Angelman Syndrome

Angelman syndrome is a rare genetic and neurological disorder characterized by severe developmental delay and learning disabilities; absence or near absence of speech; inability to coordinate voluntary movements (ataxia); tremulousness with jerky movements of the arms and legs and a distinct behavioral pattern characterized by a happy disposition and unprovoked episodes of laughter and smiling. Although those with the syndrome may be unable to speak, many gradually learn to communicate through other means such as gesturing. In addition, children may have enough receptive language ability to understand simple forms of language communication. Additional symptoms may occur including seizures, sleep disorders and feeding difficulties. Some children with Angelman syndrome may have distinctive facial features but most facial features reflect the normal parental traits. Angelman syndrome is caused by deletion or abnormal expression of the UBE3A gene (ubiquitin protein ligase E3A).

National Organization for Rare Disorders (NORD) with assistance of Charles Williams, MD,  Updated Emeritus Professor, Division of Genetics and Metabolism, Department of Pediatrics, University of Florida College of Medicine; member of the Angelman Syndrome Foundation Scientific Advisory Committee.

At this time, therapies for Angelman syndrome are symptomatic and supportive. Several clinical trials on Angelman syndrome are ongoing (see below) but there is no genetic therapy or curative medication available. Advances in neuroscience and in gene therapy techniques however hold great potential for providing meaningful treatment and/or cure of the syndrome.

The general physical health of those with Angelman syndrome is good and usual pediatric care, including customary childhood immunizations, can be provided.


For More Information

Learn more about genetics on our Genetic Disorders page.

Synonyms of Angelman Syndrome

  • AS
  • happy puppet syndrome (obsolete)

Learning to Tell Time with Dyscalculia

[Editor's note: This article was originally published on a Danish-language site about dyscalculia (no longer available as of March 2015). The original version was written by Mette Christoffersen. It was translated and posted here with her kind permission.]

Telling time – honestly, how hard can that be? Pretty hard, if you have dyscalculia. This may be due to visuospatial dyscalculia, numerical fact dyscalculia, procedural dyscalculia, and semantic retrieval dyscalculia – or all four types.

I have a little bit of everything. When it comes to clocks, however, it's the visuospatial dyscalculia that causes trouble. I first learned to tell time in my early teenage years. Most likely because it was at this time that my semantic (linguistic) understanding of numbers was established. Before this, I had trouble distinguishing the numbers from 50 to 100 – orally. That is, I understood that the written symbols for 50, 60, 70, 80, 90, and 100 were in this order, but linguistically, I mixed up the words for these numbers. And when it comes to the concept of "time", you need a linguistic understanding of numbers.

Today, I'm much better at reading clocks – but it's not something that I just do. A clock with no numbers and only hands – I can forget all about understanding that if I want to be in a good mood the rest of the day. To be 26 years old and have trouble with something as simple as a clock is not good for your self-confidence regardless of how intelligent you otherwise know you are. A clock that only shows four of the hours – six, nine, twelve, three – doesn't make the matter any easier.

Digital clocks are not a problem. ChesireKat from Dyscalculia Forum has described the difference between analog clocks and digital clocks rather well.

For me, it's like having a recipe for cookies in the one hand and a finished cookie in the other hand.

And how can that be – when I'm "number blind"? This doesn't quite make sense. A clock with numbers and nothing else ought to be the most difficult thing for me. And it is for some people with dyscalculia, especially when number fact dyscalculia is present; then you struggle with a basic understanding of numbers. And people with procedural dyscalculia can even have problems with the number sequence on a digital clock.

I think it is hard to explain why visuospatial dyscalculia makes it so difficult to read an ordinary clock. I am probably too close to the problem to be able to evaluate this objectively. Basically, the problem occurs when I have to look at the hands on the clock. Metaphorically speaking, my eyes roll around in my head when I look at the clock. I have to stop and concentrate to see that one of the hands is pointing, shall we say, between 5 and 6. Then, I have to find the other hand. It points between 11 and 12. Now I have to analyze the two hands to see which is the little hand and which is the long hand. Then I have to remember which hand represents what – does the little hand represent the hour or is that the big one? That seems like a ridiculous problem, but it's one of the hardest things for me to remember. Now let's say that I have remembered that it's the little hand that represents the hour.

(Something that makes the problem even bigger is when there's apparently no difference in the size of the hands or at least so little, that with my visuospatial problems, I cannot see a difference.)

In the meantime, I've forgotten where the hands stood on the dial, so I have to figure that out again. The little one points between 11 and 12, and the big one points between 5 and 6. So it's 11 something-or-other o’clock. Logically, in my brain, it’s 11.5/6 because the next number I found was somewhere between 5 and 6, but I'm well aware that there's no such thing as a time called 11.5/6.

