Case report. A strange case of a lousy mind in a “perfect” brain. Early pseudodementia, frontotemporal dementia, or nothing at all?

Luis Maia, PhD. Cédula Profissional da Ordem dos Psicólogos, n.º 102. Auxiliar Professor - Beira Interior University. Clinical Neuropsychologist, PhD (USAL - Spain). Neuroscientist, MsC (Medicine School of Lisbon - Portugal). Medico Legal Perit (Medicine Institute Abel Salazar - Oporto, Portugal). Graduation in Clinical Neuropsychology (USAL - Spain). Graduation in Investigative Proficiency on Psychobiology (USAL - Spain) Clinical Psychologist (Minho University - Portugal)

In this article we present a strange clinical case of a 53 years old man with severe neuropsychiatric acute syndrome. The patient is a well succeeded 53 years old business man, married, father of two adult male sons. In 12 months, a tragic neuropsychiatric picture was developed, and changed his life dramatically.

Seven years ago, but with increased incidence in the last year, the patient presented progressive deficits in operating executive functions and memory. Such deficits extended beyond events whose mnesic information could be considered as long-term, like omissions of concrete situations and experiences in the last days, weeks and even months (autobiographical memory).

This aspect provoked a worsening in his professional responsibilities (the patient is the main founder of a large company of house isolation and overheating). This is reflected in unthinkable mistakes for his normal performance, such as making incorrect measurements, forget to comply with labor commitments (e.g. forget large orders of great financial profit), shouting out loud with employees, misplacing his own responsibilities about errors committed (e.g. usually responsibility for certain loss that have occurred are attributable to itself, but in his opinion, his employees are the ones to be blamed, etc).

Overall, the patient becomes currently described by his family as childish (uses slang and inappropriate speech in front of customers and employees – this pattern does not seems to be his normal way to behave), exaggerated when defeated (e.g. forced an employee to use a cutter in a scrap of a vehicle which still had petrol and, despite the employee had called unceasingly, he forced him to use the tool, which led to vehicle went in flames, jeopardizing their integrity).

In such situation, he does not presented signs of fear and do not attempted to protect himself. Instead, took a further exasperated attitude with the employee, blaming him by the accident despite the proximity of flame and imminent risk of explosion. Similarly, responds inappropriately to what he is asked, and states that is only due to an obvious marked deficit at the level of bilateral hearing acuity (diagnosed by a specialist physician).

As a family management company (e.g. wife take care of accounting, the eldest son treats of contacts with customers, etc.) the patient presents an aggressive and humiliation behavior of family members, especially the eldest son (described by his mother as a responsible and worker person), without demonstrating discomfort with the presence of employees or customers (this completely abnormal behavior is recent in his past).

He also presents an irresponsible and inconsistent attitude. For example, having to drive heavy vehicles with chemical reagents, he often has small roadside accidents (up to now without consequences). He justify (with an infantile attitude): "Satan is my friend … he pulls me out of road (sic.)".

The wife says that, at least in three occasions, the patient entered handling vehicles in forbidden way, in traffic circle, causing great danger to him and to other drivers.

Common in each of the above-described situations is that, according to the family, when called attention to the risks, he presents an inconsistent attitude, do not seeming to present any insight of their actions dangers.

He doesn’t seem to present severe depressive symptoms. What frequently occur, is to pass considerable amounts of times lost in her own thoughts without expressing any significant attitude or behavior (“I am able to be half an hour looking for room clock without saying anything to anybody” sic.).

As a relevant aspect, it seems to us that slight changes occurred in his basic hygiene and care pattern in daily life activities. Although a significant change cannot be considered in terms of carelessness and lack of hygiene, he presents a care decrease in shaving and bathing frequency (passed from a daily based activity to 2 or 3 times per week).

Unlike his past history, he became disorganized about cleaning his own mess. Present stressed hyper-phagia and reduced libido, with absence of sexual intercourse at least for four years.

Displays monetary loosening with unnecessary expenses, without appear to commit such acts during a maniac or hypo-maniac episode. For example, on agriculture properties, that he is the owner, have already invested in four tractors vehicles and other medium-large machinery highly expensive (it should be noted that this activity is not used for commercial profit, but solely by hobby and family consumption). He also tends to borrow large machinery and cannot remember who loaned them and, because it is not returned, blame families and employees for such errors.

As a corollary of what was referred above, his wife states, "this last year was of total decadence” (sic.).

He ultimately recurred to our services were, after extensive neuropsychological evaluation and anamnesis assessment, his case seemed better explained by progressive frontal cortical degeneration. Particularly, the patient has been affected in recent years (seven years) by symptomatology indicative of frontal temporal syndrome, with a clear worsening in the last 12 months.

Neuropsychological assessment

The subject was profoundly evaluated with several neuropsychological batteries, in three different moments during the years 2009 and 2010.

Despite the results found, since the beginning, the patient presented strong alterations in learning and memory. Anterograde amnesia, neuropsychiatric signals, as alterations of personality, slang language, obscene behavior (extra marital relationships), lack of social conscience, strong irresponsibility (for example: driving a car in a forbidden way, with low sense of responsibility, hiperphagia, hypersomnolece, etc).

The first clinical hypotheses about this developing syndrome was Mild Cognitive Impairment, of Frontal–Temporal type, undoubtedly of degenerative evolutive frame.

Particularly, the major deficits in Neuropsychological areas and Neuropsychiatric symptoms that the patient shows are, as follows: a) The patient was first evaluated by a psychiatrist who allegedly, according to his wife and himself, was diagnosed with dementia of Alzheimer's type, on a single inquiry, without any diagnostic auxiliary means; b) Once he got worse she looked for a Neurologist that diagnosed a severe depression and, allegedly said: “you have a problem of soul and I don’t understand you"; c) The family finally came to our services.

Summary of Neuropsychiatric Data

MSSE: 27 points, in 30 possible; nine grade of schoolarity (primary school).

In Complex Rey Figure the subject shows a slightly deficit in re-evocation of visual-constructive material (re-evoking only some particularities of the previously submitted image). However, we can see that the small errors committed are directly related to monitoring deficits and attention focus in the organization, integration, and building an image as a whole.

In bimanual coordination capacity (conflictive go-no-go tasks – alternate hand positions simultaneously) the patient shows significant bad performance characterized by uncoordinated movements with incidence in upper left distal member. When asked to perform tasks by increasing speed, difficulties also seem to be incremented.

Finally, in verbal fluency the patient presents unsatisfactory performances in pronunciation and slowing thought (Bradiphrenia).

Other clinical data

Tumor Markers

Although there is history of prostatic inflammation, tumoral markers do not show any relevant factor to the comprehension of the entire case.