Lateral medullary syndrome
Encyclopedia
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.
) 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
, slurred speech, ataxia
, facial pain, vertigo
, nystagmus, Horner syndrome, diplopia
, and possibly palatal myoclonus
.
Affected persons have difficulty in swallowing (dysphagia
) resulting from involvement of the nucleus 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 absent corneal reflex
.
The spinothalamic tract
is damaged, resulting in loss of pain and temperature sensation to the opposite side of the body. The damage to the cerebellum
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
.
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.
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.
One of the most unique and difficult to treat symptoms that occur due to Wallenberg syndrome are interminable, violent hiccups. The hiccup
s can be so severe that patients often struggle to eat, sleep and carry on conversations. Depending on the severity of the blockage caused by the stroke
, 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.
,. First descriptions by Wallenberg
were in 1895 (clinical) and 1901 (autopsy findings).
Adolf Wallenberg
Adolf Wallenberg was a German internist and neurologist.Wallenberg was born in Preussisch Stargard. He studied at Heidelberg and Leipzig, receiving his doctorate from the latter University in 1886. From 1886 to 1888 he was assistant in the Städtisches Krankenhaus in Danzig, where he settled as a...
syndrome and posterior inferior cerebellar artery
Posterior inferior cerebellar artery
The posterior inferior cerebellar artery , the largest branch of the vertebral artery, is one of the three main arterial blood supplies for the cerebellum, part of the brain...
syndrome) is a disease in which the patient has a constellation of neurologic symptoms due to injury to the lateral part of the medulla
Medulla oblongata
The medulla oblongata is the lower half of the brainstem. In discussions of neurology and similar contexts where no ambiguity will result, it is often referred to as simply the medulla...
in the brain, resulting in tissue ischemia and necrosis.
Signs and symptoms
This syndrome is characterized by sensory deficits affecting the trunk (torsoTorso
Trunk or torso is an anatomical term for the central part of the many animal bodies from which extend the neck and limbs. The trunk includes the thorax and abdomen.-Major organs:...
) 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
Dysphagia
Dysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their dysphagia....
, slurred speech, ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
, facial pain, vertigo
Vertigo (medical)
Vertigo is a type of dizziness, where there is a feeling of motion when one is stationary. The symptoms are due to a dysfunction of the vestibular system in the inner ear...
, nystagmus, Horner syndrome, diplopia
Diplopia
Diplopia, commonly known as double vision, is the simultaneous perception of two images of a single object that may be displaced horizontally, vertically, or diagonally in relation to each other...
, and possibly palatal myoclonus
Palatal myoclonus
Palatal myoclonus is a rapid spasm of the palatal muscles, which results in clicking or popping in the ear. Chronic clonus is often due to lesions of the central tegmental tract...
.
Affected persons have difficulty in swallowing (dysphagia
Dysphagia
Dysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their dysphagia....
) resulting from involvement of the nucleus ambiguus
Nucleus ambiguus
The nucleus ambiguus is a region of histologically disparate cells located just dorsal to the inferior olivary nucleus in the lateral portion of the upper medulla...
, as well as slurred speech (dysarthria
Dysarthria
Dysarthria is a motor speech disorder resulting from neurological injury of the motor component of the motor-speech system and is characterized by poor articulation of phonemes...
)and disordered vocal quality (dysphonia
Dysphonia
Dysphonia is the medical term for disorders of the voice: an impairment in the ability to produce voice sounds using the vocal organs . Thus, dysphonia is a phonation disorder...
) . Damage to the spinal trigeminal nucleus causes absence of pain on the ipsilateral side of the face, as well as an absent corneal reflex
Corneal reflex
The corneal reflex, also known as the blink reflex, is an involuntary blinking of the eyelids elicited by stimulation of the cornea , or bright light, though could result from any peripheral stimulus. Stimulation should elicit both a direct and consensual response...
.
