thoughts and ideas about XXY

some thoughts ….

– a survey of XXY about being left-handed or right-handed

( I recently found out with help of an autism specialist that I’m actually left-handed explaining a lot of difficulties with direction of rotation since childhood)

– a survey of XXY about enhanced sensory perception (yes/no, and which senses are affected most often: hearing, visuals, taste, tactile, smell)

(there are communalities between small-scale polls in autism forums and the british XXY forum, both resulting in enhanced sensory issues with hearing and visuals)

if ADD/ADHD are rather symptoms than syndroms (about 50 % of people with autism also have ADHD, about 60 % of people with XXY have also ADHD), and mainly the result of different sensory perception…. it would mean that XXY is one of many possible conditions to obtain a different perception in general.

The idea of, e.g. self-stimulating behavior or special interests as a strategy to manage too much incoming information, social stress, sensory overload, mental overload, etc… is relatively new (but included in the latest DMS-V for diagnosing autism spectrum conditions). If both behavior (stimming and special interests) is less defined, people have to look for other „bad“ copying strategies, like frequent meltdowns, attention deficits, hyperactivity, etc…

(in the few studies about autistic symptomes in XXY, XXY reveal less special interests, repetition behavior as non-XXY autistic people, which could mean that XXY are less able to self-regulate themselves)

Attending symptoms like bad time management, organisation, planning, etc… are shared by all conditions (XXY, ADHD, autism) as a result of executive dysfunctions (something’s working wrong in the frontal lobes).

For a long time, in Germany even now, nobody thought of communalities between these conditions because all symptoms were related to the lack of testosterone. This is another fact I’m quite curious about because hormone imbalances are also reported in autism (and gender dysphoria seems to be more frequent in autism than in the general population), and some autistic women reported too high testosterone values.

I guess the discussion about these relations often stutters because of the usage of the term „autism“: Autism is highly negatively connotated. Nobody wants to be referred to. Parents are often shocked even hearing about this possibility. I approach this topic rather from bottom-up thinking. I gather as much information as possible trying to puzzle it together. I follow the principle of neurodiversity, i.e., the different perception doesn’t express a disorder but a difference. Maybe, this approach gets lost in this strong desire of having categories and stamps, coming with DSM-V and diagnosis (good read: Frances, Allen (2013). Saving Normal: An Insider’s Revolt Against Out-of-Control Psychiatric Diagnosis, DSM-5).

My intention is not to put XXY in additional categories but to destigmatize the existing categories.

Linksammlung (Querbeet)

Autismus

Autism

Satire

Geschlechteridentität (Gender identity)

47,XXY and Testosterone treatment

Executive dysfunctions

Psychologie allgemein

ADHS (ADHD)

Klinefelter Syndrome

Good terms but bad outcome?

There is an ongoing discussion about to obtain support without having a disorder. There are different terms for people with autism:

The official term is now autism spectrum disorder. People with autism tend to prefer the term autism spectrum condition sounding more value-free, without main focus on deficits. It’s also pointing towards the underlying genetic cause of autism. Everyone with autism develops different. There is no determined way for people with autism.

In a very similar way this is true for people with 47,XXY. The pure existence of an additional X doesn’t say much about his future. There are some numbers about increased probabilities of comorbid diseases or neurologic conditions but even if XXY is diagnosed before birth, nobody will know how the child will develop later. Therefore I prefer the term XXY condition for people with XXY, too.

Klinefelter syndrom is another issue, not interchangeable with XXY. Klinefelter syndrome might be called a disorder or disease because of the consequences of hypogonadism. The majority of men with Klinefelter syndrom (i.e., identifying as men) will probably suffer from enhanced breast development, small genitals as well as infertility. Some will also suffer from frequent diseases like osteoporosis or diabetes. However, it is not accurate to say all people with XXY suffer, like a mother said „my son isn’t only Klinefelter. He’s much more than it.“

So if we talk about XXY itself, we talk about XXY or XXY condition or people with XXY (condition). No offense so far.

If we talk about Klinefelter patients or people with Klinefelter syndrom, it’s used in a medical environment, talking about testosterone replacement therapy, physical traits and co-morbidities.

