Brain stem infarction is a potentially life-threatening condition which develops due to focal ischemia of the midbrain, pons, and medulla oblongata. It results in neurologic deficits involving the respiratory, cardiovascular, speech, swallowing, hearing and ocular movement centers located within the brain stem. Diagnosis is based on clinical presentation with computed tomography and magnetic resonance imaging helping to confirm the extent of the infarct.
Presentation
The brain stem consists of the midbrain, pons, and medulla. Brain stem infarction commonly occurs due to an embolus occluding large arteries (vertebral or basilar arteries) supplying it or due to an embolus traveling from the heart, or atherosclerotic plaques from the large arteries (vertebral, basilar) [1]. Brainstem infarcts are relatively less common as compared to hemispheric infarcts [2]. The common clinical presentation is sudden onset focal neurological deficits like hemiparesis, vertigo, orthostatic symptoms, diplopia, and lower cranial nerve paralysis with dysphagia, and hoarseness. Vertigo is the cardinal feature of brain stem infarction [2] followed by ipsilateral cranial nerve symptoms, contralateral hemiplegia, hemianesthesia (crossed motor or sensory signs) with bilateral involvement of the sensory tract or motor tract or both.
A few uncommon clinical features of brain stem infarction include "salt and pepper" facial pain [3], blepharospasm [4], palatal myoclonus [2], hiccough [5], tinnitus in medullary infarction [6], bruxism and trismus in basilar artery occlusion [7], and peduncular hallucinosis in midbrain infarction [8]. If there is total occlusion of the vertebrobasilar system then patients can present with tachycardia, fluctuating blood pressure, ataxia, impaired consciousness, and coma leading to death. Pontine or midbrain infarction can also present as "locked-in syndrome" in which the affected individual has sudden onset vomiting, transient loss of consciousness with quadriplegia, bilateral facial palsy, anarthria (total loss of articulation), and bilateral oculomotor nerve palsy resulting in loss of voluntary eye movements [2].
Clinical features specific to the ischemic involvement of the different regions of the brainstem are:
Midbrain: ipsilateral oculomotor (3rd cranial nerve) paralysis with contralateral hemiplegia (Weber syndrome)
Pons: 5th, 6th, 7th and 8th cranial nerve involvement, diplopia, dysarthria, vertigo, gait abnormality, and/or sensation of ear canal block, sustained horizontal nystagmus, conjugate eye movements when the gaze is directed towards the side of hemiparesis [6], ataxic hemiparesis.
Medulla: 9th, 10th, 11th and 12th cranial nerve involvement, pure motor hemiparesis if the medullary pyramid is the site of the infarction; sensory motor hemiplegia if the medial lemniscus is involved; or Dejerine syndrome wherein there is ipsilateral tongue paralysis with contralateral hemiplegia. A patient presenting with acute onset vertigo and then developing sudden respiratory failure is likely to have a medullary infarct.
Respiratoric
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Periodic Breathing
Oku Y, Okada M (2008) Periodic breathing and dysphagia associated with a localized lateral medullary infarction. Respirology (Carlton, Vic.) 13: 608–610. View Article Google Scholar 57. [journals.plos.org]
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Respiratory Abnormalities
Abstract The distribution of tyrosine hydroxylase (TH) and substance P (SP) was examined in the brain-stem of 4 infants with respiratory abnormalities associated with remote brain-stem or cerebellar infarction utilizing immunohistochemical methods. [ncbi.nlm.nih.gov]
Jaw & Teeth
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Bruxism
A few uncommon clinical features of brain stem infarction include "salt and pepper" facial pain, blepharospasm, palatal myoclonus, hiccough, tinnitus in medullary infarction, bruxism and trismus in basilar artery occlusion, and peduncular hallucinosis [symptoma.com]
Eyes
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Miosis
Neurological examinations showed left ptosis and miosis, hoarseness, dysphagia, right mild hemiparesis, left ataxia of his extremities, and truncal ataxia. He also had hypalgesia/hypotemperature on his right side. [hindawi.com]
It is typified by vertigo, ipsilateral hemiataxia, dysarthria, ptosis and miosis. Most patients with this stroke recover very well and often resume their previous activities (Nelles et al, 1998). [tchain.com]
