Neurological Disorders: Alien Hand Syndrome

Synonyms:
 Anarchic hand; Diagnostic dyspraxia;  Intermanual conflict; Magnetic apraxia; Unilateral apraxia; La main etrangere. 

Overview
     Alien hand syndrome  is a rare neurological disorder that causes hand movement without the person being aware of what is happening or having control over the action. The afflicted person may sometimes reach for objects and manipulate them without wanting to do so, even to the point of having to use the healthy hand to restrain the alien hand. A new study identified the areas of the brain involved in both voluntary and involuntary movement and found that neural activity was restricted to the primary motor cortex during the unconscious motor activity seen with AHS. The study will be published online in the official journal of the American Neurological Association.

      The alien hand syndrome, as originally defined, was used to describe cases involving anterior corpus callosal lesions producing involuntary movement and a concomitant inability to distinguish the affected hand from an examiner's hand when these were placed in the patient's unaffected hand. In recent years, acceptable usage of the term has broadened considerably, and has been defined as involuntary movement occurring in the context of feelings of estrangement from or personification of the affected limb or its movements. Three varieties of alien hand syndrome have been reported, involving lesions of the corpus callosum alone, the corpus callosum plus dominant medial frontal cortex, and posterior cortical/subcortical areas. A patient with posterior alien hand syndrome of vascular aetiology is reported and the findings are discussed in the light of a conceptualisation of posterior alien hand syndrome as a disorder which may be less associated with specific focal neuropathology than are its callosal and callosal-frontal counterparts.


Clinical case:
     A 71-year-old right-handed woman developed acute left hemiparesis and visual hallucinations. She also complained that her left hand uncontrollably scratched her and pulled at her hair. Past medical history was notable for chronic hypertension and triple coronary artery bypass graft 6 years earlier. Two weeks before the illness, she had felt diffusely weak; another hospital had attributed this weakness to metoprolol toxicity. When her complaints did not subside after 2 days off medication, her husband brought her to the emergency room. Evaluation disclosed full orientation, left lower facial weakness, right gaze bias, left hemibody hypesthesia, and extensor posturing of the left limbs, with the left hand constantly clenched. Cranial CT scan indicated acute right parietal cortical infarct and extensive bilateral subcortical white matter ischemic changes.
Read further... 




Sources:
http://jnnp.bmj.com/content/68/1/83.full
http://www.jsmf.org/meetings/2008/may/Mark%20VW%202008%20Alien%20hand%20syndrome.pdf
http://www.ncbi.nlm.nih.gov/pubmed/20220444
http://emedicine.medscape.com/article/1136037-overview

Selengkapnya →

Restless Legs Syndrome

Background

       Restless legs syndrome (RLS) is a neurologic movement disorder of the limbs that is often associated with a sleep complaint ^.Patients with RLS have a characteristic difficulty in trying to depict their symptoms. They may report sensations, such as an almost irresistible urge to move the legs, that are not painful but are distinctly bothersome. RLS can lead to significant physical and emotional disability.

   The sensations of RLS usually are worse during inactivity and often interfere with sleep, leading to chronic sleep deprivation and stress.Once correctly diagnosed, RLS can usually be treated effectively and, in some secondary cases, it can even be cured.
The term RLS was used initially in the mid-1940s by Swedish neurologist Karl A. Ekbom. However, descriptions of the disorder date back to the 17th century.
RLS is often unrecognized or misdiagnosed. Many patients are not diagnosed until 10-20 years after symptom onset. RLS may begin at any age, even as early as infancy, but most patients who are affected severely are middle-aged or older.

What are common signs and symptoms of restless legs?

People with RLS feel uncomfortable sensations in their legs, especially when sitting or lying down, accompanied by an irresistible urge to move the affected limb.  These sensations less commonly affect the arms, trunk, or head.  Although the sensations can occur on just one side of the body, they most often affect both sides.
Because moving the legs (or other affected parts of the body) relieves the discomfort, people with RLS often keep their legs in motion to minimize or prevent the sensations. They may pace the floor, constantly move their legs while sitting, and toss and turn in bed.

A classic feature of RLS is that the symptoms are worse at night with a distinct symptom-free period in the early morning, allowing for more refreshing sleep at that time.  Other triggering situations are periods of inactivity such as long car trips, sitting in a movie theater, long-distance flights, immobilization in a cast, or relaxation exercises.  Many individuals also note a worsening of symptoms if their sleep is further reduced by events or activity.
RLS symptoms may vary from day to day and in severity and frequency from person to person.  Individuals with mild RLS may have some disruption of sleep onset and minor interference in daytime activities.  In moderately severe cases, symptoms occur only once or twice a week but result in significant delay of sleep onset, with some disruption of daytime function.  In severe cases of RLS, the symptoms occur more than twice a week and result in burdensome interruption of sleep and impairment of daytime function.
Individuals with RLS can sometimes experience remissions—spontaneous improvement over a period of weeks or months before symptoms reappear—usually during the early stages of the disorder.  In general, however, symptoms become more severe over time.
People who have both RLS and an associated medical condition tend to develop more severe symptoms rapidly.  In contrast, those who have RLS that is not related to any other condition and experience onset at an early age show a very slow progression of the disorder; many years may pass before symptoms occur regularly.

