Weber's syndrome

Last updated
Weber's syndrome
Other namesMidbrain stroke syndrome Superior alternating hemiplegia
Weber's syndrome.svg
Midbrain cross section showing lesion
Specialty Neurology
Treatmentanything which can decrease the stroke's effect

Weber's syndrome, also known as midbrain stroke syndrome or superior alternating hemiplegia, is a form of stroke 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 and contralateral hemiparesis or hemiplegia.

Contents

Cause

It is mainly caused by a midbrain infarction as a result of occlusion of a branch of posterior cerebral artery most commonly or the paramedian branches of basilar bifurcation perforating arteries. [1] [2]

This lesion is usually unilateral and affects several structures in the midbrain including:

Structure damagedEffect
substantia nigra contralateral parkinsonism because it's dopaminergic projections to the basal ganglia innervate the ipsilateral hemisphere motor field, leading to a movement disorder of the contralateral body.
corticospinal fiberscontralateral hemiparesis (if lateral corticospinal tract is only affected) & contralateral hemiplegia (if both lateral & anterior corticospinal tracts are affected) and typical upper motor neuron findings. It is contralateral because it occurs before the decussation in the medulla.
corticobulbar tractdifficulty with contralateral lower facial muscles and hypoglossal nerve functions.
oculomotor nerve fibers ipsilateral lower motor neuron type oculomotor nerve palsy with a drooping eyelid (partial ptosis) and fixed wide pupil pointed down and out (mydriasis). This leads to diplopia.
Human brainstem blood supply description. Posterior cerebral artery is #6, and midbrain is behind it. Human brainstem blood supply description.JPG
Human brainstem blood supply description. Posterior cerebral artery is #6, and midbrain is behind it.

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.[ citation needed ]

Management

History

It carries the name of Sir Hermann David Weber, a German-born physician working in London, who described the condition in 1863. [3] [4] It is unrelated to Sturge–Weber syndrome, Klippel–Trénaunay–Weber syndrome or Osler–Weber–Rendu syndrome. These conditions are named for his son Frederick Parkes Weber.[ citation needed ]

See also

Related Research Articles

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Claude's syndrome is a form of brainstem stroke syndrome characterized by the presence of an ipsilateral oculomotor nerve palsy, contralateral hemiparesis, contralateral ataxia, and contralateral hemiplegia of the lower face, tongue, and shoulder. Claude's syndrome affects oculomotor nerve, red nucleus and brachium conjunctivum.

Alternating hemiplegia is a form of hemiplegia that has an ipsilateral cranial nerve palsies and contralateral hemiplegia or hemiparesis of extremities of the body. The disorder is characterized by recurrent episodes of paralysis on one side of the body. There are multiple forms of alternating hemiplegia, Weber's syndrome, middle alternating hemiplegia, and inferior alternating hemiplegia. This type of syndrome can result from a unilateral lesion in the brainstem affecting both upper motor neurons and lower motor neurons. The muscles that would receive signals from these damaged upper motor neurons result in spastic paralysis. With a lesion in the brainstem, this affects the majority of limb and trunk muscles on the contralateral side due to the upper motor neurons decussation after the brainstem. The cranial nerves and cranial nerve nuclei are also located in the brainstem making them susceptible to damage from a brainstem lesion. Cranial nerves III (Oculomotor), VI (Abducens), and XII (Hypoglossal) are most often associated with this syndrome given their close proximity with the pyramidal tract, the location which upper motor neurons are in on their way to the spinal cord. Damages to these structures produce the ipsilateral presentation of paralysis or palsy due to the lack of cranial nerve decussation before innervating their target muscles. The paralysis may be brief or it may last for several days, many times the episodes will resolve after sleep. Some common symptoms of alternating hemiplegia are mental impairment, gait and balance difficulties, excessive sweating and changes in body temperature.

References

  1. "Weber's syndrome" . GPnotebook.
  2. "Weber Syndrome". StatPearls. StatPearls. 2021.
  3. Weber HD (1863). "A contribution to the pathology of the crura cerebri". Medico-Chirurgical Transactions. 46: 121–139. doi:10.1177/095952876304600112. PMC   2147786 . PMID   20896209.
  4. Weber's syndrome at Who Named It?