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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].

Eyes

  • Strabismus

    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]

    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]

    Defisit occulomotorius termasuk ptosis, strabismus divergen, dan dilatasi pupil. Red nucleus berkoordinasi dari jaras cerebelar thalamic ke cereberel pedunkel superior (brachium conjunctivum). [docshare.tips]

    • Lesions of the internal genu of the facial nerve cause: -Ipsilateral facial paralysis -Ipsilateral loss of the corneal reflex • Lesions of the abducent nucleus cause: -Lateral rectus paralysis -Medial (convergent) strabismus -Horizontal diplopia 20 [slideshare.net]

Neurologic

  • 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]

    Benedikt syndrome - hemiplegia with clonic spasm or tremor and oculomotor paralysis on the opposite side. Medical Eponyms © Farlex 2012 [medical-dictionary.thefreedictionary.com]

    He described three similar cases involving oculomotor palsy, contralateral hemiparesis, and contralateral tremors. [pubmed.ncbi.nlm.nih.gov]

    He described three similar cases involving oculomotor palsy, contralateral hemiparesis, and contralateral tremors.[1] One of these cases was a 4-year-old with a right oculomotor palsy, contralateral hand jerking/tremor, contralateral involuntary leg movements [ncbi.nlm.nih.gov]

  • 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]

  • Postural Instability

    Damage or loss of function (as is the case seen in Parkinson disease) causes resting tremor, postural instability, and difficulty in initiating movement.[17] Cerebral peduncle: contain the main descending motor pathways, including the corticospinal tract [statpearls.com]

  • Coarse Tremor

    […] and titubation – both static and kinetic tremors occur with cerebellar damage and much of the amplitude of the tremor may arise from instability of the proximal muscles of the involved limb – the most common cerebellar tremor is a side-to-side kinetic [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

/ Management Owing to the varied etiology of this syndrome, treatment is dependent on the underlying pathology. [statpearls.com]

[…] were more likely to receive treatment than the poor ... that prevented equal access to HIV/AIDs treatment ... [liquisearch.com]

Deep brain stimulation as an effective treatment option for post-midbrain infarction-related tremor as it presents with Benedikt syndrome. Bandt SK, Anderson D, Biller J. Bandt SK, et al. [pubmed.ncbi.nlm.nih.gov]

“Deep brain stimulation as an effective treatment option for post-midbrain infarction-related tremor as it presents with Benedikt syndrome”. Journal of Neurosurgery. 109 (4): 635–639. doi : 10.3171/JNS/2008/109/10/0635. PMID 18826349. [checkrare.com]

Prognosis

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Pearls and Other Issues Enhancing [pubmed.ncbi.nlm.nih.gov]

Underlying structures involved are thought to be the superior cerebellar peduncle and tectum of the midbrain.[22][21] Prognosis Prognosis varies widely according to the underlying etiology. [statpearls.com]

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]

Etiology

Underlying structures involved are thought to be the superior cerebellar peduncle and tectum of the midbrain.[22][21] Prognosis Prognosis varies widely according to the underlying etiology. [statpearls.com]

In this article, we reported a case of Benedikt syndrome with an etiologic cause of cardioembolic stroke, who was diagnosed by the neurological examination and neuroradiological findings. [ingentaconnect.com]

These first and subsequent descriptions over the next 40 years describe patients with Benedikt syndrome with slightly varying symptoms, etiology, and lesion location. [pubmed.ncbi.nlm.nih.gov]

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]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Pearls and Other Issues Enhancing [pubmed.ncbi.nlm.nih.gov]

Due to the extremely low frequency of reported cases, there remain no epidemiological studies outlying the frequency of this condition. [statpearls.com]

Epidemiology A relatively uncommon form of stroke. [patient.info]

Pathophysiology

Medicine, Biology Cerebellum 2022 The internist Hermann Nothnagel (1841-1905) took a special interest in the cerebellum and demonstrated the involvement of the vermis in the pathophysiology of motor ataxia, culminating in the Cerebellar Classic highlighted [semanticscholar.org]

[…] 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]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Deterrence and Patient Education Pearls and Other Issues Enhancing [pubmed.ncbi.nlm.nih.gov]

However, the condition has been reported in both sexes and from around the world.[1][9][4] Pathophysiology Understanding the underlying anatomy and function of mesencephalic structures is key to understanding why specific damage in the tegmentum of the [statpearls.com]

Prevention

الصفحة 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]

Lowering of insulin levels Restoration of fertility Treatment of hirsutism or acne Restoration of regular menstruation, and prevention of endometrial hyperplasia and endometrial cancer In each of these ... the major reasons for this is the lack of large [liquisearch.com]

Even with vertebral artery occlusion, collaterals (circle of Willis) may prevent ischaemia. [patient.info]

Doctors should advise their patients to engage with healthy lifestyles to prevent cerebrovascular accidents.[18] Pearls and Other Issues Damage from any etiology to the red nucleus, oculomotor fascicles, cerebral peduncle, and substantia nigra causes [statpearls.com]

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]

References

  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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