Klazomania
Encyclopedia
Klazomania refers to compulsive shouting; it has features resembling the complex tic
Tic
A tic is a sudden, repetitive, nonrhythmic, stereotyped motor movement or vocalization involving discrete muscle groups. Tics can be invisible to the observer, such as abdominal tensing or toe crunching. Common motor and phonic tics are, respectively, eye blinking and throat clearing...

s such as echolalia
Echolalia
Echolalia is the automatic repetition of vocalizations made by another person. It is closely related to echopraxia, the automatic repetition of movements made by another person....

, palilalia
Palilalia
Palilalia is the repetition or echoing of one's own spoken words. It can be a complex tic, like echolalia and coprolalia and may sound like stuttering; all can be symptoms of Tourette syndrome, obsessive–compulsive disorder, or autism...

 and coprolalia
Coprolalia
Coprolalia is involuntary swearing or the involuntary utterance of obscene words or socially inappropriate and derogatory remarks. Coprolalia comes from the Greek κόπρος meaning "feces" and λαλιά from lalein, "to talk"...

 seen in tic disorder
Tic disorder
Tic disorders are defined in the Diagnostic and Statistical Manual of Mental Disorders based on type and duration of tics...

s, but has been seen in people with encephalitis lethargica
Encephalitis lethargica
Encephalitis lethargica or von Economo disease is an atypical form of encephalitis. Also known as "sleepy sickness" , it was first described by the neurologist Constantin von Economo in 1917. The disease attacks the brain, leaving some victims in a statue-like condition, speechless and motionless...

, alcohol abuse and carbon monoxide poisoning
Carbon monoxide poisoning
Carbon monoxide poisoning occurs after enough inhalation of carbon monoxide . Carbon monoxide is a toxic gas, but, being colorless, odorless, tasteless, and initially non-irritating, it is very difficult for people to detect...

. It was first reported by L. Benedek in 1925 in a patient with postencephalitic parkinsonism
Postencephalitic parkinsonism
Post-encephalitic Parkinsonism is a disease believed to be caused by a viral illness, that triggers degeneration of the nerve cells in the substantia nigra. Overall, this degeneration leads to clinical Parkinsonism and the disease is followed by encephalitis lethargica, a condition also referred...

. Little is known about the condition, and few cases have been reported.

Classification

Klazomania shares some features with vocal tic
Tic
A tic is a sudden, repetitive, nonrhythmic, stereotyped motor movement or vocalization involving discrete muscle groups. Tics can be invisible to the observer, such as abdominal tensing or toe crunching. Common motor and phonic tics are, respectively, eye blinking and throat clearing...

s seen in tic disorder
Tic disorder
Tic disorders are defined in the Diagnostic and Statistical Manual of Mental Disorders based on type and duration of tics...

s including Tourette syndrome
Tourette syndrome
Tourette syndrome is an inherited neuropsychiatric disorder with onset in childhood, characterized by multiple physical tics and at least one vocal tic; these tics characteristically wax and wane...

 (TS). Klazomania was described in a 2006 journal review as a cause of tics differentiated from TS (tourettism
Tourettism
Tourettism refers to the presence of Tourette-like symptoms in the absence of Tourette syndrome, as the result of other diseases or conditions, known as "secondary causes"....

), attributed to infectious processes (encaphalitis) rather than TS. A 1996 case report on one patient by Bates et al suggested klazomania was a vocal tic.

Signs and symptoms

Klazomania is similar to other complex tics including echolalia
Echolalia
Echolalia is the automatic repetition of vocalizations made by another person. It is closely related to echopraxia, the automatic repetition of movements made by another person....

, palilalia
Palilalia
Palilalia is the repetition or echoing of one's own spoken words. It can be a complex tic, like echolalia and coprolalia and may sound like stuttering; all can be symptoms of Tourette syndrome, obsessive–compulsive disorder, or autism...

 and coprolalia
Coprolalia
Coprolalia is involuntary swearing or the involuntary utterance of obscene words or socially inappropriate and derogatory remarks. Coprolalia comes from the Greek κόπρος meaning "feces" and λαλιά from lalein, "to talk"...

. It is defined as compulsive shouting, which can be in the form of swearing, grunting or 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
Oculogyric crisis
Oculogyric crisis is the name of a dystonic reaction to certain drugs or medical conditions. The term "Oculogyric" refers to rotating of eyeballs, but several other responses are associated with the crisis.-Causes:...

 or other involuntary movements. The presentation of klazomania has been compared to temporal lobe epilepsy
Temporal lobe epilepsy
Temporal lobe epilepsy a.k.a. Psychomotor epilepsy, is a form of focal epilepsy, a chronic neurological condition characterized by recurrent seizures. Over 40 types of epilepsies are known. They fall into two main categories: partial-onset epilepsies and generalized-onset epilepsies...