The spinothalamic tract
Spinothalamic tract
The spinothalamic tract is a sensory pathway originating in the spinal cord. It transmits information to the thalamus about pain, temperature, itch and crude touch...
is damaged, resulting in loss of pain and temperature sensation to the opposite side of the body. The damage to the cerebellum
Cerebellum
The cerebellum is a region of the brain that plays an important role in motor control. It may also be involved in some cognitive functions such as attention and language, and in regulating fear and pleasure responses, but its movement-related functions are the most solidly established...
or the inferior cerebellar peduncle
Inferior cerebellar peduncle
The upper part of the posterior district of the medulla oblongata is occupied by the inferior cerebellar peduncle , a thick rope-like strand situated between the lower part of the fourth ventricle and the roots of the glossopharyngeal and vagus nerves.Each cerebellar inferior peduncle connects the...
can cause ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
. Damage to the hypothalamospinal fibers disrupts sympathetic nervous system
Sympathetic nervous system
The sympathetic nervous system is one of the three parts of the autonomic nervous system, along with the enteric and parasympathetic systems. Its general action is to mobilize the body's nervous system fight-or-flight response...
relay and gives symptoms analogous to Horner syndrome.
Nystagmus
Nystagmus
Nystagmus is a condition of involuntary eye movement, acquired in infancy or later in life, that may result in reduced or limited vision.There are two key forms of Nystagmus: pathological and physiological, with variations within each type. Nystagmus may be caused by congenital disorders,...
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
Vertigo
Vertigo is a form of dizziness.Vertigo may also refer to:* Vertigo , a 1958 film by Alfred Hitchcock**Vertigo , its soundtrack** Vertigo effect, or Dolly zoom, a special effect in film, named after the movie...
.
Palatal myoclonus may be observed due to disruption of the central tegmental tract
Central tegmental tract
The central tegmental tract is a structure in the midbrain and pons.* The central tegmental tract includes ascending axonal fibers that arise from the caudal nucleus solitarius and terminate in the ventral posteromedial nucleus of thalamus...
.
Features
| Dysfunction | Effects >- | vestibular nuclei Vestibular nuclei The vestibular nuclei are the cranial nuclei for the vestibular nerve.In Terminologia Anatomica they are grouped in both the pons and medulla.-Subnuclei:There are 4 subnuclei; they are situated at the floor of the fourth ventricle.... |
vestibular system Vestibular system The vestibular system, which contributes to balance in most mammals and to the sense of spatial orientation, is the sensory system that provides the leading contribution about movement and sense of balance. Together with the cochlea, a part of the auditory system, it constitutes the labyrinth of... : vomiting Vomiting Vomiting is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose... , vertigo Vertigo (medical) Vertigo is a type of dizziness, where there is a feeling of motion when one is stationary. The symptoms are due to a dysfunction of the vestibular system in the inner ear... , nystagmus, >- | inferior cerebellar peduncle Inferior cerebellar peduncle The upper part of the posterior district of the medulla oblongata is occupied by the inferior cerebellar peduncle , a thick rope-like strand situated between the lower part of the fourth ventricle and the roots of the glossopharyngeal and vagus nerves.Each cerebellar inferior peduncle connects the... |
ataxia Ataxia Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum... , dysmetria (past pointing), dysdiadokokinesia >- | central tegmental tract Central tegmental tract The central tegmental tract is a structure in the midbrain and pons.* The central tegmental tract includes ascending axonal fibers that arise from the caudal nucleus solitarius and terminate in the ventral posteromedial nucleus of thalamus... |
palatal myoclonus Palatal myoclonus Palatal myoclonus is a rapid spasm of the palatal muscles, which results in clicking or popping in the ear. Chronic clonus is often due to lesions of the central tegmental tract... >- | lateral spinothalamic tract Lateral spinothalamic tract The lateral spinothalamic tract , which is a part of the Anterolateral system, is a bundle of sensory axons ascending through the white matter of the spinal cord, carrying sensory information to the brain. It carries pain and temperature sensory information to the thalamus of the brain... |
>- | >- | dysphagia Dysphagia Dysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their dysphagia.... , hoarseness, diminished gag reflex Gag reflex The pharyngeal reflex or gag reflex is a reflex contraction of the back of the throat, evoked by touching the soft palate or sometimes the back of the tongue. It prevents something from entering the throat except as part of normal swallowing and helps prevent choking... (efferent limb - CN.X) >- | descending sympathetic fibers |
ipsilateral Horner's syndrome Horner's syndrome Horner's syndrome is the combination of drooping of the eyelid and constriction of the pupil , sometimes accompanied by decreased sweating of the face on the same side; redness of the conjunctiva of the eye is often also present... (ptosis, miosis, & anhydrosis) |
Cause
Posterior inferior cerebellar artery
The posterior inferior cerebellar artery , the largest branch of the vertebral artery, is one of the three main arterial blood supplies for the cerebellum, part of the brain...
