Lateral pontine syndrome | |
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Pons | |
Specialty | Neurology |
Lateral pontine syndrome, also known as Marie-Foix syndrome or Marie-Foix-Alajouanine syndrome [1] is one of the brainstem stroke syndromes of the lateral aspect of the pons. A lateral pontine syndrome is a lesion which is similar to the lateral medullary syndrome, but because it occurs in the pons, it also involves the cranial nerve nuclei of the pons. [2]
Lateral pontine syndrome was first described in France by French neurologists Pierre Marie (1853-1940), Charles Foix (1882-1927), and Théophile Alajouanine (1890-1980) in 1922. They were the first to identify and describe the symptoms and causes of this syndrome. In their original description, they reported findings from autopsies that showed spinal cord necrosis and multiple tortuous and thickened blood vessels on the surface of the spinal cord. This condition was later called necrotizing myelopathy. [3]
They emphasized that in their two cases, no thrombosis was present. They considered the vascular component of the entity they reported to be a wall thickening, without luminal narrowing or obliteration of the cord vessels (arteries as well as veins). They addressed, and ruled out, the possibility of vascular malformations. [4]
Damage to the following areas produces symptoms (from medial to lateral):
Structure affected | Effect |
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Lateral spinothalamic tract | Contralateral loss of pain and temperature from the trunk and extremities. |
Facial nucleus & facial Nerve (CN.VII) | (1) Ipsilateral paralysis of the upper and lower face (lower motor neuron lesion). (2) Ipsilateral loss of lacrimation and reduced salivation. (3) Ipsilateral loss of taste from the anterior two-thirds of the tongue. (4) Loss of corneal reflex (efferent limb). |
Principal sensory trigeminal nucleus and tract | Ipsilateral loss of all sensory modalities to the face (facial hemianesthesia) |
Vestibular Nuclei and intraaxial nerve fibers | Nystagmus, nausea, vomiting, and vertigo |
Cochlear nuclei and intraaxial nerve fibers | Hearing loss - ipsilateral central deafness |
Middle & inferior cerebellar peduncle | Ipsilateral limb and gait ataxia |
Descending sympathetic tract | Ipsilateral Horner's syndrome (ptosis, miosis, & anhydrosis) |
The syndrome occurs due to occlusion of perforating branches of the basilar and anterior inferior cerebellar (AICA) arteries. [5] [6] [7] It can also be caused by an interruption to the blood supply of the anterior inferior cerebellar artery or circumferential arteries. [8]
The treatment for lateral pontine syndrome varies greatly, so there are different medications for different symptoms. [9] Sometimes blood thinning agents are prescribed to remove blood flow hindrance. [10] Other than these medications, physical therapy is also necessary [11]
An arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system. Usually congenital, this vascular anomaly is widely known because of its occurrence in the central nervous system, but can appear anywhere in the body. The symptoms of AVMs can range from none at all to intense pain or bleeding, and they can lead to other serious medical problems.
Foix–Alajouanine syndrome, also called subacute ascending necrotizing myelitis, is a disease caused by an arteriovenous malformation of the spinal cord. In particular, most cases involve dural arteriovenous malformations that present in the lower thoracic or lumbar spinal cord. The condition is named after Charles Foix and Théophile Alajouanine who first described the condition in 1926.
The medulla oblongata or simply medulla is a long stem-like structure which makes up the lower part of the brainstem. It is anterior and partially inferior to the cerebellum. It is a cone-shaped neuronal mass responsible for autonomic (involuntary) functions, ranging from vomiting to sneezing. The medulla contains the cardiovascular center, the respiratory center, vomiting and vasomotor centers, responsible for the autonomic functions of breathing, heart rate and blood pressure as well as the sleep–wake cycle. "Medulla" is from Latin, ‘pith or marrow’. And "oblongata" is from Latin, ‘lengthened or longish or elongated'.
The pons is part of the brainstem that in humans and other mammals, lies inferior to the midbrain, superior to the medulla oblongata and anterior to the cerebellum.
The brainstem is the posterior stalk-like part of the brain that connects the cerebrum with the spinal cord. In the human brain the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is continuous with the thalamus of the diencephalon through the tentorial notch, and sometimes the diencephalon is included in the brainstem.
Lateral medullary syndrome is a neurological disorder causing a range of symptoms due to ischemia in the lateral part of the medulla oblongata in the brainstem. The ischemia is a result of a blockage most commonly in the vertebral artery or the posterior inferior cerebellar artery. Lateral medullary syndrome is also called Wallenberg's syndrome, posterior inferior cerebellar artery (PICA) syndrome and vertebral artery syndrome.
Medial 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. This results in the infarction of medial part of the medulla oblongata.
