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Benedict Syndrome

Hemiplegia Eye Movement Syndrome

Benedict syndrome (BS), also termed paramedian midbrain syndrome, is a rare stroke manifestation involving a lesion of the tegmentum of the midbrain. Infarctions of the red nucleus, cerebral peduncle, oculomotor fascicles, and the lower oculomotor nucleus are most commonly observed. BS manifestation is normally a consequence of infarcted branches of the posterior cerebral artery. BS is closely related to Weber's syndrome and Claude's syndrome.


Presentation

Benedict syndrome (BS) is caused by a stroke in the midbrain and usually presents as an ipsilateral oculomotor nerve palsy with contralateral hemi-tremor. Most common symptoms in BS are impaired eye movements affecting only one eye, hemichorea, slow one-sided tremor in the limbs, contralateral ataxia, dilated unresponsive pupils, ptosis, diplopia, hyperactive tendon reflexes, adiadokokinesis, contralateral hyperaesthesia and contralateral hemiparesis [1] [2] [3]. BS manifests with symptoms reminiscent of Weber's syndrome (WS) and Claude's syndrome (CS). BS is more often associated with hemiataxia, i.e. incoordination of movements, while WS more often presents with paralysis symptoms. An identifying symptom for BS in comparison to CS is contralateral hemiparesis, which never occurs in CS. Patients with BS have a rhythmic tremor of the contralateral hand and foot, which intensifies as a consequence of emotional excitement and during voluntary movements. Tremor is always absent during sleep in BS patients [4] [5].

BS is caused by a lesion, i.e. an infarction, hemorrhage or a tumor in the tegmentum of the midbrain and cerebellum. Infarctions of the posterior cerebral artery are suspected to be conducive to BS in most reported cases. Tuberculosis can also cause BS. Disruptions in the third cranial nerve, the red nucleus, the corticospinal tracts, the brachium conjunctivum, and the superior cerebellar peduncle decussation are normally associated with BS manifestations [1].

Motor anomalies in BS patients include a loss in arm swing on the affected side while the patient walks, a permanently semi-flexed arm and uncoordinated walking steps. These steps are characterized by swinging legs and a quick foot drop [4].

Cognitive functions remain unaffected in BS patients [1] [6].

Strabismus
  • Defisit occulomotorius termasuk ptosis, strabismus divergen, dan dilatasi pupil. Red nucleus berkoordinasi dari jaras cerebelar thalamic ke cereberel pedunkel superior (brachium conjunctivum).[docshare.tips]
  • With the eyelids retracted the right eye had a dilated pupil and a lateral strabismus. On attempted lateral gaze to the left the right eye would not proceed across the midline.[lksom.temple.edu]
  • Kelemahan otot-otot bola mata (opthalmoparese/opthalmoplegi) berupa:(1) gerakan terbatas, (2) kontraksi skunder dari anta-gonisnya, (3) strabismus, (4) diplopia 2.[dokmud.wordpress.com]
  • LR; medial strabismus, horizontal diplopia Dorsal Midbrain (Parinaud's Syndrome) Causes; pinealoma or germinoma 1. Superior colliculus and pretectal area. Paralysis of upward & downward gaze, pupillary disturbances & absence of convergence 2.[cram.com]
Tremor
  • Patients with BS have a rhythmic tremor of the contralateral hand and foot, which intensifies as a consequence of emotional excitement and during voluntary movements. Tremor is always absent during sleep in BS patients.[symptoma.com]
  • Lesi di red nucleus menghasilkan deficit dalam berkoordinasi kontralateral (ataxia), dysmetria, dysdiadokokinesis, rubral tremor ( resting tremor kasar yang meningkat apabila bergerak), dan pseudoparkinson tremor.[docshare.tips]
  • “rubral tremor (The blue fibers being the sup.[medicowesome.com]
  • […] hyperkinesias (rubral tremor).[jnnp.bmj.com]
  • Signs and symptoms [ edit ] It is characterized by the presence of an oculomotor nerve (CN III) palsy and cerebellar ataxia including tremor and involuntary choreoathetotic movements.[en.wikipedia.org]
Hemichorea
  • Most common symptoms in BS are impaired eye movements affecting only one eye, hemichorea, slow one-sided tremor in the limbs, contralateral ataxia, dilated unresponsive pupils, ptosis, diplopia, hyperactive tendon reflexes, adiadokokinesis, contralateral[symptoma.com]
  • This syndrome, first described in 1889 by Moritz Benedikt, is a constellation of symptoms including ipsilateral oculomotor palsy, cerebellar ataxia, and contralateral involuntary movements such as intention tremors, hemichorea or hemiathetosis.[acphospitalist.org]
  • The clinical manifestations are an ipsilateral third nerve paresis, usually with pupillary dilation, and a contralateral hemitremor, hemiathetosis, or hemichorea.[neupsykey.com]
Coarse Tremor
  • tremors Rostral basilar artery syndrome – occurs in 25% of cases of SCA occlusion – some of the following clinical features may be present visual field defect, vomiting, dizziness, dipoplia, limb clumsiness, weakness, paresthesia, drowsiness /- cortical[lifeinthefastlane.com]
Motor Symptoms
  • Although there are predominant motor symptoms, which predominate in the upper extremity because of the somatotopic organization of the corticospinal tract in the basis pontis, variable impaired touch and proprioception may also occur.[neupsykey.com]

