Doose syndrome

by Zhang Dan on March 6, 2011

Doose syndrome, which is also named as myoclonic-astatic epilepsy(MAE), is a rare epilepsy syndrome of children.

94% patients onset before age of 5, with a peak of 3-4 year-old. Usually, they are normal children before onset.

Doose syndrome has multiple types of seizures.  Although myclonus and astatic attack are the main characteristics of Doose syndrome, 60% patients start with general tonic-clonic seizures with or without fever. Myoclonic jerks, astonic or myoclonic-astatic seizure could be seen in 100% patients. Besides, absence seizure and nonconvulsive status seizure could also exist.

Now, I wil describe the main characteristic, myoclonic-astatic seizure, for you. This usually started with brief myoclonic jerks with wide range of intensity, from facial muscle twitching to symmetrical arms and shoulders jerking. Astatic attacks immediately followed myoclonus, ranging from head drop to fall down to the ground.

There are special Electroencephalogram(EEG)  findings in Doose patients. Considering the complexity of EEG, I will skip this part to avoid giving you confusion.

Doose syndrome needs to be differentiated from Dravet syndrome, West syndrome, febrile convulsion, Lennox-Gastaut syndrome and so on. But differentiation would be quite difficult in some children. So, if your doctor changed the diagnosis as the diseas went by, it would not be strange at all.

As to antiepileptic drugs(AEDs), Valproic acid is the first choice. But other drugs can also be used according to types of seizures and response to initial treatment. Many researches show that ketogenic diet is even more effective than any kinds of AEDs.

The prognosis is generally good. 80-90% patients would have no or just mild cognitive impairment.

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keep fit

by Zhang Dan on February 25, 2011

It is quite accurate to say keep fit.

In this recent issure of NEJM, a study about 1 million Asians shows when Body-Mass Index(BMI) is between 22.6-27.5, the risk of  death is the lowest.

People too thin may have nutritional problems or may be too weak to survive from some health problems

And for people too fat, there is a greater chance to develop metabolic and locomotor problems.

BMI=body weight(Kg)/height(cm)/height(cm)

http://www.nejm.org/doi/full/10.1056/NEJMoa1010679

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several things about epilepsy

by Zhang Dan on November 22, 2010

Recently, I have beening working with my mentor in epilepsy center. I think it would be useful to pointing out some important things for patients.

1. Seizure happened just one time may not be epilepsy or a big problem. But if seizures happened twice or more, you’d better go to neurologists. If it was really epilepsy, the prognosis would be better if treatments were given earlier.

2. If the diagnosis was confirmed and your doctor start medication for you, please do take medicine regularly as doctors prescribed.  Don’t forget to take medicine. Don’t stop medicine or decrease dose without consultation.

3.  Seizures may relate to tiredness, drinking coffea, lack of sleep, flash light or other situations. But it may not the same for everyone. If you find your seizures strongly or frequently related to something. You should try to avoid that.

4. When people around you have seizures or tics, do not press him or her violently to stop seizures. Just make sure he or she would not hurt by things around during seizures, such as falling down stairs, getting  head struck, or getting tongues bited.

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fat liquefaction of incision

by Zhang Dan on October 14, 2010

Well, I wrote this because one of my patients is suffering fat liquefaction of incision.

First, it has to be clear that fat liquefaction is not infection of incision. Fat liquefaction occurs when the blood supply of fat tissue decreased to some extent during surgery. Cause when a incision was made, the fat tissue would be cut through, some small vessels coming across would be cut off. So after suture, if the fat tissue can not get enough blood supply, fat liquefaction would happen.

1.what  patients can do to avoid fat liquefaction? 

LOSING WEIGHT! The thicker fat tissue is, the more likely fat liquefaction would occur.

2.what surgeons can do to avoid fat liquefaction? 

  •      avoid cutting fat tissue repeatedly.
  •      use bandages to protect incision during surgery.
  •      eliminate cavity during suture of subcutaneous tissue.

3. what can doctors do when fat liquefaction occurs?

 Open the incision, clear all the necrotic tissue, change dressing everyday to ensure dry circumstance of the incision, wait for granulation tissue to recover the incision. This would be a time-consuming course.

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How to quickly identify a stroke?

by Zhang Dan on October 7, 2010

When the blood supply of some part of the brain decreased to some degree, neuron damage would occur and finally cause neurological dysfunction.  This is now how we define stroke. Stroke can be divided into two catogeries, ischemic stroke and hemorrhagic stroke. The former is caused by cerebral arteries’ occlusion, the later is caused by brain bleeding.

When a stroke happens, the crux is to restore the blood supply as soon as possible. Time is brain. If lacking of blood supply persists, more and more neurons would die, which in turn causes worse and worse neurological dysfucntion.

So, here comes the question. How can we distinguish a stroke quickly?

Here are some simple but useful ways.

First, I’d like to introduce The Cincinnati Prehospital Stroke Scale.

According to some retrospective studies, the sensitivity of this scale is 100%, and the specificity of it is 80%.

It contains three signs of neurological dysfunction.

1.Facial droop: Have the person smile or show his or her teeth. If one side doesn’t move as well as the other so it seems to droop, that could be sign of a stroke.

  • Normal: Both sides of face move equally
  • Abnormal: One side of face does not move as well as the other (or at all)

2.Arm drift: Have the person close his or her eyes and hold his or her arms straight out in front for about 10 seconds. If one arm does not move, or one arm winds up drifting down more than the other, that could be a sign of a stroke.

  • Normal: Both arms move equally or not at all
  • Abnormal: One arm does not move, or one arm drifts down compared with the other side

3. Speech: Have the person say, “You can’t teach an old dog new tricks,” or some other simple, familiar saying. If the person slurs the words, gets some words wrong, or is unable to speak, that could be sign of stroke.

  • Normal: Patient uses correct words with no slurring
  • Abnormal: Slurred or inappropriate words or mute

Second, let’s talk about a simpler one. FAST, Face Arm Speech Test.

To be as simple as possible, when a person shows facial palsy, distortion of commissure, have difficulty to move arms or legs or have difficulty to speak. This indicates a stroke.

After considering stroke as a possibility, what you should do next is call 911 for help, or send the patient to Emergency room as soon as possible.

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why Neurology

by Zhang Dan on October 1, 2010

“Why do you choose Neurology as your major?” I remember this clearly as my mentor asked me 4 years ago.

I tryed to figure out answers quickly in through my twisted stomach.

“Beacause it bridges physical stuff and mental stuff”

Then why it is not psychology?

“Because it is a potential major in the 21st century”

Don’t give me such cliche.

“Because one of my grandpa died of Parkinson disease, while another granda died of brain hemorrhage”

Well, hope you can treat other people’s relatives as your own.

Recently, I have read a book about relationship between doctors and patients in China. It mentioned that LOVE, FAITH and HOPE was so important to give people mentally support. Although the circumstance is the  worst in China, I choose to be a doctor, under all kinds of threats.

So why Neurology?

Because this is the aim I worked for during the past decade. Becase I feel such great passion and joy when I reading and dealing things related to Neurology.  Because this is my love. And the love I can’t explain why I love it.

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