(PICA) or one of its branches or of the vertebral artery
Vertebral artery
The vertebral arteries are major arteries of the neck. They branch from the subclavian arteries and merge to form the single midline basilar artery in a complex called the vertebrobasilar system, which supplies blood to the posterior part of the circle of Willis and thus significant portions of the...
, in which the lateral part of the medulla oblongata
Medulla oblongata
The medulla oblongata is the lower half of the brainstem. In discussions of neurology and similar contexts where no ambiguity will result, it is often referred to as simply the medulla...
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.
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
Gastrostomy
Gastrostomy refers to a surgical opening into the stomach. Creation of an artificial external opening into the stomach for nutritional support or gastrointestinal compression....
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
Aspiration
Aspiration may refer to:In linguistics:*Aspirated consonant, a plosive pronounced with a strong burst of air*Debuccalization, the conversion of a consonant to [h] or [ʔ]*Voiceless glottal fricative In engine technology:...
pneumonia
Pneumonia
Pneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...
. 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
Gabapentin
Gabapentin is a pharmaceutical drug, specifically a GABA analogue. It was originally developed for the treatment of epilepsy, and currently is also used to relieve neuropathic pain...
. 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.
One of the most unique and difficult to treat symptoms that occur due to Wallenberg syndrome are interminable, violent hiccups. The hiccup
Hiccup
A hiccup or hiccough is a myoclonus of the diaphragm that repeats several times per minute. In humans, the abrupt rush of air into the lungs causes the vocal cords to close, creating a "hic" sound....
s can be so severe that patients often struggle to eat, sleep and carry on conversations. Depending on the severity of the blockage caused by the stroke
Stroke
A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...
, 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
Warfarin
Warfarin is an anticoagulant. It is most likely to be the drug popularly referred to as a "blood thinner," yet this is a misnomer, since it does not affect the thickness or viscosity of blood...
. Patients will often remain on these medications or an aspirin
Aspirin
Aspirin , also known as acetylsalicylic acid , is a salicylate drug, often used as an analgesic to relieve minor aches and pains, as an antipyretic to reduce fever, and as an anti-inflammatory medication. It was discovered by Arthur Eichengrun, a chemist with the German company Bayer...
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.
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.History
This syndrome was first described in 1808 by Gaspard VieusseuxGaspard Vieusseux
Gaspard Vieusseux was a Swiss physician who was born in Geneva. In 1766 he obtained his doctorate from the University of Leiden, and subsequently returned to Geneva to practice medicine....
,. First descriptions by Wallenberg
Adolf Wallenberg
Adolf Wallenberg was a German internist and neurologist.Wallenberg was born in Preussisch Stargard. He studied at Heidelberg and Leipzig, receiving his doctorate from the latter University in 1886. From 1886 to 1888 he was assistant in the Städtisches Krankenhaus in Danzig, where he settled as a...
were in 1895 (clinical) and 1901 (autopsy findings).
See also
- Stroke Recovery
- Medial medullary syndromeMedial medullary syndromeMedial medullary syndrome, also known as inferior alternating syndrome, hypoglossal alternating hemiplegia, lower alternating hemiplegia, or Dejerine syndrome, is a type of alternating hemiplegia characterized by a set of clinical features resulting from occlusion of the anterior spinal artery...
- Weber syndrome
- Benedikt syndrome
External links
- Lateral Medullary Syndrome or Wallenberg Syndrome| Medchrome
- Department of Biomedical Engineering at Johns Hopkins UniversityJohns Hopkins UniversityThe Johns Hopkins University, commonly referred to as Johns Hopkins, JHU, or simply Hopkins, is a private research university based in Baltimore, Maryland, United States...
- MRI of Lateral Medullary Infarction (Wallenberg) MedPix Images