Workup

Diagnosis of BS relies on a confirming the patient's stroke risk factors, on clinical investigations and on brain imaging studies. In selected cases with a possible connection to a tuberculosis infection, an analysis of cerebrospinal fluid is also necessary [1].

A patient history featuring a smoking habit, pronounced hypertension, prolonged untreated diabetes, hypercholesterolemia and a familial predisposition for stroke-related disorders are crucial parameters for the diagnosis [7][8].

Clinical examination should focus on obvious BS symptoms like ptosis, eye deviations, pupillary anomalies, contralateral hemiparesis, lack of voluntary motor coordination and tremors. Eye deviations occur because of an injury to the oculomotor nerve and force the eyes outward or downward [1].

Magnetic resonance imaging (MRI) and computer tomography (CT) scans are the final step in the workup since these methods provide the best chance to exclude other ailments with similar symptoms, e.g. cavernous sinus thrombosis. Both methods can detect disruptions in the above-mentioned midbrain areas [1] [9].

Deep brain stimulation may provide a valuable treatment option for the BS associated intention tremor [10].

Treatment

  • Deep brain stimulation may provide a valuable treatment option for the BS associated intention tremor.[symptoma.com]
  • The clinical features of this patient provide additional symptoms to the syndrome of PCA stenosis, and their recognition may hasten the diagnosis of PCA stenosis and appropriate treatment.[docshare.tips]
  • You'll confidently identify the signs and symptoms of disease, know when additional diagnostic testing is indicated, accurately interpret diagnostic data, and recommend effective treatment options.[books.google.com]
  • […] in long-term outcomes for both of these treatments.[patient.info]

Prognosis

  • Causes and prognosis in 4,278 cases of paralysis of the oculomotor, trochlear, and abducens cranial nerves. Am J Ophthalmol . 1992 ; 113(5) : 489 –496.[aao.org]
  • Prognosis This will depend on the extent of disease; however: Acute basilar artery occlusion has a very high mortality rate. Vertebrobasilar stroke usually leaves significant neurological deficits.[patient.info]
  • The rostral basilar artery syndrome: diagnosis, etiology, prognosis. Neurology 1989 ; 39 : 9 –16. Lhermitte F . Le syndrome cérebelleux: étude anatomo-clinique chez l’adulte. Rev Neurol 1958 ; 98 : 435 –77. Mehler MF .[jnnp.bmj.com]
  • Bilateral occlusions are much rarer than unilateral, and have a slowly progressive course and poor prognosis (Caplan, 1983). MRA combined with MRI is usually the best way to make this diagnosis.[dizziness-and-balance.com]

Etiology

  • From Jump to: navigation , search Contents 1 Etiology 2 Epidemiology 3 Pathology 4 Clinical-manifestations 5 Management 6 More General Terms 7 References Etiology ischemic stoke hemorrhagic stroke brain tumor tuberculosis Epidemiology rare Pathology stroke[anvita.info]
  • . • Small vessel disease is the prime etiology in thalamic infarcts. • The etiology of midbrain infarcts remains undetermined in up to 50% of cases.[medlink.com]
  • The original reference is provided for historical interest, and review articles are included to show recent advances in etiology and treatment.[books.google.com]
  • It shows the main etiologic cerebral infarcts are not always well explored. Indi aspects.[books.google.com]
  • Progression of ocular misalignment beyond 2 weeks or failure to improve within 3 months is inconsistent with this cause of cranial neuropathy and should prompt a thorough evaluation for another etiology.[aao.org]

