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10.18.2011

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A stroke is a medical emergency. Strokes happen when blood flow to your brain stops. Within minutes, brain cells begin to die. There are two kinds of stroke. The more common kind, called ischemic stroke, is caused by a blood clot that blocks or plugs a blood vessel in the brain. The other kind, called hemorrhagic stroke, is caused by a blood vessel that breaks and bleeds into the brain. "Mini-strokes" ortransient ischemic attacks (TIAs), occur when the blood supply to the brain is briefly interrupted.
Symptoms of stroke are
  • Sudden numbness or weakness of the face, arm or leg (especially on one side of the body)
  • Sudden confusion, trouble speaking or understanding speech
  • Sudden trouble seeing in one or both eyes
  • Sudden trouble walking, dizziness, loss of balance or coordination
  • Sudden severe headache with no known cause
If you have any of these symptoms, you must get to a hospital quickly to begin treatment. Acute stroke therapies try to stop a stroke while it is happening by quickly dissolving the blood clot or by stopping the bleeding. Post-stroke rehabilitation helps individuals overcome disabilities that result from stroke damage. Drug therapy with blood thinners is the most common treatment for stroke.
NIH: National Institute of Neurological Disorders and Stroke
 
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Spinal Muscular Atrophy





What is Wallenberg's Syndrome?

Wallenberg’s syndrome is a neurological condition caused by a stroke in the vertebral or posterior inferior cerebellar artery of the brain stem.  Symptoms include difficulties with swallowing, hoarseness, dizziness, nausea and vomiting, rapid involuntary movements of the eyes (nystagmus), and problems with balance and gait coordination.  Some individuals will experience a lack of pain and temperature sensation on only one side of the face, or a pattern of symptoms on opposite sides of the body – such as paralysis or numbness in the right side of the face, with weak or numb limbs on the left side.  Uncontrollable hiccups may also occur, and some individuals will lose their sense of taste on one side of the tongue, while preserving taste sensations on the other side.  Some people with Wallenberg’s syndrome report that the world seems to be tilted in an unsettling way, which makes it difficult to keep their balance when they walk. 

Is there any treatment?

Treatment for Wallenberg's syndrome is symptomatic. A feeding tube may be necessary if swallowing is very difficult.  Speech/swallowing therapy  may be beneficial. In some cases, medication may be used to reduce or eliminate pain.  Some doctors report that the anti-epileptic drug gabapentin appears to be an effective medication for individuals with chronic pain. 

What is the prognosis?

The outlook for someone with Wallenberg’s syndrome depends upon the size and location of the area of the brain stem damaged by the stroke.  Some individuals may see a decrease in their symptoms within weeks or months.  Others may be left with significant neurological disabilities for years after the initial symptoms appeared. 

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) conducts research related to Wallenberg’s syndrome in its laboratories at the National Institutes of Health (NIH), and also supports additional research through grants to major medical institutions across the country.  Much of this research focuses on finding better ways to prevent, treat, and ultimately cure disorders such as Wallenberg’s syndrome.

NIH Patient Recruitment for Wallenberg's Syndrome Clinical Tria




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    The neuropathy of CMT affects both motor and sensory nerves. (Motor nerves cause muscles to contract and control voluntary muscle activity such as speaking, walking, breathing, and swallowing.)  A typical feature includes weakness of the foot and lower leg muscles, which may result in foot drop and a high-stepped gait with frequent tripping or falls. Foot deformities, such as high arches and hammertoes (a condition in which the middle joint of a toe bends upwards) are also characteristic due to weakness of the small muscles in the feet. In addition, the lower legs may take on an "inverted champagne bottle" appearance due to the loss of muscle bulk. Later in the disease, weakness and muscle atrophy may occur in the hands, resulting in difficulty with carrying out fine motor skills (the coordination of small movements usually in the fingers, hands, wrists, feet, and tongue).
    Onset of symptoms is most often in adolescence or early adulthood, but some individuals develop symptoms in mid-adulthood. The severity of symptoms varies greatly among individuals and even among family members with the disease. Progression of symptoms is gradual. Pain can range from mild to severe, and some people may need to rely on foot or leg braces or other orthopedic devices to maintain mobility. Although in rare cases, individuals may have respiratory muscle weakness, CMT is not considered a fatal disease and people with most forms of CMT have a normal life expectancy.
gingival fibrosis
  • Craniofacial dysarthrosis
  • Craniofacial Dysostosis
  • Craniofacial dysostosis syndrome
  • Craniofacial dysostosis, type 1; CFD1
  • Crouzon craniofacial dysostosis
  • Crouzons Disease
  • Crouzon's Disease