The basilar artery is one of the arteries that supplies the brain with oxygen-rich blood.
Brain herniation is a potentially deadly side effect of very high pressure within the skull that occurs when a part of the brain is squeezed across structures within the skull. The brain can shift across such structures as the falx cerebri, the tentorium cerebelli, and even through the foramen magnum. Herniation can be caused by a number of factors that cause a mass effect and increase intracranial pressure (ICP): these include traumatic brain injury, intracranial hemorrhage, or brain tumor.
Vertebrobasilar insufficiency (VBI) describes a temporary set of symptoms due to decreased blood flow (ischemia) in the posterior circulation of the brain. The posterior circulation supplies the medulla, pons, midbrain, cerebellum and supplies the posterior cerebellar artery to the thalamus and occipital cortex. As a result, symptoms vary widely depending which brain region is predominantly affected.
The anterior inferior cerebellar artery (AICA) is one of three pairs of arteries that supplies blood to the cerebellum.
The basilar part of pons, also known as basis pontis, or basilar pons, is the ventral part of the pons in the brainstem; the dorsal part is known as the pontine tegmentum.
Posterior spinal artery syndrome(PSAS), also known as posterior spinal cord syndrome, is a type of incomplete spinal cord injury. PSAS is the least commonly occurring of the six clinical spinal cord injury syndromes, with an incidence rate of less than 1%.
The central tegmental tract is a tract that carries ascending and descending fibers, situated in the midbrain tegmentum, and the pontine tegmentum. The tract is situated in the central portion of the reticular formation.
Vascular myelopathy refers to an abnormality of the spinal cord in regard to its blood supply. The blood supply is complicated and supplied by two major vessel groups: the posterior spinal arteries and the anterior spinal arteries—of which the Artery of Adamkiewicz is the largest. Both the posterior and anterior spinal arteries run the entire length of the spinal cord and receive anastomotic (conjoined) vessels in many places. The anterior spinal artery has a less efficient supply of blood and is therefore more susceptible to vascular disease. Whilst atherosclerosis of spinal arteries is rare, necrosis in the anterior artery can be caused by disease in vessels originating from the segmental arteries such as atheroma or aortic dissection.
The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal cord is hollow and contains a structure called the central canal, which contains cerebrospinal fluid. The spinal cord is also covered by meninges and enclosed by the neural arches. Together, the brain and spinal cord make up the central nervous system.
Cobb syndrome is a rare congenital disorder characterized by visible skin lesions and spinal angiomas or arteriovenous malformations (AVMs). The skin lesions of Cobb syndrome typically are present as port wine stains or angiomas, but reports exist of angiokeratomas, angiolipomas, and lymphangioma circumscriptum. The intraspinal lesions may be angiomas or AVMs and occur at levels of the spinal cord corresponding to the affected skin dermatomes. They may in turn produce spinal cord dysfunction and weakness or paralysis.
A brainstem stroke syndrome falls under the broader category of stroke syndromes, or specific symptoms caused by vascular injury to an area of brain. As the brainstem contains numerous cranial nuclei and white matter tracts, a stroke in this area can have a number of unique symptoms depending on the particular blood vessel that was injured and the group of cranial nerves and tracts that are no longer perfused. Symptoms of a brainstem stroke frequently include sudden vertigo and ataxia, with or without weakness. Brainstem stroke can also cause diplopia, slurred speech and decreased level of consciousness. A more serious outcome is locked-in syndrome.
The Anatomy of the Cerebellum can be viewed at three levels. At the level of gross anatomy, the cerebellum consists of a tightly folded and crumpled layer of cortex, with white matter underneath, several deep nuclei embedded in the white matter, and a fluid-filled ventricle in the middle. At the intermediate level, the cerebellum and its auxiliary structures can be broken down into several hundred or thousand independently functioning modules or compartments known as microzones. At the microscopic level, each module consists of the same small set of neuronal elements, laid out with a highly stereotyped geometry.
Cerebellar degeneration is a condition in which cerebellar cells, otherwise known as neurons, become damaged and progressively weaken in the cerebellum. There are two types of cerebellar degeneration; paraneoplastic cerebellar degeneration, and alcoholic or nutritional cerebellar degeneration. As the cerebellum contributes to the coordination and regulation of motor activities, as well as controlling equilibrium of the human body, any degeneration to this part of the organ can be life-threatening. Cerebellar degeneration can result in disorders in fine movement, posture, and motor learning in humans, due to a disturbance of the vestibular system. This condition may not only cause cerebellar damage on a temporary or permanent basis, but can also affect other tissues of the central nervous system, those including the cerebral cortex, spinal cord and the brainstem.