The main difficulty is, however, to obtain support if necessary. To get help from health care or other institutes, it’s necessary to have a diagnosis. A diagnose, however, is given in a purely negative context, i.e., something is wrong compared with the general population, like a disorder. Although we know that autism is just a different way of human being, not necessarily negative, the majority of autism experts as well as the people we usually deal with every day look at autism as a disorder. So it’s kind of a strong perception bias preventing us from using the terms we would like to use to look at us in a more value-free way, without prejudice. For support, we have to play out the card of suffering while to present ourselves in the best light we have to highlight the advantages of autistic thinking. We are forced to use a double-tracked way to succeed in everydaylife, at work, in school, etc.

Simon Baron-Cohen already wrote a long discussion about the term disability for autism in 2000:

He mentions this dichotomy in a subchapter voting for going on with the term disability to ensue special support.

Special funding does not automatically flow simply because one regards the child as ‚different‘. Given this economic reality, one should not remove the term ‚disability‘ from the description of AS/HFA without ensuring that extra provision would still be available even if the term ‚difference‘ was more appropriate. This is really an issue relating to social policy, health and education economics, and the legal system.

Moreover, his discussion is only addressed to people with Asperger’s syndrome or high functioning autism (which is another arguable term) whose assets are more obvious to the outside than in people with low functioning autism. Here is maybe the tricky part of this consideration. While the deficits of low functioning autism are quite obvious to necessitate special funding and support, the deficits of high functional autism are less obvious, and a significant number is late diagnosed.

So probably – at least at present – we depend on these negatively-loaden terms to explain why we need special support although we look like typical people, we are able to speak and we may have partners, children and even a full-time job. The more people with autism will give up hiding, spread their feelings and wishes with help of social networks and help reducing autism clichés and prejudice, the less we have to rely on labels we don’t like for us.

  • (sidenote in german: In Deutschland gibt es die autistische Störung, die Autismus-Spektrums-Störung und den Oberbegriff Krankheitsbild, manchmal wird auch von autistischer Erkrankung gesprochen. Eine treffende Entsprechung für condition ist mir noch nicht eingefallen, am ehesten noch Veranlagung, im Hinblick auf die (unbekannte) genetische Ursache. In jedem Fall sorgt alleine die Sprache im deutschsprachigen Raum für einen Schwerpunkt auf den Schwächen von Autismus und der Abweichung vom ‚Normalen‘.)

Lack of Impulse Control: When Communication is Overdosed.

Recently, I published a blog text about online dependence leading to a greater dispute about the question whether online communication could be considered as addictive behavior.

I understand the intention of people with autism to refuse their special interests and excessive online communication to be considered as addictive behavior. As I went into behavorial therapy a few years ago, I accidently happened to meet a specialist for prevention of addiction – not online but drugs in general. Addictive behavior is divided into substance (alcohol, drugs) and non-substance (buying, betting, sports, internet). It is caused by loosing impulse control and leads to self-damaging behavior without recognizing it.

As I tapped into the trap of addictive behavior, I was far away from knowing about my neurologic conditions. Executive dysfunctions are core features of autism and 47,XXY. My former therapist didn’t know about my conditions, either, and did consider my online addictive behavior as main reason for difficulties in everydaylife. He tried to shift my focus to (offline) everydaylife but I didn’t succeed because of my verbal communication difficulties.

I tried a cold turkey because I thought my internet behavior is the main reason. Ok, ten years ago, it would have been possible to stay out of the virtual world for a certain period. Today, however, in 2015, situation has changed. Moreover, the majority of my special interests is only possible with aid of internet tools, like weather charts, radar, weather data in general and a lot of other things, like writing blogs, communicating with journalists, etc. A cold turkey would have destroyed me in the long term since I had difficulties to manage my everydaylife.