Lateral medullary syndrome
Classification and external resources

Medulla oblongata, shown by a transverse section passing through the middle of the olive. (Lateral medullary syndrome can affect structures in upper left: #9, #10, #11, #12, #13, and #14.)
ICD-10G46.4
DiseasesDB10449
eMedicineemerg/834
MeSHD014854
Lateral medullary syndrome (also called Wallenberg syndrome and posterior inferior cerebellar artery syndrome) is a disease in which the patient has a constellation of neurologic symptoms due to injury to the lateral part of the medulla in the brain, resulting in tissue ischemia and necrosis.

Contents

  [hide

[edit]Signs and symptoms

This syndrome is characterized by sensory deficits affecting the trunk (torso) and extremities on the opposite side of the infarction and sensory deficits affecting the face and cranial nerves on the same side with the infarct. Specifically, there is a loss of pain and temperature sensation on the contralateral (opposite) side of the body and ipsilateral (same) side of the face. This crossed finding is diagnostic for the syndrome.
Clinical symptoms include swallowing difficulty, or dysphagia,[1] slurred speech, ataxia, facial pain, vertigonystagmusHorner syndromediplopia, and possibly palatal myoclonus.
Affected persons have difficulty in swallowing (dysphagia) resulting from involvement of thenucleus ambiguus, as well as slurred speech (dysarthria)and disordered vocal quality (dysphonia) . Damage to the spinal trigeminal nucleus causes absence of pain on the ipsilateral side of the face, as well as an absentcorneal reflex.
The spinothalamic tract is damaged, resulting in loss of pain and temperature sensation to the opposite side of the body. The damage to thecerebellum or the inferior cerebellar peduncle can cause ataxia. Damage to the hypothalamospinal fibers disrupts sympathetic nervous system relay and gives symptoms analogous to Horner syndrome.
Nystagmus and vertigo, which may result in falling, caused from involvement of the region of Deiters' nucleus and other vestibular nuclei. Onset is usually acute with severe vertigo.
Palatal myoclonus may be observed due to disruption of the central tegmental tract.
Clinical B1000 diffusion weighted MRI image showing an acute left sided dorsal lateral medullary infarct

[edit]Features

Features of lateral medullary syndrome
DysfunctionEffects
vestibular nucleivestibular systemvomitingvertigo,nystagmus,
inferior cerebellar peduncleIpsilateral cerebellar signs includingataxia, dysmetria (past pointing), dysdiadokokinesia
central tegmental tractpalatal myoclonus
lateral spinothalamic tractcontralateral deficits in pain and temperature sensation from body (limbs and torso)
spinal trigeminal nucleus & tractipsilateral loss of pain, and temperature sensation from face
nucleus ambiguus - (which affects vagus nerve andglossopharyngeal nerve - localizing lesion (all other deficits are present in lateral pontine syndrome as well)ipsilateral laryngeal, pharyngeal, and palatal hemiparalysis: dysphagia,hoarseness, diminished gag reflex(efferent limb - CN.X)
descending sympathetic fibersipsilateral Horner's syndrome (ptosis, miosis, & anhydrosis)

[edit]Cause

The three major arteries of the cerebellum: the SCA, AICA, and PICA. (Posterior inferior cerebellar artery is PICA.)
Human brainstem blood supply description. PICA is #12.
It is the clinical manifestation resulting from occlusion of the posterior inferior cerebellar artery(PICA) or one of its branches or of the vertebral artery, in which the lateral part of the medulla oblongata infarcts, resulting in a typical pattern. The most commonly affected artery is the vertebral artery, followed by the PICA, superior middle and inferior medullary arteries.