[…] the distribution of the 5th cranial nerve (this nucleus is a long vertical structure that extends in the lateral aspect of the pons down into the medulla) Sympathetic pathway : ipsilateral Horner’s syndrome, that is partial ptosis and a small pupil (miosis [lifeinthefastlane.com]
Ipsilateral clinical features include the following: Ataxia and dysmetria, due to damage to the inferior cerebellar peduncle and cerebellum Horner syndrome (eg, ptosis, miosis, hypohidrosis or anhidrosis, enophthalmos), due to damage to descending sympathetic [emedicine.medscape.com]
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Ciliary Injection
Slit-lamp examination showed ciliary injection and keratic precipitates. Fundus examination demonstrated vasculitis in both eyes (Figures 1(a) and 1(b)). Physical examination showed exudative erythema nodosum on his trunk and both thighs. [hindawi.com]
Neurologic
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Dizziness
Two days after discharge from the hospital he was admitted to the neurological department in the university hospital with dizziness, hickups, reduced sensitivity of the right side of face and trunk. [relis.no]
Two days after his first visit, he reported dizziness, speech disturbance, clumsiness of his right extremities, and gait disturbances. [hindawi.com]
Dizziness. You may feel too dizzy to stand up. Weakness or numbness in your arm, leg, or face. This may happen on only one side of your body. Confusion, or trouble speaking. Unable to see out of one or both of your eyes. Copyright 2011. [drugs.com]
A person may have vertigo, dizziness and severe imbalance without the hallmark of most strokes — weakness on one side of the body. The symptoms of vertigo dizziness or imbalance usually occur together; dizziness alone is not a sign of stroke. [stroke.org]
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Nystagmus
[…] oculomotor (3rd cranial nerve) paralysis with contralateral hemiplegia (Weber syndrome) Pons: 5th, 6th, 7th and 8th cranial nerve involvement, diplopia, dysarthria, vertigo, gait abnormality, and/or sensation of ear canal block, sustained horizontal nystagmus [symptoma.com]
In the author's experience, these patients often exhibit rebound nystagmus, which is a variant of gaze-evoked nystagmus. Some of these patients have upbeating nystagmus supine, often confused with BPPV. [dizziness-and-balance.com]
Acute onset in an unknown diabetic associated with brain-stem infarction and death. ( 4212098 ) Siegel A.J....Bell W.R. 1974 43 See-saw nystagmus: an unusual sign of brain-stem infarction. ( 4847304 ) Mastaglia F.L. 1974 44 Sleep patterns in a patient [malacards.org]
In the author's experience, these patients often exhibit rebound nystagmus, which is a variant of gaze-evoked nystagmus. [tchain.com]
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Cranial Nerve Involvement
Medulla: 9th, 10th, 11th and 12th cranial nerve involvement, pure motor hemiparesis if the medullary pyramid is the site of the infarction; sensory motor hemiplegia if the medial lemniscus is involved; or Dejerine syndrome wherein there is ipsilateral [symptoma.com]
Clinically, in localizing strokes to the brainstem one looks for the "cardinal" feature of an ipsilateral peripheral cranial nerve involvement, and a contralateral weakness or sensory deficit. Cerebellar signs, if present, should be ipsilateral. [tchain.com]
Contra pain and temp Vascular - Pica or vertebral Symptoms of brainstem stroke Clinically, in localizing strokes to the brainstem one looks for the "cardinal" feature of an ipsilateral peripheral cranial nerve involvement, and a contralateral weakness [dizziness-and-balance.com]
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Stupor
Postoperative stupor and coma. Mayo Clin Proc. 2005 Mar;80(3):350-4. [clinicclinic2.cafe24.com]
However, this trial did not include patients in stupor or coma, and that criterion probably excluded patients who suffered a basilar artery occlusion. Moreover, the trial did not study the vascular anatomy systematically in all patients. [emedicine.medscape.com]
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Altered Mental Status
Patients with bilateral VAI usually presented with more severe symptoms, including an altered mental status, pinpoint pupils, and even sudden respiratory arrest [6, 12, 16, 17]. Occasionally, bilateral VAI patients remain asymptomatic [1, 2, 18]. [pssjournal.biomedcentral.com]
Workup
The aim of the workup is to determine the characteristics of the vascular lesion and the etiology of the brain stem infarction. A detailed history during workup provides clues to the risk factors and etiology of the condition while a thorough neurological, cardiovascular, and bedside glucose testing are mandatory to determine further workup and management.