Therefore I’d like to emphasize it for everyone of you who wants to slam this in my face „I’m not addicted, I need it to survive!!“: A therapist without any knowledge about the cause of „online addictive behavior“ is capable of producing serious damage to a not-recognized autist or XXY forcing him to a cold turkey! Psychologists specialised in addictive behavior should be aware of internet addictive behavior as a symptome, as it is also written in Allen Frances “Saving Normal: An Insider’s Revolt Against Out-of-Control Psychiatric Diagnosis, DSM-5, Big Pharma, and the Medicalization of Ordinary Life

I didn’t make official definitions. We have to deal with offical definitions also for autism which are probably insulting for us, like special interests seen as a symptom of a disability as well as lack of eye contact which is common in other cultures in the world. So please don’t blame me for using the the ICD-10 definition for addictive behavior:

  1. A strong wish or kind of obsession to consume substance causing addiction.
  2. Reduced ability to control start, termination and quantity of the substance.
  3. Physical acute delirium when consumption is finished or reduced.
  4. Proof of tolerance: To obtain the effect of originally lower amounts of the substance, increasingly higher amounts are necessary.
  5. Progressing negligence of other interests and pleasure in favour of the consumption of the addictive drug and/or enhanced expenditure of time to acquire and consume the substance or to recover from the effects.
  6. Persistent drug abuse despite the evidence of clearly damaging consequences (physical, psychological and social)

Source: http://www.suchtmr.de/index.php?id=140

The factors 1-5 seem to be typical for special interests in autism spectrum conditions. Moreover, we feel much more relaxed communicating in a written form instead of a phone call or a face-to-face meeting. I don’t want to list up all advantages. Most of you know them well, otherwise you wouldn’t read my blog.

Despite having great advantages of using internet excessively, I had some serious negative impact I can’t blend out.

In 2003, I missed the famous aurora in Central Europe because I preferred to chat instead of going onto the balcony.
In 2005, I missed a tornado because I preferred to chat in ICQ and write in a weather forum instead of going outdoor.
I cancelled a dinner with a friend he made for me with high effords because I preferred to stay at home and chat. I rapidly lost concentration and focus on reading scientific books and papers well as learning for exams if a computer stood nearby in the same room. I also felt uncomfortable when I couldn’t go online for a longer time than a few hours.

I almost fucked up my studies because I lost the balance of being online and offline (number 5)

Being excessively online (or using a computer in general) had also serious physical consequences: I ate too much fast food because cooking prevented me from staying online. I lacked physical training and was rapidly exhausted in rare occasions like hiking tours. As a result of sitting for hours and days, I tended to have frequent gastrointestinal troubles and constipation. Physical and sleep hygiene has been suffering, too. I couldn’t manage my everydaylife anymore (number 6)

I was asked whether the inability to manage everydaylife is the cause or effect of being online.

In the case of people with social communication difficulties, it might be both of it. On the one hand, social communication and chances to manage everydaylife with internet tools, is a blessing for us. We need it to express our feelings and wishes, opinion and existence. We stay in persistent contact with people we like and people who help us. We will likely have a much harder time without it, especially if the social environment isn’t holding but detrimental. All of us benefitting from this opportunity shouldn’t be blamed as addictive or even pathologic.

I guess… when it comes to therapy and addictive behavior is mentioned either by your parents, friends or therapist, addictive behavior may arise as a primary diagnosis. The reason to write this blog text is, to look behind the obvious symptomes and to look for the true reasons. As ADD, ADHD, autism, diabetes, osteoporosis, etc… may all result from having 47,XXY or related genetic conditions, some primary diagnoses turn out to be wrong (the genetic condition is the primary diagnosis, and autism the result of it).

The term for your behavior may be still addictive behavior, it doesn’t matter for officials if you like it or not.

So why changing behavior which seems to help us?

There are at least some exceptions. There is no black and  white in any behavior.

Whether you’re autistic or not, everyone needs sufficient sleep, good food and physical as well as mental health. It’s important to retain control. Control your feelings when you’re at work (not having emotional outbursts in front of your boss), control spending money if you’re strapped, control to handle your daily work.

I experienced to loose control of nearly any important piece of my life.

To balance online and offline life (our body lives offline and we will die offline, children are born offline), I prefer to do hiking tours when I’m disconnected from the internet. In the nature I can relax, get fresh air, free thinking and develope ideas. I can forget things at least for a short time stressing me. Keeping my sleep hygiene under control means I need to go in bed early enough if I have an appointment or work to do the next day.

Depending on the amount of support in everydaylife, some of us need a job to survive. It’s nice to have special interests like video games or writing hundreds of blog entries. Are they sufficient to earn money with it? Some people with autism are working as game developers, others are writing books or drawing cartoons. Even someone who has a spleen to identify train types may work someday at a railway company. I was fascinated by weather and studied meteorology.

