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The tale of the storyteller and the painter: The paradoxes in nature

Introduction: Brain as the seat of behavior is acknowledged from the times of Charaka, however where neurology ends and philosophy begins remains an enigma. It is certainly every neurologist's observation that there is loss of function either region based or domain based in progressive diseases of the nervous system making it the seat of all useful activities. However, there are references to occurrence of new skills seen during various illnesses causing progressive cognitive dysfunction. This serves as a pharmaco-sparing agent in behavior management and therefore serves as a rehabilitatory tool. However, its pathomechanism is not clear. Patient and Methods: Two patients comprising one male and one female who were being evaluated for progressive cognitive dysfunction and were found to have interesting creative skills and are being described. Results: The first patient is a case of young onset behavioral variant frontotemporal dementia and the second patient is a case of neurosarcoidosis. Conclusion: The emergence of these skills could be due to disinhibition of some of the innate skills of the patients during degeneration or establishment of new data linking circuits with creative potential during attempted repair.


INTRODUCTION
Brain as the seat of behavior is acknowledged from the times of Charaka, the ancient Indian physician and he described the relationship between the brain and mind as that of a pot of copper filled with ghee; [1] so much interdependent that if one is heated, the other also heats up. The knowledge is the product of combination of objects (Indriya Artha), sense faculties (Indriya), mind (Manas), intellect (Buddhi), and soul (Atma); they are momentary and determinative. Hippocrates also felt brain is the seat of most of the behavioral functions. [2] However, knowledge of human behavior is remarkably limited in spite of continued attempts to explore these secrets. Mental states are clearly subjective, whereas the brain is an objective reality.

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Therefore, the study of human behavior had been an arena of the spiritual and philosophical pursuers and only recently, people have started searching for the seat of the mind-body interaction. Neuroscience can certainly not explain aspects of the several nonphysical realities but ablation studies and studies by function mapping indeed indicate brain as the seat of several complex human capacities. [3] We now have advanced enough to consider the science of neuropsychiatry. [4] This marks the firmer understanding neurobiological basis of behavior. [5] When a piece of poetry, art, etc., are seen, the question always arises, where in the brain does processes come, why does it to come to some people, and not all with no definite answers. The phenomenas which the observer experiences as creative may be the default skills present in the concerned person. However, remained suppressed because of the set goals which where probably prioritised. Therefore, the apparent creative function of the patient is likely to be a lower level human function which remained inhibited due to other lifestyle needs. Second hypotheisis is the creative skills seen may be truly new and useful and therefore a higher order cognitive function process through new data linking circuits formed during repair. [6] Greater is the mystery that shroud neuroregressive diseases when closely observed beyond the genes, cytoarchitecture, and chemistry into the great islands of wisdom that is seen in several of our patients who are considered ill by certain yardsticks but are extraordinarily well if looked at from a different perspective. Progressive cognitive dysfunction occurs in young people in a variety of neurological disorders. They include congenital, metabolic disorders, infective, inflammatory, vascular, autoimmune demyelinating, and degenerative causes. All these conditions are associated with loss of skills in a progressive or episodic way in a region specific or domain specific way based on the nature of illness. However, there is situation that the reverses are seen in the form of pseudonormalization of behavior for a short period, [7] development of creative skills, etc., which come to stay with our patients for a brief period. Their pathogenesis is variably postulated as disinhibition, regeneration, etc., we in this write-up report two such cases which astonished us.

PATIENT AND METHODS
Two young persons in the course of a neurodegenerative process developed paradoxically new talents and their details are discussed.