Computed tomography scanning (CT) is performed first to differentiate between an ischemic infarct and hemorrhagic stroke as it has 95% sensitivity in identifying hemorrhage especially if done within 24 hours of onset of symptoms [9] [10]. Occluded and dolichoectatic arteries can be identified better with spiral CT angiography [11] [12]. Magnetic resonance imaging (MRI) has greater sensitivity than CT in the identification of ischemia with small lacunar infarcts being seen only on MRI. Diffusion-weighted MRI is highly sensitive for early ischemia and should be ordered after the CT scan. MRI and magnetic resonance angiography (MRA) are useful in detecting dissection, demyelinating plaques, vertebrobasilar dolichoectasia, and dissection. [13] [14] [15].
In addition, other tests are indicated based on the clinical evaluation to detect the etiology and risk factors of the brainstem infarct:
Cardiac factors: electrocardiography (in all cases), telemetry or holter monitoring, serum troponin (in cases suspected to have myocardial ischemia), and echocardiography [16] especially in patients under 45 years of age or in those with basilar artery occlusion.
Vascular factors: blood tests to rule out thrombotic disorders (homocysteine levels, antiphospholipid antibodies, protein S, protein C, antithrombin III, factor V Leiden) and routine tests like complete blood count, platelet count, PT/PTT, fasting blood glucose, lipid profile, and serology for syphilis.
Autoimmune factors: antinuclear antibodies, rheumatoid factor, erythrocyte sedimentation rate.
Urine toxicology and drug screening for stimulants like cocaine and amphetamines.
Microbiology
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Treponema Pallidum
Studies for young stroke showed positive rapid plasma reagin (RPR) (1:32), positive treponema pallidum haemagglutination ( 1:1280), positive anti-HIV antibody and confirmed with western blot. [academic.oup.com]
Treatment
We fully realize that early and appropriate treatment are essential to improve the treatment results, and constructing a medical system with a team of orthopedists, radiologists, and neurosurgeons is also very important. [ncbi.nlm.nih.gov]
Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. [drugs.com]
1 Endoscopic surgical treatment of Cushing's disease: A single-center experience of cauterization of peritumoral tissues. 61 31777545 2019 2 [Catheter balloon dilation combined with acupuncture for cricopharyngeal achalasia after brain stem infarction [malacards.org]
Prognosis
Preceding TIAs, blood pressure level, serum cholesterol and triglyceride values, and aortic arch angiogram findings, on the other hand, had no effect upon the prognosis. The effects of body build on prognosis remained obscure. [ncbi.nlm.nih.gov]
The majority of patients had a benign prognosis. [journals.sagepub.com]
Results Overall, 14 (45.2%) patients had a good prognosis, 13 (41.9%) patients had a poor prognosis, and four (12.9%) patients died. Dysphagia, vomiting, cranial neuropathy, and high NIHSS score on admission were associated with a poor outcome. [sjamf.eg.net]
[…] they carry a poor prognosis. 2 Reply [dailyrounds.org]
The functional prognosis of these patients is excellent, although the INO tends to last longer when there are other neurologic deficits. Received October 6, 2003. Accepted in final form January 28, 2004. [neurology.org]
Etiology
The aim of the workup is to determine the characteristics of the vascular lesion and the etiology of the brain stem infarction. [symptoma.com]
Moyamoya disease (MMD) is a rare cerebrovascular disease with an unknown etiology and is characterized by intrinsic fragility in the intracranial vascular walls such as the affected internal elastic lamina and thinning medial layer. [ncbi.nlm.nih.gov]
Our results suggest that a single brainstem lesion constitutes a syndrome of multiple etiologies, and that these etiologies and prognoses can differ widely. [journals.sagepub.com]
• 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]
Epidemiology
Epidemiology 1998; 9(6): 596-600. [relis.no]