It was especially this point in the learning process that confused me as a child. It had not stuck in my mind that I should start with the number 12 on the dial and count from there. That made no sense, and I didn't understand it when others tried to explain it to me. Why from 12? And if we’re starting from 12, should I not continue counting from there, that is, 13, 14, 15…? That's actually what I did for a while, when the starting point of 12 was established in my mind. Today, I understand the idea of counting 1, 2, 3,… from 12. But this still haunts me for a moment when I have to figure out the time between, for example, 5 and 6.

Okay, now I need to find out what the hand between 5 and 6 is saying. On a clock like this, I count 5 minutes at a time. Now I need to use the 5-tables, which are not yet fixed in my mind – but it’s OK when I concentrate. My eyes need to follow along as I say the numbers. 5 (eyes on the 1), 10 (eyes on the 2), 15 (eyes on the 3), 20 (eyes on the 4), 25 (eyes on the 5)… And then I get stuck when there is no longer a 5-table to cling to. By the way, during this whole process, I've been fighting with the logic of, for example, looking at the 3 and saying 15. Now I must focus visually on an entirely different place on the clock. I must count tiny lines, and the tiny lines hop. I don't have dyslexia – on the contrary – but I really understand what some people with dyslexia mean when they say the letters hop. Stripes, lines, dots – they hop and hop like fleas. The numbers don't hop though. I must really concentrate and, at the same time, remember the number 25 because that's what I had reached. 25… 26… 27… approximately. Okay, I'll say 27. Now I have to go back and look at the little hand because I have forgotten that number in the meantime. It is between 11 and 12. Today, I know that the number located before the little hand is the number that counts. I struggled with that when I was little. So I know the hour is 11. Therefore, it is 11.27.

When I describe all this in words, it takes up a lot of space. In reality, it takes about 15 seconds to understand an analog clock. 15 seconds doesn't sound like much, but just try to determine how long it takes someone who doesn't have dyscalculia. Less than a split second – it’s probably nothing that you think about. That's how I feel about digital clocks.

When there are only analog clocks nearby, and I am together with other people, I simply ask them what time it is. As mentioned previously, it's not every day that one can be bothered with the defeat of reading a clock incorrectly. Linguistically and "clock-numerically", I understand 11.27 today. I know that 27 is close to 30, and 30 is equal to half an hour. That is why digital clocks are so easy. And I can count from 27 to 30, so I know there are 3 minutes until the break is over.

This is a long blog entry. I tried to describe it briefly, but simple problems are probably never as simple as they sound. In the entire clock dilemma, there's also the problem of a lack of understanding of the concept of time – but that's material for a different blog entry someday.

To those parents who are sitting out there and struggling with the issue of their child not understanding how to tell time, I can tell you that for the majority of people with dyscalculia, it does sink in at some point. That's the wisdom I hear from members on the Dyscalculia Forum. I cannot explain why – in my case, it was probably when the numbers over 50 semantically (linguistically) sank in. In the meantime, if my child had dyscalculia, I would choose to focus on learning how to read digital clocks. They are easier for most people with dyscalculia. Digital clocks are not a temporary solution – they are a solution.

Here are some tips for learning how to tell time, from which I could have benefited:

  1. A diagnosis of dyscalculia that describes in detail which type of dyscalculia the child has, to understand why the problem even exists.
  2. The knowledge of numbers up to 100 must be in place – numerically and linguistically.
  3. Understanding what the words hour, minute, second, quarter, quarter to, quarter past, twenty to, and so on, mean.
  4. It's always a good idea to have an eye checkup – eyesight can play a role when visually arranging the hands in your mind. This can be due to nearsightedness or farsightedness, but also astigmatism.
  5. Physical aids can help a few. In my class, we had to set the time on the clock using rubber bands on nails on a piece of wood. This didn't help me, mostly due to linguistic and motor-skill difficulties, but do try it. Even though I didn't get it, I thought it was fun. Things are often absorbed more easily when you have fun. People with dyscalculia often have anxiety about everything concerning numbers – and anxiety stops all learning.
  6. Check whether the concept of time is sinking in. Some need to learn how to tell time before the concept can sink in, but those who lack the whole sense and perception of time need to learn about the concept of time first.
  7. Time must be set aside for learning about time. The child will learn, and if they find the learning difficult, they must know that is OK.
Photo of Astronomical Clock in Prague by grufnik (from Flickr)
Astronomical Clock in Prague, Czech Republic by Grufnik,
Flickr image (license CC BY-NC-ND 2.0)

The Dyscalculia Forum mentioned in the article is no longer available as of March 2015. However, you can try the “Related Disabilities” sub-forum on the British Being Dyslexic forum site. You can bring up dyscalculia questions there.

The British Being Dyslexic forum is no longer available as of March 2020

The photo by Grufnik of the Astronomical Clock (Astronomical Dial), Prague, Czech Republic comes from is licensed under Creative Commons BY-NC-ND 2.0.