Weber's Syndrome
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Weber's syndrome, also known as midbrain stroke syndrome or superior alternating hemiplegia, is a form of
stroke A stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functionin ...
that affects the medial portion of the midbrain. It involves oculomotor fascicles in the interpeduncular cisterns and cerebral peduncle so it characterizes the presence of an ipsilateral lower motor neuron type
oculomotor nerve palsy Oculomotor nerve palsy or oculomotor neuropathy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. As the name suggests, the oculomotor nerve supplies the majority of the muscles controlling eye movements (fou ...
and contralateral
hemiparesis Hemiparesis, or unilateral paresis, is weakness of one entire side of the body (''wikt:hemi-#Prefix, hemi-'' means "half"). Hemiplegia is, in its most severe form, complete paralysis of half of the body. Hemiparesis and hemiplegia can be caused ...
or hemiplegia.


Cause

It is mainly caused by a
midbrain The midbrain or mesencephalon is the forward-most portion of the brainstem and is associated with vision, hearing, motor control, sleep and wakefulness, arousal (alertness), and temperature regulation. The name comes from the Greek ''mesos'', " ...
infarction Infarction is tissue death (necrosis) due to inadequate blood supply to the affected area. It may be caused by artery blockages, rupture, mechanical compression, or vasoconstriction. The resulting lesion is referred to as an infarct (from the ...
as a result of occlusion of a branch of
posterior cerebral artery The posterior cerebral artery (PCA) is one of a pair of cerebral arteries that supply oxygenated blood to the occipital lobe, part of the back of the human brain. The two arteries originate from the distal end of the basilar artery, where it bifur ...
most commonly or the paramedian branches of basilar bifurcation perforating arteries. This lesion is usually unilateral and affects several structures in the midbrain including:


Diagnosis

Clinical findings mainly eyeball is down and out ipsilateral lateral squint. Ptosis present as the levator palpebrae superioris nerve supply is disrupted. Pupil dilated and fixed. Contralateral hemiplegia CT scan or MRI might help in delineating the cause or the vessel or region of brain involved in stroke.


Management


History

It carries the name of Sir
Hermann David Weber Sir Herman David Weber FRCP (30 December 1823 – 11 November 1918) was a German physician who practiced medicine in England. Biography Weber attended Gymnasium in Fulda in 1838, but started his medical studies at Marburg University in 18 ...
, a German-born physician working in London, who described the condition in 1863. It is unrelated to
Sturge–Weber syndrome Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, i ...
, Klippel–Trénaunay–Weber syndrome or
Osler–Weber–Rendu syndrome Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease and Osler–Weber–Rendu syndrome, is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, ...
. These conditions are named for his son
Frederick Parkes Weber Frederick Parkes Weber (8 May 1863 – 2 June 1962) was an English dermatologist and author who practiced medicine in London. Background Weber's father, Sir Hermann David Weber (1823–1918), was a personal physician to Queen Victoria. ...
.


See also

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Alternating hemiplegia of childhood Alternating hemiplegia of childhood (AHC) is an ultra-rare neurological disorder named for the transient episodes, often referred to as "attacks", of hemiplegia (weakness or paralysis) from which those with the disorder suffer. It typically present ...
*
Lateral medullary syndrome 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 p ...
*
Lateral pontine syndrome 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. Symptoms Damage to the following areas produces symptoms (from me ...
*
Medial medullary 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 result ...
*
Medial pontine syndrome Medial inferior pontine syndrome is a condition associated with a contralateral hemiplegia."Medial inferior pontine syndrome" has been described as equivalent to Foville's syndrome. Presentation Although medial pontine syndrome has many similari ...


References


External links

{{Lesions of spinal cord and brainstem Stroke Syndromes affecting the nervous system