What causes restless legs syndrome?

In most cases, the cause of RLS is unknown. However, it may have a genetic component; RLS is often found in families where the onset of symptoms is before age 40. Specific gene variants have been associated with RLS. Evidence indicates that low levels of iron in the brain also may be responsible for RLS.
Considerable evidence suggests that RLS is related to a dysfunction in the brain’s basal ganglia circuits that use the neurotransmitter dopamine, which is needed to produce smooth, purposeful muscle activity and movement. Disruption of these pathways frequently results in involuntary movements. Individuals with Parkinson’s disease, another disorder of the basal ganglia’s dopamine pathways, often have RLS as well.
RLS also appears to be related to the following factors or conditions, although researchers do not yet know if these factors actually cause RLS:

• Chronic diseases such as kidney failure, diabetes, and peripheral neuropathy. Treating the underlying condition often provides relief from RLS symptoms.
• Certain medications that may aggravate symptoms. These medications include antinausea drugs (prochlorperazine or metoclopramide), antipsychotic drugs (haloperidol or phenothiazine derivatives), antidepressants that increase serotonin, and some cold and allergy medications-that contain sedating antihistamines.
• Pregnancy, especially in the last trimester. In most cases, symptoms usually disappear within 4 weeks after delivery.

Alcohol and sleep deprivation also may aggravate or trigger symptoms in some individuals. Reducing or completely eliminating these factors may relieve symptoms, but it is unclear if this can prevent RLS symptoms from occurring at all.

How is restless legs syndrome diagnosed?

There is no specific test for RLS.  The four basic criteria for diagnosing the disorder are:

• Symptoms that are worse at night and are absent or negligible in the morning;
• A strong and often overwhelming need or urge to move the affected limb(s), often associated with paresthesias or dysesthesias;
• Sensory symptoms that are triggered by rest, relaxation, or sleep; and
• Sensory symptoms that are relieved with movement and the relief persists as long as the movement continues.
  

    Physicians should focus largely on the individual’s descriptions of symptoms, their triggers and relieving factors, as well as the presence or absence of symptoms throughout the day.   A neurological and physical exam, plus information from the individual’s medical and family history and list of current medications, may be helpful.  Individuals may be asked about frequency, duration, and intensity of symptoms as well as their tendency toward daytime sleep patterns and sleepiness, disturbance of sleep, or daytime function.
Laboratory tests may be performed to rule out other conditions.  Blood tests can identify iron and vitamin deficiencies as well as other medical disorders associated with RLS.  In some cases, sleep studies such as polysomnography (a test that records the individual’s brain waves, heartbeat, breathing, and leg movements during an entire night) may identify the presence of other causes of sleep disruption (e.g., sleep apnea), which may impact management of the disorder.
Diagnosing RLS in children may be especially difficult, since it may be hard for a child to describe where it hurts, when and how often the symptoms occur, and how long symptoms last.  Pediatric RLS can sometimes be misdiagnosed as "growing pains" or attention deficit disorder.

How is restless legs syndrome treated?

       RLS can be treated, with care directed toward relieving symptoms. Moving the affected limb(s) may provide temporary relief. Sometimes RLS symptoms can be controlled by finding and treating an associated medical condition, such as peripheral neuropathy or diabetes.
Certain lifestyle changes and activities that may reduce symptoms in persons with mild to moderate symptoms include decreased use of caffeine, alcohol, and tobacco; supplements to correct deficiencies in iron, folate, and magnesium; changing or maintaining a regular sleep pattern; a program of moderate exercise; and massaging the legs, taking a hot bath, or using a heating pad or ice pack. A trial of iron supplements is recommended only for individuals with low iron levels. Although many people find some relief with such measures, rarely do these efforts completely eliminate symptoms.
Medications are usually helpful but no single medication effectively manages RLS for all individuals. Trials of different drugs may be necessary. In addition, medications taken regularly may lose their effect over time, making it necessary to change medications periodically.
Common drugs prescribed to treat RLS include:
Dopaminergic agents (drugs that increase dopamine), largely used to treat Parkinson's disease, have been shown to reduce symptoms of RLS and PLMS when they are taken at bedtime and are considered the initial treatment of choice. The U.S. Food and Drug Administration has approved pramipexole and ropinirole to treat moderate to severe RLS. Both drugs are generally well tolerated but can cause nausea, dizziness, or other side effects. Good short-term results of treatment with levodopa plus carbidopa have been reported.
Although dopamine-related medications are effective in managing RLS, long-term use can lead to worsening of the symptoms in many individuals. This apparent progressive worsening is referred to as “augmentation.” With chronic use, a person may begin to experience symptoms earlier in the evening than in the afternoon until finally the symptoms are present around the clock. The initial evening or bedtime dose becomes less effective, the symptoms at night become more intense, and symptoms begin to affect the arms or trunk. Fortunately, this apparent progression is reversible by removing the person from all dopamine-related medications. Another important adverse effect of dopamine medications that occurs in some people is the development of impulsive or obsessive behaviors such as obsessive gambling or shopping. Should they occur, these behaviors can be reversed by stopping the medication.
Other medications may be prescribed “off-label” (not specifically designed to treat RLS) to relieve some of the symptoms of the disorder.