, 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 by Wohlfart described a postman known as K.R. who contracted encephalitis lethargica
Encephalitis lethargica
Encephalitis lethargica or von Economo disease is an atypical form of encephalitis. Also known as "sleepy sickness" , it was first described by the neurologist Constantin von Economo in 1917. The disease attacks the brain, leaving some victims in a statue-like condition, speechless and motionless...

 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 dyskinesia
Dyskinesia
Dyskinesia is a movement disorder which consists of effects including diminished voluntary movements and the presence of involuntary movements, similar to tics or choreia. Dyskinesia can be anything from a slight tremor of the hands to uncontrollable movement of, most commonly, the upper body but...

s of the mouth and tongue. At the age of 44, the patient experienced his first bout of klazomania. 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.

According to Wohlfart's account of one patient, 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
Echolalia
Echolalia is the automatic repetition of vocalizations made by another person. It is closely related to echopraxia, the automatic repetition of movements made by another person....

. 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
Tachycardia
Tachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...

. The peak of the attack lasted 30 minutes; the intensity then started to subside, though the patient still exhibited bouts of shouting and movement after several minutes of remission. The remission periods between the shouting episodes became longer, until the entire attack was over in about an hour and a half.

Causes

Although the cause of klazomania is unknown, it is considered to be associated with encephalitis lethargica; a 2006 journal review by Jankovic and Mejia attributes klazomania to tourettism
Tourettism
Tourettism refers to the presence of Tourette-like symptoms in the absence of Tourette syndrome, as the result of other diseases or conditions, known as "secondary causes"....

 (tics not due to Tourette syndrome), widely seen after the encephalitic lethargica pandemic of 1916 to 1927.

Wohlfart (1961) hypothesized that klazomania is caused by an irritating lesion in the mesencephalon
Mesencephalon
The midbrain or mesencephalon is a portion of the central nervous system associated with vision, hearing, motor control, sleep/wake, arousal , and temperature regulation....

 and a malfunction in the control of the motor circuit from the substantia nigra
Substantia nigra
The substantia nigra is a brain structure located in the mesencephalon that plays an important role in reward, addiction, and movement. Substantia nigra is Latin for "black substance", as parts of the substantia nigra appear darker than neighboring areas due to high levels of melanin in...

 in the mesencephalon to the globus pallidus
Globus pallidus
The globus pallidus also known as paleostriatum, is a sub-cortical structure of the brain. Topographically, it is part of the telencephalon, but retains close functional ties with the subthalamus - both of which are part of the extrapyramidal motor system...

 in the striatum
Striatum
The striatum, also known as the neostriatum or striate nucleus, is a subcortical part of the forebrain. It is the major input station of the basal ganglia system. The striatum, in turn, gets input from the cerebral cortex...

 (mesostraital pathway). This circuit becomes overstimulated during a mesencephalic "fit".

In a 1996 report of one case, Bates et al. postulated that klazomania is similar to the vocal tics of Tourette syndrome
Tourette syndrome
Tourette syndrome is an inherited neuropsychiatric disorder with onset in childhood, characterized by multiple physical tics and at least one vocal tic; these tics characteristically wax and wane...

, although patients with klazomania may not have the motor tics necessary for a diagnosis of TS. Bates and colleagues observed a case where alcoholism and encephalitis
Encephalitis
Encephalitis is an acute inflammation of the brain. Encephalitis with meningitis is known as meningoencephalitis. Symptoms include headache, fever, confusion, drowsiness, and fatigue...

 were accompanied by vocal tics and occasionally klazomania. They hypothesized that the cause of klazomania is linked to the combined effects of brain damage due to alcoholism or encephalitis.

Pathophysiology

Jankovic and Mejia's 2006 review indicated that autopsies of victims of the 1917 to 1926 encepahlitis lethargica pandemic revealed "neurofibrillary tangles and neuronal loss in the globus pallidus
Globus pallidus
The globus pallidus also known as paleostriatum, is a sub-cortical structure of the brain. Topographically, it is part of the telencephalon, but retains close functional ties with the subthalamus - both of which are part of the extrapyramidal motor system...

, hypothalamus
Hypothalamus
The Hypothalamus is a portion of the brain that contains a number of small nuclei with a variety of functions...

, midbrain tegmentum
Tegmentum
The tegmentum is a general area within the brainstem. It is located between the ventricular system and distinctive basal or ventral structures at each level...

