions are a result of an obsessive thought that brings mental anxiety if the thought is not acted upon. Compulsions and complex tics, such as klazomania, are difficult to differentiate from each other and share some similar neural pathophysiology. Disorders where compulsive behavior is exhibited are obsessive-compulsive disorder (OCD) and autism. There is disagreement whether klazomania is considered part of the spectrum of tic disorders or due to other infectious (encephalitic) processes.

Signs and symptoms

Klazomania is similar to other complex tics including echolalia, palilalia and coprolalia. It is defined as compulsive shouting, which can be in the form of swearing, grunting or even barking. The subject may appear flushed, and klazomania can occur with increasing frequency if the person is agitated. The duration of the incident depends on the individual, but it can be characterized by a peak period, followed by intermittent remissions of less intensity. Although the individual may sound like they are in pain, there does not appear to be any actual physical discomfort. The shouting can be accompanied by other symptoms, such as oculogyric crises or other involuntary movements. The presentation of klazomania is comparable to temporal lobe epilepsy, although the two can be distinguished by the duration of the attack and the fact that the patient experiencing klazomania appears to retain consciousness.

A 1961 report desribed a postman known as K.R. who contracted encephalitis lethargica at the age of 12. While he reported no significant ill effects from the disease, he was irritable and complained of fatigue for years after recovering. At 22, the patient received a head injury, though he did not sustain a concussion or cranial fracture from the incident. Six months later, he developed oculogyric spasms, as well as dyskinesias of the mouth and tongue. In 1957, at the age of 44, the patient experienced his first bout of klazomania while working in the post office. He remained conscious for the entire incident, while he shouted for about half an hour and appeared "crazy" for hours after the shouting ended. The next day, he felt better, though he did report being tired. The patient continued to suffer from the attacks for the next few years before coming under observation of Wohlfart and colleagues. He subsequently served as a model to describe klazomania from beginning to end.

Onset is sometimes characterized by absentmindedness: the patient K.R. stared straight ahead and only responded in monosyllables in the minutes leading up to the incident. An oculogyric spasm then developed, during which he demonstrated [[echolalia. After 15 minutes, further motor symptoms arose, with the patient making small jerky motions with his arms that developed into larger, circular movements. At 20 minutes, the attack reached its peak, with the patient becoming bright red and making large compulsive movements with his arms and kicking his legs. He began swearing, shouting, screaming, grunting and barking loudly, with intermittent bouts of heavy panting. He remarked upon the people present, with his comments being related to the situation in question. He attempted at times to excuse his behavior. Afterwards the patient was able to provide an account of what had happened. Wohlfart et al. concluded that the patient was aware of his surroundings during the attack, with the patient even expressing concern over missing a scheduled appointment; the patient demonstrated some ability to control his behavior when spoken to in a sharp tone, but he would inevitably return to his shouting and movements after a few seconds of stillness. The episode lasted an hour and a half and was accompanied by salivation, sweating, and tachycardia. The peak of the attack lasted 30 minutes; the intensity then started to subside, though the patient presented with intermittent remissions.

Causes

Although the cause of klazomania is unknown, researchers have hypothesized that klazomania is caused by an irritating lesion in the mesencephalon. It is believed that there is a malfunction in the control of the motor circuit from the substantia nigra in the mesencephalon to the globus pallidus in the striatum (mesostraital pathway). This circuit becomes overstimulated during a mesencephalic "fit", which could be caused by tumors in the posterior fossa comprised of the cerebellum, medulla, and pons.

Accounts of klazomania are similar to the tics of Tourette syndrome, although some patients with klazomania do not have the motor tics of TS. Alcoholism and infections such as encephalitis are accompanied by phonic tics and occasionally klazomania. It is hypothesized that the cause of klazomania is linked to the combined effects of brain damage due to alcoholism or encephalitis.

Pathophysiology

Changes are observed on EEG in the substantia nigra and the globus pallidus with klazomania. The mesostriatal pathway involving the substantia nigra and the globus pallidus are both dopaminergic pathways, and therefore it is believed that dopamine is one of the main neurotransmitters involved in klazomania. This belief is corroborated by the fact that dopamine antagonists, such as haloperidol, alleviate the symptoms of klazomania by blocking dopamine activity. Other brain abnormalities discovered with an EEG include slight cortical atrophy and a marginally enlarged lateral ventricle.