• Benzodiazepines can help individuals who have mild or intermittent symptoms obtain a more restful sleep. However, even if taken only at bedtime they can sometimes cause daytime sleepiness. Benzodiazepines such as clonazepam and diazepam are generally prescribed to treat anxiety, muscle spasms, and insomnia. Because these drugs also may induce or aggravate sleep apnea in some cases, they should not be used in people with this condition.

• Opioids such as codeine, propoxyphene, or oxycodone may be prescribed at night to diminish pain and help to relax individuals with more severe symptoms. Side effects include dizziness, nausea, exacerbation of sleep apnea, and the risk of addiction.

• Anticonvulsants such as gabapentin and pregabalin can decrease the sensory disturbances such as creeping and crawling sensations and nerve pain. Dizziness, fatigue, and sleepiness are among the possible side effects.

What is the prognosis of people with restless legs?

RLS is generally a lifelong condition for which there is no cure. Nevertheless, current therapies can control the disorder, minimizing symptoms and increasing periods of restful sleep. Symptoms may gradually worsen with age, although the decline may be somewhat faster for individuals who also suffer from an associated medical condition. In addition, some individuals have remissions—periods in which symptoms decrease or disappear for days, weeks, or months—although symptoms usually eventually reappear. A diagnosis of RLS does not indicate the onset of another neurological disease, such as Parkinson’s disease.

Sources:
The Restless Legs Syndrome Foundation
What Are the Signs and Symptoms of Restless Legs Syndrome?
Night Walker: Restless Legs Syndrome- Do creepy-crawly feelings in your legs have you walking the night away? You may have restless legs syndrome. http://www.webmd.com/sleep-disorders/rls-08/rls-sleep-problems
Restless Legs Syndrome http://emedicine.medscape.com/article/1188327-overview
Video: Discovery Health CME:  Restless Legs Syndrome http://health.discovery.com/videos/discovery-health-cme-restless-legs-syndrome.html

Selengkapnya →

#Medical Case: 7-year old with Limbs Growing Out of his Chest

           A 7-year-old boy Deepak Kumar Paswaan, who lives in Buxar, Bihar, India, was born with extra arms, legs and buttocks of a parasitic twin protruding from his chest, which looks similar to the Indian man’s extra body.

• This case is similar to how conjoined twins are formed, except there is a malformation of one of those twins.As a result, the twin never fully develops and has caused the boy to live with extra arms and legs.The worst part is, the legs are growing at the same rate as the boy, adding to the weight the boy must carry.
  

• As “usual,” he was worshipped as an incarnation of Lakshmi, the multi-limbed Hindu goddess of wealth and prosperity but there are also people who cast stones to him to driveout the “demon” in his body. 

When Deepak was born with the parasitic twin growing out of his abdomen the doctors in the village warned he wouldn’t have lived for more than a few days. However little Deepak fought through all the odds and survived as a healthy child though he was much behind his peers and led a socially restricted life. The contrasting ways in which he led is life is best exemplified by the fact that Deepak was treated as a religious icon and was worshipped by a set of villagers as God, while a few took him as a devil born to the village that would cast bad luck and attacked him with stones which left the little boy petrified and restricted his movement out of his home, mingling with his peers or even going to school with his brothers.

• “It is heart wrenching for a parent to see his child suffer like this, though many people come from distant villages to worship him, offer money, sweets, flowers etc but I never had any intention of earning money through my child. I wanted to see my son like any other normal child being accepted by the society and so I decided to go for the surgery and get rid of the abnormal part of his body” says Viresh Paswan, Deepak’s father a construction worker at Bhelhari, a village 125 kilometers away from Patna.

