Bell's mania

When examined, patients with Bell's mania fail to recall names, recent experiences and are poorly oriented for location, date, and time.[12] Moreover, their blood pressure and respiratory rate are increased.[13] Additionally, mental status examination using questionnaires[13] and three diagnostic tests are taken, including drawing a clock-face,[14] The Face-Hand test[15] and Hidden figures tests.[16] Patients with Bell's mania tend to make obvious mistakes in these tests, for instance drawing a clock-face with incorrect numbering or missing clock hands.[14]

Upon acute onset of the symptoms, an instant investigation for a toxic or systemic cause is undertaken.[12] Prominence of thought disorder, grandiosity and delusional ideation, and catatonic signs indicates the diagnosis of acute schizophrenia, bipolar disorder and catatonia respectively.[12] Diagnostic complications arise as these signs are also often the notable feature of Bell's mania.[8] With the cause undetermined, Bell's mania diagnosis is usually justified with the presence of both mania and delirium regardless of the catatonic symptoms.[12]

Bell's mania and catatonia are regarded as "overlapping syndromes",[16] making differential diagnosis essential when catatonic signs are observed.[12] Thus, researchers must distinguish between excited catatonia and Bell's mania, and among malignant catatonia, excited catatonia, and neuroleptic malignant syndrome (NMS).[12] When catatonic features are prominent, it is diagnosed as excited catatonia and when absent or subtle, it is identified as Bell's mania.[12] Alternatively, the presence of delirium is recognized as the discerning factor. A difference between the two is that catatonia is viewed from a movement aspect, whereas delirium from consciousness.[7]

Nevertheless, a formal set of diagnostic criteria is required to distinguish between Bell's mania and catatonia.[11] Failure to diagnose Bell's mania appearing as catatonia could lead to deleterious consequences and, in worse cases, death.[1][8]

Mania is a prominent symptom of both bipolar disorder and Bell's mania. Hence, studying bipolar patients can provide insight into the pathophysiology of this Bell's mania. PET scans of manic patients illustrate anomalous activation of the dorsal anterior cingulate, right inferior frontal cortical regions.[6] Manic symptoms exacerbate with increasing anterior cingulate activation, which is posited to be associated with escalating dopamine transmission in the nucleus accumbens.[3]

Dopamine transporter regulates the reuptake of dopamine to keep the synaptic dopamine levels within normal range.[6] Hence, the elevation of such transporter levels in the striatum decreases neurotransmission.[18] Genetic studies have hypothesized a relationship between low transporter protein levels and the gene for dopamine transporter in bipolar affective patients.[6]

External sources contributing to the hypothesized hyperdopaminergic mechanism include psychostimulants like cocaine.[6] These substances provoke behavioral changes similar to mania.[19] In chronic users, drug sensitization occurs which induces increased neurotransmission and modified protein expression within the mesolimbic dopamine neurons. Adaptations in dopamine transporters is further triggered causing behavioral sensitization.[20] This phenomenon is not distinct to drug abuse but also other psychomotor stimulants such as stress.[6]

Dopaminergic transporters dysfunction is caused by acute mania of bipolar disorder, psychostimulant use, and environmental stress. It is suggested to be common mechanism in excited delirium (EXD).[6] EXD is commonly observed in psychostimulant abusers as these drugs directly impact the dopaminergic transporters, increasing the extracellular dopamine levels.[21][6]

Amplified excitation of the dopaminergic systems can induce extreme fear and magnify both approach and avoidance behaviors.[6] The hyperdopaminergic state triggers aggression, agitation and psychomotor excitement.[6] Additionally, CNS dopamine signaling is active in heart rate, respiration and body temperature regulation.[22] Dopamine imbalance can hence result in hyperthermia, tachycardia, hyperventilation, hypertension and sleep disturbance symptoms.[6][3]

Over the course of time the significance of this syndrome has been increasingly recognized in correspondence to the manner of death, specifically because the anatomic cause of death is hard to define during autopsy. Recent studies have elicited neurochemical imbalances[24] resulting in autonomic hyperactivity and increase in dopamine levels in the victims.[3] Emergency personnel need to recognize these symptoms promptly to avoid the individual from spiraling into metabolic acidosis, rhabdomyolysis, multiorgan failure and ultimately death. In light of the clinical findings, some treatments have been described which include effective sedation, followed closely by external cooling, monitoring medical complications and the administration of intravenous (IV) fluids.[3]

Benzodiazepines are a type of tranquilizers used in the treatment of Bell's Mania that take effect by acting on GABA neurotransmitters in the brain. It helps in bringing extreme agitation and catatonia under control.