In my opinion, it’s even possible as a person with autism or genetically determined difficulties with impulse control, not to use autism as an excuse to loose control but to look for possibilities to stay fit for everydaylife. Unfortunately, we don’t live in a world where everybody is taking care of your difficulties. Unfortunately, there are still situations where we need these offline skills like having a phone call with officials, going shopping, having a job interview, etc.

I certainly know it’s not easy to learn it, especially when your neurologic package is enhanced by anxiety, traumatic experience and depression.

If I look back, I know it’s possible to increase life quality and still remaining in contact with all online contacts I won in the recent couple of years. Life quality for me includes physical health (enough sport, balanced food, enough sleep), the feeling of well-being when I’m hiking alone in the mountains, as well as managing the to-do-list of necessary and rarely loved things in everydaylife. Sometimes, I fail doing so but I don’t blame myself for it. I know I’m more rapidly exhausted as a neurodiverse person. It’s ok to fail.

*

A minor remark on the opinion online addictive behavior is an artificial diagnose to create a lucrative profession for therapists

I had rather negative experience coming with a suspicion of a diagnosis. „You can’t have it.“ – „It’s something different. You pretend to have it“ – „It doesn’t exist, it’s just fiction to feed the pharma industry.“

A lot of people with autism and attention-deficit disorder will experience that, too, especially when it comes to seek for a diagnosis and disclosure afterwards. For those of us having doubts about potential addictive behavior with internet, don’t deny to have had these experiences.

Whether the term addictive behavior and standard therapy to reduce it without taking the environment into account, is the right path when you benefit from more internet usage than the average population, is another question to discuss. However, there are much more than us really suffering from it, or having had at least a history of negative impact (like myself). I’m able to admit that and try to focus on the advantages now and in the future.

What is mild autism?

Current autism diagnosis in Europe still refers to ICD-10 /DSM-IV where autism is divided into several categories like infantile autism (Kanner autism), Asperger’s syndrome, high-functional autism and pervasive developmental disorder not otherwise specified (PDD-NOS) which addresses to autism as social communication disorder without significant stereotypical behavior, restricted interests or rigid routines.

In the US, the DSM-V is already introduced merging all subtypes of autism into autism spectrum disorders, and determining severity or degree of support with additional numbers.

Most of the subtypes mentioned above are grown historically. Asperger’s syndrome has been called after the Austrian psychiatrist Hans Asperger while Kanner autism has been called after Leo Kanner. High-Functional Autism (HFA) is said to be classic autism from early childhood but with higher intellectual abilities (Tony Attwood doubts the differentation in HFA and Asperger’s syndrome is necessary). PDD-NOS covers the atypical autism when core autism symptomes are present but the development is atypical (which is often the case with 47,XXY-people).

In any case, there are a few key features of autism present in all subtypes:

  • enhanced sensitivity to sensory stimuli (sensory processing disorder, high emotional sensitivity, impaired gross and fine motorics) as „intense world syndrome“
  • different way of thinking, oftentimes pattern or visual thinking
  • difficulties to pick up social cues, prosody, facial expressions, reading between the lines

In some journals, self-support guides and parental reports, Asperger’s syndrome and PDD-NOS are considered as mild autism, attempting to euphemize the consequences of this diagnosis.

The perspective of mild autism may arise from the absence of

  • intellectual disability (i.e., IQ < 70)
  • rigid routines and rituals, stereotypes and repetitive behavior (making everydaylife and employment difficult to organize)
  • inability to speak
  • necessity to have full-time health care or special education/schools.

However, if you ask people with Asperger’s or PDD-NOS themselves, they will probably answer very different how severe they are affected. A study of Asperger’s and HFA asking them about their life quality has shown that the entire lifetime was characterized by ill-defined social development. Social difficulties are highly dominating throughout childhood and youth and will slightly decrease during adulthood. Growing up, the motivation to encounter social contacts increases but friendships are still relatively seldom reaching adulthood. The lack of partnership is specific for HFA/Asperger’s. With respect to therapy and support, comprehension, acceptance and emotional support are considered as most helpful for these persons.