, periaqueductal gray
Periaqueductal gray
Periaqueductal gray is the gray matter located around the cerebral aqueduct within the tegmentum of the midbrain. It plays a role in the descending modulation of pain and in defensive behaviour...

 matter, striatum
Striatum
The striatum, also known as the neostriatum or striate nucleus, is a subcortical part of the forebrain. It is the major input station of the basal ganglia system. The striatum, in turn, gets input from the cerebral cortex...

, and the substantia nigra
Substantia nigra
The substantia nigra is a brain structure located in the mesencephalon that plays an important role in reward, addiction, and movement. Substantia nigra is Latin for "black substance", as parts of the substantia nigra appear darker than neighboring areas due to high levels of melanin in...

".

Wohlfart et al hypothesized 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. Wohlfart and colleagues hypothesized that the stimulation of the autonomic nervous system by the posterior hypothalamus is involved in klazomania, adding that klazomania resembles sham rage
Sham rage
Sham rage is behavior such as biting, clawing, hissing, arching the back and "violent alternating limb movements" produced in animal experiments by removing the cerebral cortex. It also describes rage seen in persons with hypothalamic brain lesions....

 in animals, which is controlled by stimulation of the sympathetic nervous system
Sympathetic nervous system
The sympathetic nervous system is one of the three parts of the autonomic nervous system, along with the enteric and parasympathetic systems. Its general action is to mobilize the body's nervous system fight-or-flight response...

. During klazomania, a person may experience pupil dilation, tachycardia
Tachycardia
Tachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...

, salivation, increased blood pressure, retraction of lips, barking, grunting, and rage just as an animal would if presenting sham rage. Bates and colleagues (1996) say that neuroimaging and pathology results do not support evidence of hypothalamic involvement similar to that found in sham rage.

Diagnosis

Jankovic and Mejia describe klazomania as due to tourettism
Tourettism
Tourettism refers to the presence of Tourette-like symptoms in the absence of Tourette syndrome, as the result of other diseases or conditions, known as "secondary causes"....

 (tics having Tourette syndrome-like features but not due to TS). According to the revised fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR), Tourette's may be diagnosed when the other diagnostic criteria are met and symptoms cannot be attributed to another general medical condition. Hence, other medical conditions that include tics or tic-like movements—such as tourettism
Tourettism
Tourettism refers to the presence of Tourette-like symptoms in the absence of Tourette syndrome, as the result of other diseases or conditions, known as "secondary causes"....

—must be ruled out before a TS diagnosis can be conferred. There are no specific tests; the diagnosis is based on history and symptoms.

According to Bates (1996), electroencephalography
Electroencephalography
Electroencephalography is the recording of electrical activity along the scalp. EEG measures voltage fluctuations resulting from ionic current flows within the neurons of the brain...

 (EEG) abnormalities are not observed during klazomania, and a link between klazomania and seizure
Seizure
An epileptic seizure, occasionally referred to as a fit, is defined as a transient symptom of "abnormal excessive or synchronous neuronal activity in the brain". The outward effect can be as dramatic as a wild thrashing movement or as mild as a brief loss of awareness...

s is not likely.

Treatment

In large doses, atropine sulfate helped control the involuntary movements associated with klazomania in one patient; attempts to treat with a combination of phenobarbital
Phenobarbital
Phenobarbital or phenobarbitone is a barbiturate, first marketed as Luminal by Friedr. Bayer et comp. It is the most widely used anticonvulsant worldwide, and the oldest still commonly used. It also has sedative and hypnotic properties but, as with other barbiturates, has been superseded by the...

 and trihexyphenidyl
Trihexyphenidyl
Trihexyphenidyl , also known as benzhexol, is an antiparkinsonian agent of the antimuscarinic class. It has been in clinical usage for decades...

 (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
Parkinson's disease
Parkinson's disease is a degenerative disorder of the central nervous system...

; 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
Postencephalitic parkinsonism
Post-encephalitic Parkinsonism is a disease believed to be caused by a viral illness, that triggers degeneration of the nerve cells in the substantia nigra. Overall, this degeneration leads to clinical Parkinsonism and the disease is followed by encephalitis lethargica, a condition also referred...

. 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.

One of the first instances in which an infectious disease was associated with klazomania was the notable pandemic of the encephalitis lethargica from 1916 to 1927. This pandemic also gave rise to observations of other tics that came to be associated with encephalitis lethargica such as complex vocalizations of blocking, echolalia, palilalia, and oculogyric crises.

In 1961, 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

Research on klazomania is scarce. While klazomania is associated with other complex phonic tics, research focused on the disease have explored klazomania associated with encephalitis lethargica individuals suffering from a form of encephalitis also exhibited compulsive shouting (along with other behavioral disorders) as an apparent result of this neurological disease.
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