One hypothesis is that klazomania originates in the periaqueductal gray matter in the mesencephalon. The vocalizing center in animals is located in the periaqueductal gray matter and a klazomania-like episode involving grunts and animal sounds can be evoked by electrical stimulation of this region. This phenomenon can be linked to klazomania if it can be proved that humans also possess this vocalizing center.

Researchers have hypothesized that the stimulation of the autonomic nervous system by the posterior hypothalamus is involved in klazomania. Klazomania resembles sham rage in animals, which is controlled by stimulation of the sympathetic nervous system. During klazomania, a person may experience pupil dilation, tachycardia, salivation, increased blood pressure, retraction of lips, barking, grunting, and rage just as an animal would if presenting sham rage.

Diagnosis

EEG abnormalities are not observed during klazomania, and a link between klazomania and seizures is not likely.

Management

n large doses, atropine sulfate helped control the involuntary movements associated with klazomania in one patient; attempts to treat with a combination of phenobarbital and trihexyphenidyl (also known as Artane) were also made. Phenobarbital acts as an anticonvulsant and is generally used to treat seizures, while Artane is used to treat involuntary movements in Parkinson's disease; however, this combination was found to have no beneficial effect in treating klazomania. Klazomania does not respond to anti-epileptic medications.

History

The word "klazomania" comes from the Greek klazo, meaning "to scream". The term was coined by L. Benedek in 1925 when he witnessed bouts of compulsive shouting in a patient with postencephalitic parkinsonism. He reported that the attacks would last for up to several hours and seemed to be outside of the patient's control. He characterized the shouting as extremely loud, noting that it could be in the form of syllables, vowels or even animal noises. In addition, he observed that while the nature of the shouting could suggest that the patient was in pain, the sounds themselves were unrelated to any physical discomfort. He stated that the patient appeared to have the ability to anticipate an incident and could even prevent it through deep and rapid breathing. However, he noted that the effort required to suppress klazomania could be even more tiring than enduring it. He said that though anxiety could increase the frequency of klazomania, it did not affect the overall presentation.

Two of Benedek’s colleagues, E. Von Thurzó and T. Katona, recorded two further instances of klazomania in 1927. They expanded upon Benedek’s earlier observations, describing the angry flushed face of one patient, as well as extreme restlessness and agitation. They noted that afterwards the patient apologized for the incident, suggesting awareness of the behavior. From this, Thurzó and Katona proposed that there is no loss of consciousness during klazomania and that individuals may remain fully aware of their surroundings.

In 1961, Gunnar Wohlfart et al. reported a case of klazomania accompanied by oculogyric crises, another symptom of postencephalitic Parkinsonian syndrome. Klazomania was proposed to be associated with chronic alcohol abuse and carbon monoxide poisoning in 1996. Bates et al. reported on a 63-year-old who was admitted to a psychiatric hospital with a two-year history of sudden episodes of shouting. The man claimed to have no memory of the attacks, which he could anticipate by a few seconds. The episodes were characterized by shouting of "aagh" or "help" and he was reported to appear angry during the incidents. At the end of the outbursts he would appear surprised, though he was able to continue a conversation. The patient remained fully oriented between attacks. The episodes themselves occurred at a frequency of one or two a month, generally taking place in the evening and they got progressively worse from the time of their first presentation.

Research directions

While klazomania is associated with other complex phonic tics, research focused on the disease has suggested that the rare brain inflammatory disease encephalitis lethargica—and only a few individuals diagnosed with encephalitis lethargica placed under research observation—have exhibited klazomaniac symptoms; individuals suffering from a form of encephalitis also exhibited compulsive shouting (along with other behavioral disorders) as an apparent result of this neurological disease.

Research on klazomania is scarce. The demand for research for klazomania may not be as high as for other tics due to the relatively few cases of the symptom recorded, and further discouraged due to the overlap of klazomania symptoms with other tics of Tourette syndrome and other more common phonic tic disorders. Finally, difficulties in the passage of drugs through the blood brain barrier makes studying treatment possibilities for klazomania challenging.