*

Even if someone with Asperger’s syndrome seems to function well in everydaylife, they might suffer from difficulties having and maintaining friendships, from continuous misunderstandings and erraneous picture of their personality. Someone who tends to talk in monologue might be perceived as arrogant and selfish while the opposite is actually the case. What you don’t realize, either, is the degree of sensory processing disorder. They may feel painful when background noise is given and are unable to have a conversation in a loud environment, which is a serious problem going to a busy party, into a loud restaurant or to a concert but also at work when many distractions are present.

You may not notice they have great difficulties with executive functions like planning, organisation, dealing with sudden changes, new things or simple housework stuff. They might have difficulties to accept a phone call or to call a stranger as it is often the case with public authorities. They would enjoy many opportunities but are unable to predict the unexpected to realize it.

As a summary, mild is a relative term because it is a judgement from outside.

  • Some severely autistic people want to be freed from their cage – they’re suffering from high amounts of incoming sensory stimuli.
  • Other severely affected autists may deal better with their situation and perhaps don’t consider themselves as severely affected.

The same holds true for so-called high-functional or Asperger’s or PDD-NOS autists:

  • Some of them might deal so well they are not even officially diagnosed. They have a job, partnership, kids and are satisfied with their life quality.
  • Some are diagnosed soon or later during their lifetime but are still able to manage well their situation.
  • And quite a lot of them are considered to have mild autism but suffer from lacking comprehension and acceptance.

If they tell about their suspicion or even official diagnosis, nonautistic people tend to imagine stereotypic pictures of classic autism and are unable to transfer this prejudice to their close friend or colleague or son/daughter. They may look at their „confession“ as an excuse instead of a reason for their odd behavior. They still believe it would be sufficient to „change the attitude“, „pull one’s socks up“ and „buck up“. To be seen as someone who pushs too hard is very disappointing and hurting for people with „mild“ autism. Life is difficult enough. Comprehension would relieve the lonely pain.

So if you talk or write about mild autism, especially about someone you know or want to describe, just ask them before if they agree with you.

Brain, behavior and life of XXY people: a new study (2015)

The entire title of the paper is „Neuropsychology and socioeconomic aspects of Klinefelter syndrome: new developments“ by Skakkebaek et al. (2015)

I will summarize the most important findings of the paper and will add some points I missed to be discussed.

Behavior, psychiatric conditions and brain differences:

Verbal abilities are most severely affected, IQ scores are slightly lower than average.

The majority suffers from …

  • delayed early language development
  • general learning disabilities in reading and spelling
  • impairments with production of syntax, phonemic processing, word retrieval, comprehension, encoding verbal information and decreased processing speed, verbal fluency
  • executive dysfunctions related to attention, response inhibition flexibility and planning

In contrast, visiospatial function and performance IQ seem to be unaffected. (1)

There is a charateristic personality profile of XXY people, displaying a higher level of neuroticism (emotional instability) and lower levels of extraversion, openness to experience and conscientiousness.

These data are confirmed by anecdotal descriptions revealing

  • anxiety
  • increased emotional arousal
  • serious emotional difficulties
  • being unassertive
  • quiet
  • passive with withdrawn behaviour
  • having difficulties in approaching new events

Psychiatric conditions associated with XXY

  • Depression (35 % in general population, 70 % in XXY)
  • Anxiety
  • Schizophrenia
  • Autism (prevalence of 1 % in the general population, 11-27 % in XXY)
  • Attention-deficit/hyperactivity syndrome (5 % in general, 63 % in XXY)

XXY is often associated with increased level of psychological distress. Higher levels of emotional instability contribute to increased risk of depression and anxiety.

Brain differences

  • Global brain volume, total brain volume, total gray and white matter volumes were found to be significantly smaller in XXY.
  • Volumes of temporal lobe, hippocampus and amygdala were also smaller.
  • All studies except one didn’t find any correlation between cognitive performance scores and brain volumes.

It is assumed that microchanges of brain structures are more important.

Van Rijn examined the brain activity during social judgements of faces and found that XXY people had decreased activity in brain regions related to face processing (inferior temporal regions) and to the limbic system (amygdala, insula). Two other studies found that decreased language activation and/or decreased language lateralization in the posterior temporal language regions were present.

There is still uncertainty about the exact mechanisms of parental origin of the extra X chromsoome, X-chromosome inactivation and androgen recepter CAG repeat length.

