Superior Alternating Hemiplegia
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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, a German-born physician working in London, who described the condition in 1863. It is unrelated to Sturge–Weber syndrome, 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

* Alternating hemiplegia of childhood * Lateral medullary syndrome * Lateral pontine syndrome * Medial medullary syndrome * Medial pontine syndrome


References


External links

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