Epidemiology

  • From Jump to: navigation , search Contents 1 Etiology 2 Epidemiology 3 Pathology 4 Clinical-manifestations 5 Management 6 More General Terms 7 References Etiology ischemic stoke hemorrhagic stroke brain tumor tuberculosis Epidemiology rare Pathology stroke[anvita.info]
  • Epidemiology A relatively uncommon form of stroke.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • […] signification worked out study than one exclusively devoted to a of imaging and magnetic resonance imaging, which limited number of scientific papers. will perhaps allow us to detect ischemic edema in the After a brief clinical and pathophysiological[books.google.com]
  • This is probably because the pathophysiology of the third cranial nerve lesion involves leakage of blood from the aneurysm dome into the nerve across its outer margin.[emedicine.medscape.com]
  • Pathophysiology Atherosclerosis : the most common vascular disease affecting the vertebrobasilar system: [ 1 ] This affects large vessels, causing narrowing and occlusion.[patient.info]
  • The pathophysiology is not fully understood, but defects in T-cell and humoral homeostasis have been implicated in villous injury.[acphospitalist.org]

Prevention

  • Seite 1607 - Centers for Disease Control and Prevention: 1994 Revised Classification System for Human Immunodeficiency Virus Infection in Children Less Than 13 Years of Age, MMWR, 43, 1-19, No. ‎[books.google.com]
  • Caucasian patients were more likely than African Americans to receive treatment , while the middle to upper classes were more likely to receive treatment than the ... obstacles that prevented equal access to HIV/AIDs treatment ...[liquisearch.com]
  • Even with vertebral artery occlusion, collaterals (circle of Willis) may prevent ischaemia.[patient.info]
  • In this article, the authors present in depth the clinical correlates of midbrain and thalamic ischemic lesions, while also summarizing the advances in treatment and prevention of ischemic lesions involving the different vascular territories of the thalamus[medlink.com]
  • Serologic samples sent to the Centers for Disease Control and Prevention (CDC) and the Rhode Island Department of Health returned positive for anti-chikungunya virus IgM antibodies. The patient was discharged home after 5 days of supportive care.[acphospitalist.org]

References

Article

  1. Ruchalski K, Hathout GM. A Medley of Midbrain Maladies: A Brief Review of Midbrain Anatomy and Syndromology for Radiologists. Radiol Res Pract. 2012;258524.
  2. Khan AO. Pupil-sparing complete third nerve palsy from cryptogenic midbrain stroke in an otherwise healthy young adult with patent foramen ovale. 2012; 19(2): 237–239.
  3. Cormier PJ, Long ER, Russell EJ. MR imaging of posterior fossa infarctions: vascular territories and clinical correlates. Radiographics. 1992; 12(6):1079-1096.
  4. Fujieda T, Yamauchi T, Takahasi S, Moroji T. Letter: Effect of levodopa on tremor in Benedikt's syndrome. Br Med J. 1974; 1(5905):456-457.
  5. Afifi AK, Bergman RA. Functional Neuroanatomy: Text and Atlas. New York, NY: McGraw-Hill Companies, Health Professions Division; 1998.
  6. Hebb ALO, Brandman D, Shankar JAI, Hebb AO. Visualizing Recovery of Cognitive Function in Stroke. Journal of Behavioral and Brain Science. 2013; 3: 641-652.
  7. Spence JD. Intensive risk factor control in stroke prevention. F1000Prime Rep. 2013; 5:42.
  8. Nicol MB, Thrift AG. Knowledge of Risk Factors and Warning Signs of Stroke. Vasc Health Risk Manag. 2005; 1(2): 137–147.
  9. Nouh A, Remke J, Ruland S. Ischemic Posterior Circulation Stroke: A Review of Anatomy, Clinical Presentations, Diagnosis, and Current Management. Front Neurol. 2014; 5:30.
  10. Bandt SK, Anderson D, Biller J. Deep brain stimulation as an effective treatment option for post-midbrain infarction-related tremor as it presents with Benedikt syndrome. J Neurosurg. 2008; 109(4):635-639.

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Last updated: 2018-06-22 07:28