Education, living, mortality and criminality

Several studies suggest that behavioral problems, learning disorders, poor educational outcome and criminal conduct could be seen.

It is also emphasized that many led normal lives and the impact of syndromal effects subsided with advancing age. (2)

XXY men  …

  • have significantly fewer partnerships
  • enter later into such partnerships
  • achieve fewer fatherhoods and for those who had luck they occur later

However, at least 25 % of all Danish Klinefelter Syndrome were registered as fathers, probably mostly due to donor semen donation.

Data also show that …

  • educational level is low leading to a lower income throughout their lifetime and that many retire early (43,5 vs. 60,3 years)
  • mortality is almost doubled, partly influenced by cohabitation and educational status (without them, less prominent)
  • criminality is enhanced for sexual abuse, arson, burglary and ‚other offenses‘ but decreased for traffic crimes (3)

If the social and economic background is taken into account, the risk is generally reduced.

XXY are relatively seldom diagnosed…. There are long delays and frequent-false negatives. Only about 25 % are diagnosed, and the majority has to wait until adulthood.

Several problems follow:

1. all current XXY studies may have selection bias and the present knowledge may not cover the undiagnosed cases.

2. 90 % of XXY remain undiagnosed until after 15 years of age, missing an important window of opportunity for correcting or alleviating the symptoms

3. we should change our current diagnostic strategy and introduce a new one, diagnosing XXY on blood from neonatal heel prick test (Guthrie test).

Early diagnosis would improve

  • cognitive functions, learning, verbal abilities and behavior, if it turns out that early testosterone supplementation is efficient, and that neuropsychological intervention before puberty is effective.(4)
  • the unhealthy body composition, with increased risk of type 2 diabetes and metabolic syndrome seen in adulthood, as well as bone structure.

Future:

Studies are currently missing focusing on proper treatment or intervention to better the phenotype.

neurocognitive deficits, linked to dyslexia and other learning-related problems, may well lead to poor socioal and economic outcome.

A holistic approach is needed.

Conclusion:

The neurocognitive phenotype of Klinefelter syndrome is clearly abnormal (5) and the need for psychological and cognitive treatment in many cases is evident.

*

Remarks on statements:

(1) „visiospatial function“ seems to be unaffected.  Studies by Jay Giedd show that visual and spatial thinking of XXY people are actually a strength of their thinking architecture. So, visiospatial function isn’t only normal but better pronounced. A lot of XXY people have a good visual memory.

(2) The social environment and intervention is very important. In countries and regions with poor density of experienced specialists, therapeutic outcome will be probably less satisfying, and increasing age could strengthen depressive mood and anxiety.

(3) The enhanced risk to commit crimes of sexual abuse [and arson] could probably be related to inappropriate testosterone supplement therapy. Overdosing testosterone might enhance emotional instability and overemphasize masculine behavior of males. It would be interesting to know whether the participants were already taking testosterone supplements and whether on a daily basis (self-medication) or in larger intervals (injections).

(4) It is important to emphasize that testosterone supplement is neither a one-cure-for-everything therapy nor necessarily suited for all children and adolescents. See additional remarks.

(5) The neurocognitive phenotype of Klinefelter syndrome is clearly DIFFERENT.

Additional remarks:

1. Though I know that science in genetics and behavior usually concentrates on deficit thinking in genetic anomalies, it would be helpful for us affected persons to highlight strengths and positive outcome. Anecdotal descriptions reveal enhanced sense of creativity, sensitivity, social justice, honesty, enhanced detail perception, good visual memory/long-term memory, good with animals.

2. In the vast majority of these studies and papers, the term „men or boys with Klinefelter syndrome“ is used, neglecting a minority of XXY people who do not identify as men, for different reasons… Either there are born intersex, or born as transgender preferring to transition into female later as a teenager or adult. Some identify as male but don’t feel well with masculinization through testosterone supplement therapy, either. A few XXY are also reported to have androgen insensitivity syndrome and testosterone therapy will probably not work for them.

One of the most difficult and heavily discussed topics in the XXY community is whether early intervention with testosterone therapy is a benefit for all XXY children, as transgender or persons who don’t want to be masculinized may not be suited to receive additional testosterone or even require estrogen therapy instead.  In these cases, the term Klinefelter’s syndrome referring to hypogonadism (testosterone deficit) doesn not seem to be appropriate.