[edit]Treatment

Treatment for lateral medullary syndrome involves focusing on relief of symptoms and active rehabilitation to help those suffering from the stroke syndrome recover their activities of daily living and cope with neurologic loss that can be psychologically devastating. Depressed mood and withdrawal from society can be seen in patients following the initial neurologic insult.
A feeding tube inserted through the mouth or gastrostomy may be necessary if swallowing is impaired. Speech therapy may be beneficial in diet recommendations and helping to understand if there is risk for aspiration pneumonia. In some cases, medication may be used to reduce or eliminate pain. Some studies have reported success in mitigating the chronic neuropathic pain associated with the syndrome with anti-epileptics such as gabapentin. The small caliber of the affected PICA does not typically lend itself to reliable surgical recanalization, although a cerebral angiogram is typically required by a neuroradiologist to make this distinction.[2]
One of the most unique and difficult to treat symptoms that occur due to Wallenberg syndrome are interminable, violent hiccups. The hiccups can be so severe that patients often struggle to eat, sleep and carry on conversations. Depending on the severity of the blockage caused by thestroke, the hiccups can last for weeks. Unfortunately there are very few successful medications available to mediate the inconvenience of constant hiccups.
Long term treatment generally involves the use of blood thinners like warfarin. Patients will often remain on these medications or an aspirin regimen for the rest of their lives in order to minimize the risk of another stroke. Other medications may be necessary in order to suppress high blood pressure and risk factors associated with strokes.
Treatment for this disease can be disconcerting because some individuals will always have residual symptoms due to the severity of the blockage. Two patients may present with the same initial symptoms right after the stroke has occurred, but after several months one patient may fully recover while the other is still severely handicapped. [3]

[edit]Prognosis

The outlook for someone with lateral medullary syndrome depends upon the size and location of the area of the brain stem damaged by the stroke. Some individuals may see a decrease in their symptoms within weeks or months. Others may be left with significant neurological disabilities for years after the initial symptoms appeared.[2]

[edit]History

This syndrome was first described in 1808 by Gaspard Vieusseux,.[4] First descriptions by Wallenberg were in 1895 (clinical) and 1901 (autopsy findings).