Dr. Ramcharan Thiagarajan the lead surgeon of Deepak’s case felt that  the boy and the parasitic twin shared the chest wall and the abdomen. “I was concerned that there may be sharing of the thoracic organs such as heart or lungs in addition to the abdominal organs“. We were confident about providing care to this boy as we have an outstanding cardiac and liver unit at Fortis Hospitals Bangalore. The MRI scans which were sent to us showed that the parasitic twin was protruding from his lower chest and abdomen and was fused to Deepak with sharing of liver, intestine and possibly other structures. On clinical examination, the parasite was attached to the lower part of the chest and the most of the upper abdomen and it looked as if he was carrying a baby. It had two legs, a pelvis, abdomen and two poorly formed upper arms.

• Once Deepak was brought into the hospital a CT scan was performed and it revealed that the sharing of the intestine, had vascular anomalies, a huge hernia and possible involvement of the liver. After an intensive discussion amongst the team of specialists which comprised of the liver and digestive surgical team, radiologist, anesthesiologist, neonatologist and child psychologist they concluded that the parasite can be safely removed without Deepak compromising his life. However there is always a possibility of complication after surgery either from excessive bleeding or from sharing of organs. The involvement of a neonatologist and a child psychologist was important because the physical disability affected Deepak’s social behavior; he seemed to have suffered from neurological imbalance and was mal nourished to a large extent as the parasite was taking most of the nutrition from his body.

• According to Dr. Murali Chakravarthy, Chief Anesthesiologist “Anesthetizing parasitic twins is always a challenge. Deepak was no exception. We were not worried about gaining control of blood pressure fluctuations; because continuous monitoring of the hemodynamic parameters began from the word go. Grossly hypertrophied artery from the chest was supplying the parasitic twin. We had concerns about the management of the subsequent clamp of this while separating the twin. Thanks to the preparations, we could handle them without causing any danger to Deepak. Pain relief after a major surgery requiring a long surgical incision was yet another challenge, which was adequately taken care by insertion of the thoracic epidural catheter. Overall the job was well planned and executed. A beaming Deepak is a testimony to that.

“We were well prepared and started the surgery with an incision on the parasite to save skin and muscle to use for Deepak at the time of closure. We went into the parasites body and found that there were loops of intestine, piece of urinary bladder densely adhered to Deepak’s liver which was enlarged and also creating a huge hernia coming out through Deepak’s abdominal wall little bit below the fusion. The parasitic content was meticulous dissected off from liver and excised without compromising Deepak’s internal organs. The skin, muscle and the fascia (fused muscle providing strength) of the parasite was used to reconstruct Deepak’s abdominal wall” explained Dr. Ramcharan Thiagarajan. “What was originally seen as a sharing of the liver was found to an enlarged liver Deepak’s recovery has been quite fast, and he was moved out of the PICU on the first post op day and he started walking on his own on the 2nd post op day. He will be able to lead a normal life with pride and dignity like any other child without any physical difficulty in further”.

• An emotionally charged Indu, Deepak’s mother says “I was initially not sure with the decision of Deepak’s surgery and was upset with my husband as I thought it would put my son’s life at risk. Though I wanted Deepak to have a normal life but as a mother my heart wept constantly with the fear of losing him in the process as I was scared by the villagers saying no surgery in this world can correct Deepak’s condition. But now when I see him smiling and playing with our 3 year old daughter just after two days of the surgery, my faith has strengthened and I believe that medical science has progressed so much that anything is possible if there is enough conviction”.

• “I was confident that my child will fight a way out of this however an unknown fear was always there. I am eagerly waiting to go back to our village and show to all those people who considered my child as a devil, that my son is one of the luckiest and the bravest child in our village. I want to give him a good life and education so that he doesn’t remain a prey to society’s superstitious thoughts. The hospital and doctors have not only given my child a new life, they have restored his dignity” said Viresh.

Dr. Ramcharan added “Deepak has recovered very well and our entire team of doctors feels highly gratified that we have helped this child get back to a normal life just like any other boy of his age. It is sad that he could have been fixed long time back and not go through physical and emotional trauma all these years. He is also lucky that this one surgery will completely fix him and he would not require any more surgery contrary to other such where these children go through multiple remedial procedures.”

• “At Fortis Hospitals our commitment in contributing towards clinical procedures which are truly life changing has always reflected in all our medical marvels. To us this case reflects the contribution that modern medicine can make in healing lives. Through our clinical expertise and our passion to be relevant to society we are glad to have given this little child from Bihar a new lease of life and free him of the social stigma. Deepak is a real hero for us who has fought the social and medical battle, he has won over the hearts of all the employees at hospital with his spark and brilliance. We wish him a good life and a great future ahead” said Mr. Vishal Bali, Chief Executive Officer, Fortis Hospitals.

Source:
http://www.dailymail.co.uk
http://www.telegraph.co.uk

Selengkapnya →