I hope we – as XXY community and individuals – are able to convince the scientific community to put more focus on gender identity in XXY before recommending one-size-fit-all-cures for young XXY.

3. I missed some lines about sensory processing disorder. There is only one study about that:

Van Rijn et al, Psychophysiological Markers of Vulnerability to Psychopathology in Men with an Extra X Chromosome (XXY), PLoS ONE, 6(5): 2011

confirming sensory gating disorder in XXY (in other words, XXY often have difficulties to filter out background noise/distraction)

The existence of a handout about sensory processing disorder on AXYS as well as several reports about motoric difficulties suggest that sensory processing and integration disorder is likely to be common in XXY.

Anecdotal evidence is furthermore given about enhanced sensitivity to sensory stimuli like noise, light, motions, smell/taste and touch suggesting a crucial commonality with autism spectrum conditions. One should probably think of XXY as possible specific subtype of the large autism landscape.

Experience with disclosure – a mixed blessing

As written by Mamma Bluette, I don’t think it’s necessary to disclose our special physical characteristics…. That’s pure personal stuff and should be left as such.  One of the greatest difficulties for most of us XXY people is: Nobody ever heared of it…. except for some few lines during biology in school or when chromosome aneuploidies were brought up during medical school. Even if you tell them about XXY or Klinefelter, there isn’t any serious source to rely on…

  • it’s difficult explaining something when only few studies are known, and much fewer about XXY adults.
  • the Internet is full of shit about Klinefelter, much outdated and misleading information.
  • the people to whom you’re disclosing are not interested or don’t want to deal with it after your disclosure, because they are overstrained or don’t know how to react on you being so open
  • disclosure might be interpreted as an excuse (instead of a reason)

If I had known earlier, I would have probably chosen other words than in my very first disclosure. What appeared relatively reasonable for me, has been confusing for the people to whom I disclosed. Maybe the first mistake…. I thought most of my problems originated from the lack of testosterone and the hormone replacement therapy would cure it.

I didn’t expect Klinefelter’s syndrome to be one of MANY possible sideeffects of having an extra X chromosome. I didn’t expect to be autistic and that XXY strongly favours a diagnosis within the autistic spectrum (varying from high-functional, Asperger’s to not otherwise specified, PDD-NOS). Though it’s called mild autism, I don’t feel anything mild in it. It’s not really helpful to compare my degree of autism with other autistics who look clearly more autistic from the outside. I also know that my autism is different from idiopathic autism, i.e. when the cause is still unknown. XXY autism tends to be less pronounced with repetitive behavior and stereotypes but also more towards anxiety disorders. XXY generally tend to reveal a more female-like (autistic) phenotype, which sounds reasonable given the additional X chromosome. But that’s only  my personal interpretation of my behavior and anecdotal evidence of other XXY.

What it makes really difficult for me …. I don’t fit in… neither in typical males, typical autistics (according to a smattering of knowledge coming from media reports and cliché-driving movies) nor in typical XXY. So whenever I’m compared with autistic people, I’m different. Whenever I’m compared with XXY people, I’m different. It seems as if I have parts of each condition in me but do not fulfill all criteria… the least to the outside. Given the case, a XXY individum is also autistic, it’s even more difficult to disclose since much less is known about XXY and we are often reduced to our physical appearances and testosterone deficits.

The reason for which I disclosed has been strong psychological strain. I couldn’t think about right or wrong in that period of my life when everything seemed to crash. I disclosed having XXY/Klinefelter and tried to explain my autistic behavior (without calling it so). I failed, very likely misunderstood as looking for an excuse. Though I tried hard to understand what is actually going on with me…. it’s not so simple… I have to rethink the entire past which is a still ongoing process.

One of the most prominent arguments against disclosure: If you disclose, you will be teased or even dismissed.

Yes, these cases occurred. However, many people with XXY/autism experience it even without disclosure. It won’t make any difference. I decided to be open about it…. because there is no other way to remove clichés, and I’ll have difficulties anyway… even knowing the reason doesn’t mean you can switch off your problems.

My goal is that people with Klinefelter’s syndrome are received as people first. People with extra X also allowing for strengths and talents.