Case 1
A 36-year-old female nurse working in Dubai was brought to us by her parents for some behavioral problems, for which she was diagnosed as having bipolar affective disorder. Although first-hand information was not available to us from the husband, we were told that the problem started after she was asked to resign her high profile job by her husband to look after his parents in India. She was eating very often in large quantities; she was obsessed with eating nonvegetarian food very frequently. She will plead for the food with her parents as if very hungry. She showed personality change in the sense that she was not wearing matching dresses any more, she was not cooking at all, she showed tendency to wander away whenever the family was less cautious. She was adamant in getting her demands completed and claimed she has divine powers. She was found to be praying often and was not showing interest in the activities of children. She was being treated as suffering from bipolar affective disorder. Her parents observed a very interesting behavior in their daughter. She was entertaining her visitors whoever it may be by non stop story telling. When one story is over, she will drift to the next. The stories were constructed in English with proper grammar. She could read, write in her mother tongue, and comprehend well and her HMSE score was full. However, she will always drift to storytelling with similar themes and minor change in characters. The heroine was always herself as a nurse with extraordinary talents and integrity. The stories always involved people of great social standing such as the King of Arabia, Ambassador from the USA, and high profile couple from Canada and Delhi, one story lead to the other nonstop even when interrupted and requested to stop. In a day-to-day basis, the members in the storytelling changed. However, all stories expressed grandiose ideas about herself how she had been more efficient than doctors in diagnosis and treatment and how she was offered gifts by her patients who could not forget her timely interventions which saved their lives, but she never accepted anything from them and offered their gifts to the free donation box [Video 1]. Her children aged 9 years and 8 years are reported to be hostile and violent with mother and physically abusing her. Her magnetic resonance imaging showed diffuse atrophy with significant loss in frontal, temporal regions [ Figure 1].

Case 2
A 37-year-old man, a BITS Pilani graduate, was highly placed with a very heavy salary. He observed 11 years ago that his secondary sexual characters such as facial hair and voice were changing, for which he consulted a doctor without informing his parents. He was found to have low levels of testosterone and was treated with hormone replacement. However, no specific attempt was made to investigate the cause of his hypogonadism. At this point of time, his employers put him in a lower scale of work for unknown reason as the patient was living alone in a single room apartment. His parents thought that it could be due to depression as he was put in a lower cadre job. At this time, he was not communicating either with parents or friends but was continuously drawing. He drew excellent pictures of gods drawn in perfect accuracy with what he saw in books. They were  Figures 2-4]. Some were colored and some black and white. They contained only gods and famous people. Later, he was found to be clumsy in movement and his artistic talents declined slowly over the next 1 year. At this point, about 5 years after the onset of his hormonal dysfunction, his boss called the parents and informed them that he needs care. He was put in lower cadre job as he was slow in his job and made errors and failed to carry out the targets. Later, they had observed that he was walking with lot of dancing movements and found to suffer from frequent respiratory problems. At this point, his parents suddenly realized that their son is indeed ill and met several doctors and finally 9 years after his illness came to us. At that time, we found him with marfanoid body proportions, generalized choreiform movements and features of severe apathy and executive function impairment, dorsolateral prefrontal involvement, medial temporal involvement in the form of recent memory problems. He was investigated for Huntington's disease, mitochondrial disorders, cerebral autosomal-dominant arteriopathy with subcortical infarcts, and leukoencephalopathy, immune-mediated disorders including complete vasculitic workup, voltage-gated potassium channel, N-methyl-d-aspartate, glutamate decarboxylase antibody, and complete neurometabolic workup, all were negative. His MRI showed leukoencephalopathy and atrophy [ Figure 5]. Angiotensin converting enzyme levels were high, and therefore, the possibility of neurosarcoidosis was considered and the patient put on steroids and followed with azathioprine and symptomatic drugs. His choreiform movements improved very well. His cognitive deterioration got arrested. However, in spite of adequate precautions, he developed osteoporosis and fracture of right femoral neck which needed two surgeries. His primary illness remains stable.

RESULTS
Two patients comprising one with a young onset frontotemporal dementia (FTD) and another with a immune-mediated disease untreated and going for regressive course with features of creative work are being reported. The FTD patient is in storytelling and the second person is in painting. However, both are unique in the sense that the story contained grandiose self-information with creative characters but not really carrying the inspiring aspects of an exciting storyteller and the paintings are also reproductions of the real images which attracted him always as a child who strongly believed in the superhuman existence of the divine.

CONCLUSION
New skills are sometimes seen during various illnesses causing progressive cognitive dysfunction of various causes. This serves as a pharmaco-sparing agent in behavior management and therefore serves as a rehabilitatory tool. The emergence of these skills could be due to disinhibition of some of the innate skills of the patient during degeneration or establishment of new data linking circuits during attempted repair.

Financial support and sponsorship
Nil.
There are no conflicts of interest.