One management technique is rapid sedation in view of the unpredictable aggressive nature of the patient with Bell's Mania, especially if the symptoms that need to be handled are associated with the causes like dopamine regulation. Turning off the catecholamine cascade and rapidly sedating the patient using several sedatives like Benzodiazepines or Neuroleptics[25] can help. Several studies also point at the increased effectiveness of combination of two or more sedatives in the treatment of hyper agitated patients.[26][27]

The use of Intramuscular Ketamine Injections is a popular method in the treatment of Bell's Mania.

Patients with Bell's mania may not have optimum time for the sedatives to start showing effect. Due to this fact, electroconvulsive therapy[1] and Intramuscular Ketamine injections[3] are alternative solutions that have been proposed. With an onset time of 30 seconds to 4 minutes ketamine proves to be more effective than Benzodiazepines.[28] Although adult data on the use of Ketamine on patients isn't readily available, a study by Strayer et al.[29] concluded that the use of ketamine for controlling the hyperactivity was reliable and can further facilitate other management techniques with fewer side effects.[30]

Along with sedation techniques, a few other prevention and protection measures can decrease fatal outcomes, some of which are:

• management of catecholamine cascade,
• basic medical monitoring of bodily functions,
• blood tests,
• physical examination and
• rapid cooling of the body temperature.[3]

The urgency and medical severity of the condition needs to be given impetus in terms of handling patients with Bell's Mania. Due to the homologous nature of this syndrome with malignant hyperthermia (MH) and neuroleptic malignant syndrome (NMS),[31] Dantrolene[32] is also a probable treatment route owing to its swift acidosis correction. Although more research is needed in correspondence to the cause and consequences of this disease, the significance of the behavioral and physical symptoms need to be given importance to provide medical institutions as well the constabulary the necessary information to respond to Bell's Mania appropriately.

Bell's Mania is a syndrome with unexplained etiology which was first explained by American psychiatrist Luther Bell in the 1850s after observing institutionalized psychiatric patients. The first clinical reports and descriptions of people with acute exhaustive mania and delirium were provided by a few psychiatrists in the United States of America, France and the United Kingdom.[6] The description of the symptoms seemed to be quite similar to that of patients with schizophrenia (hallucinations and delirium) however additional hyperactivity, heightened arousal, and altered sleep cycle was also reported in patients with Bell's Mania.[6]

The suggestive symptoms of this disorder were first observed in the 19th century, out of which some of the most significant ones are the onset of aggression, bizarre behavior, violence, excessive shouting, panic, paranoia and increase in body temperature.[4] In 1934, Stauder described a series of acute onset of psychomotor agitation in young people with no history of physical or psychological disturbances.[35] He termed this description as Lethal Catatonia. Other reasons for the manifestation of Bell's Mania, points at the use of stimulant drugs in excessive amounts and also psychiatric diseases like depression or schizophrenia.[12][36]

In 1985, Bell's Mania was first mentioned in a definitive manner using the term Excited Delirium (EXD). Prior to that year, most cases of death by cocaine intoxication[37] during the cocaine epidemic that happened in a sudden manner. This involved the exposure to highly toxic amounts of the drug due to the bursting of cocaine packets being carried within the body by "body stuffers".[38] In the same year a series of observations were made by Welti and Fishbain regarding psychosis, cardiorespiratory arrest and sudden death[39] in individuals with cocaine addiction. Since the law enforcement were often called to contain violent behavior exhibited by these individuals. It was speculated that police brutality might be the underlying cause of the misshapen. Upon medical review of the cases related to the use of batons, pepper sprays and restraint methods[38] did not disclose any autonomic cause behind the death, albeit problems like cardiac diseases and trauma was excluded from the extensive evaluation.[38][4][5]