[edit]See also

[edit]References

[edit]External links

Fibromuscular dysplasia (FMD) is the abnormal development or growth of cells in the walls of the body’s arteries. As a result of this growth, areas of the arteries can thicken, narrow and even enlarge, making it difficult for blood to flow though them.
angiogram
Angiogram to diagnose FMD
Fibromuscular DysplasiaWhat is fibromuscular dysplasia?
Fibromuscular dysplasia (FMD) is the abnormal development or growth of cells in the walls of the body’s arteries. As a result of this growth, areas of the arteries can thicken, narrow and even enlarge, making it difficult for blood to flow though them.
FMD most often affects the renal arteries, which supply the kidneys with blood. It also occurs in the carotid arteries, which bring blood to the brain. Less commonly, FMD develops in the arteries of abdomen (mesenteric arteries) or the arteries of the arms and legs. In nearly one-third of people with FMD, more than one artery is affected.
Depending on which arteries are affected, FMD can increase the risk of high blood pressure, impaired kidney function, aneurysm, stroke and other complications. FMD affects between one and five percent of Americans, typically women under age 50.
What causes fibromuscular dysplasia?
The cause of FMD is still unknown. However, several factors may play a role in its development. A combination of these factors is likely responsible:
  • Genetics. Research suggests that about 10 percent of cases appear in families. People who have a family member with FMD may develop the condition in different arteries than their relative, experience a more or less severe version of the disease, or may not develop FMD at all
  • Hormones. FMD is three to four times more common in premenopausal women than in men, suggesting that sex hormones may be involved in its development
  • Abnormal arteries. A lack of oxygen to the artery walls may cause them to form abnormally. Arteries may also be located abnormally within the body, predisposing them to FMD
What conditions are associated with fibromuscular dysplasia?
FMD can increase risk of several conditions, including:
  • High blood pressure. When the arteries become narrowed, blood pressure can increase. High blood pressure (hypertension) is the most common complication of FMD
  • Kidney dysfunction or failure. Reduced blood flow to the kidneys can impair kidney function and, in rare cases, lead to kidney failure 
  • Pain or cramping in lower legs (intermittent claudication). FMD that affects the arteries in the legs can cause discomfort or pain when walking and exercising
  • A tear in the artery (dissection). The lining of the artery wall may tear, causing blood to leak into the wall
  • Aneurysm. The pressure of blood flow through a narrowed artery can create a weakened area or bulge in the artery wall called an aneurysm. An aneurysm may rupture, resulting in a life-threatening situation
  • Stroke. A stroke may occur if an aneurysm in one of the carotid arteries ruptures or if one of the carotid arteries dissects, disrupting the flow of blood to the brain
What are the risk factors for fibromuscular dysplasia?
Risk factors for FMD include:
  • Gender. FMD affects more women than men
  • Age. FMD is most common in premenopausal women younger than age 50
  • Family history. FMD appears to have a genetic basis. About 10 percent of people with FMD have a relative with the condition
What are the symptoms of fibromuscular dysplasia?
Many people with FMD do not develop symptoms. When symptoms do occur, they depend on the location of the affected arteries. In the renal arteries, FMD can cause:
  • high blood pressure
  • shrinkage (atrophy) of the kidney, which is often painless
  • impaired kidney function
In the carotid arteries, FMD can cause:
  • dizziness
  • ringing in the ears
  • headache
  • blurred vision or temporary loss of vision
  • neck pain
In the mesenteric arteries, FMD can cause:
  • abdominal pain after eating
  • unintended weight loss
In the arteries of the arms and legs, FMD can cause:
  • numbness
  • weakness
  • discomfort when moving the limb
How is fibromuscular dysplasia diagnosed?
In addition to a complete medical history and physical examination, physicians may use one or more the following tests to diagnose FMD:
  • Computed tomography angiography (CTA). This test uses a combination of X-rays, contrast dye and computer technology to produce cross-sectional images (often called slices) of the body
  • Duplex ultrasonography. This technique uses high-frequency sound waves and a computer to create images of blood vessels, tissues and organs. Duplex ultrasonography is used to measure and assess the flow of blood
  • Magnetic resonance angiography (MRA). This noninvasive procedure that uses a combination of a large magnet, radiofrequencies and a computer to produce detailed images of organs and structures within the body
What is the treatment for fibromuscular dysplasia?
Even though there is no cure for FMD, it can be controlled. A multidisciplinary team of physicians will determine the best treatment based on the patient’s individual case. Treatment for FMD includes:
Medical therapy. The physician may prescribe medications to help control high blood pressure, including ACE inhibitors, beta blockers and calcium channel blockers. People with FMD may also need to take antiplatelet drugs, such as aspirin, to prevent blood clots.
Interventional therapy. Physicians may use percutaneous transluminal angioplasty (PTA) to open narrowed sections of arteries. In this technique, a balloon-tipped catheter (thin, flexible tube) is threaded through the affected artery to expand it. A stent, which is a tiny metal-mesh tube, rarely needs to be inserted to keep the artery open in patients with FMD. PTA is less invasive than open surgery and results in faster recovery times.
Surgery. This intervention re-routes blood flow around the diseased artery and may be used in severe cases or when PTA is not an option.
Genetic counseling. Because FMD appears to run in families, women of childbearing age may receive counseling for the genetic basis of the condition. There is not yet a genetic test for FMD.
Psychosocial treatment. FMD often affects young, otherwise healthy women, and coping with it can be difficult. Psychologists or other mental health professionals can offer counseling to help patients deal with the stress and anxiety that may accompany having FMD.
Obstetrics/gynecological care. Specialists in obstetrics and gynecology can advise patients with FMD about the use of oral contraceptives, estrogen therapy and other hormone-based medications, which can affect blood flow in the arteries.
How long will treatment for FMD continue?
Although FMD can be managed successfully, it can recur in some patients. For this reason, people with FMD should continue to be monitored by their physicians even after interventional or surgical treatments are completed. Patients may be seen for follow-up visits every few months or once or twice a year, depending on their individual case. Physicians will monitor patients’ medications and assess signs of possible recurrence.


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