[Epidemiology of stroke in the elderly : “the silver tsunami”]. Rev Prat, 62(9), 1221-1224. Kim, J. S., & Lee, H. (2009). Inner Ear Dysfunction Due to Vertebrobasilar Ischemic Stroke. [Article]. [hearinghealthmatters.org]
The investigators found the greatest risk of subsequent stroke to be in those individuals with a combination of low blood flow and blood pressure below 140/90 mm Hg. [6] Epidemiology of Vertebrobasilar Stroke The frequency, incidence, and prevalence of [emedicine.medscape.com]
However, epidemiological data have shown a high prevalence of central sleep apnea in patient groups with heart involvement. Depending on the NYHA classification and the diagnostic methods, it varies around 40 to 60% [59] or even more [60]. [journals.plos.org]
Pathophysiology
The pathophysiological background and potential therapeutic strategies are discussed. [ncbi.nlm.nih.gov]
Cigolini M Cappellari F Hypertensive brain stem encephalopathy: clinically silent massive edema of the pons Neurol Sci 2001 22 317 320 6 Fugate JE Rabinstein AA Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology [jkna.org]
The pathophysiology of otologic facial paralysis. Otolaryngol Clin North Am1974;7:309–30. Launay M, Fredy D, Merland JJ, et al. Narrowing and occlusion of arteries by intracranial tumors. Review of the literature and report of 25 cases. [jnnp.bmj.com]
In Barnet HJM (and others, Eds), Stroke: Pathophysiology, Diagnosis and Management. New York: Chrchill-Livingstone, pp 549-619, 1986 Dai, A. I. and M. Wasay (2006). "Wernekink comissure syndrome: a rare midbrain syndrome secondary to stroke." [dizziness-and-balance.com]
Prevention
Even if laryngeal penetration and pulmonary aspiration are observed, chest infection could be prevented by swallowing exercise combined with voluntary cough. [ncbi.nlm.nih.gov]
Ask your caregiver for more information about preventing pressure sores. Things that you can do to help prevent a brainstem infarction: Do not smoke. Do not smoke tobacco products, or use illegal (street) drugs. [drugs.com]
References
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- Sinha KK. Brain Stem Infarction: Clinical clues to localise them. Journal, Indian Academy of Clinical Medicine. 2000 Oct-Dec; 1:3: 214 -221
- Caplan L, Gorelick P. “Salt and pepper on the face” pain in acute brain stem ischaemia. Ann Neurol 1983; 13:344-5.
- Jankovic J, Patel SC. Blepharospasm associated with brain stem lesions. Neurology.1983; 33: 1237-40.
- Sacco RL, Freddo L, Bello JA, et al. Wallenberg’s lateral medullary syndrome. Clinical, magnetic resonance imaging correlations. Arch Neurol 1993; 50: 609-14.
- Fisher CM, Tapia J. Lateral medullary infarction extending to the lower pons. J Neurol Neurosurg Psychiat. 1987; 50: 620-4.
- Patterson JR, Grabosis M. Locked in syndrome. A review of 139 cases. Stroke 1986; 17: 758-63.
- Mekee AC, Levin DN, Kowall NW, et al. Peduncular hallucinosis associated with isolated infarction of the substantia nigra reticularis. Ann Neurol. 1990; 27: 500 -04
- Bahouth MN, LaMonte MP. Acute ischemic stroke: evaluation and management strategies. Top Adv Pract Nurs. 2005; 5(4)
- Kim D, Liebeskind DS. Neuroimaging advances and the transformation of acute stroke care. Semin Neurol. 2005 Dec.; 25(4):345-61.
- Puetz V, Sylaja PN, Coutts SB, et al. Extent of hypoattenuation on CT angiography source images predicts functional outcome in patients with basilar artery occlusion. Stroke. 2008 Sep.; 39(9):2485-90.
- Sylaja PN, Puetz V, Dzialowski I, et al. Prognostic value of CT angiography in patients with suspected vertebrobasilar ischemia. J Neuroimaging. 2008 Jan.; 18(1):46-9.
- Aichner FT, Felber SR, Birbamer GG. Magnetic resonance imaging and magnetic resonance angiography of vertebrobasilar dolichoectasia. Cerebrovasc Dis. 1993; 3:280-4.
- Kitanaka C, Tanaka J, Kuwahara M, et al. Magnetic resonance imaging study of intracranial vertebrobasilar artery dissections. Stroke. 1994 Mar; 25(3):571-5.
- Knepper L, Biller J, Adams HP Jr, et al. MR imaging of basilar artery occlusion. J Comput Assist Tomogr. 1990 Jan-Feb.; 14(1):32-5.
- Rem JA, Hachinski VC, Boughner DR, et al. Value of cardiac monitoring and echocardiography in TIA and stroke patients. Stroke. 1985 Nov-Dec.; 16(6):950-6