In addition, wallenberg syndrome accounts for at least 2. Case a 58 yo was referred to you because of recent onset of left hemiparesis, leftsided loss of propioception and rightsided tongue deviation. Number of baseline lesions has been shown to predict future attacks and disability in clinically isolated syndromes cis. Keibel f, mall fp eds manual of human embryology, vol 2. Radiological findings in brainstem infarction syndromes. The most rostral part of the brainstem is the midbrain. It is characterized by four bumps, the paired superior and inferior colliculi on its dorsal posterior surface, and by the large cerebral peduncles containing the descending corticospinal and corticopontine tracts on its ventral anterior surface. Clinical presentation clinical picture is characteristic and includes ipsilateral oculomotor nerve palsy and contra. Development and developmental disorders of the brain stem. If you establish where the meridians of longitude and parallels of latitude intersect then you have established the site of the lesion. This video is part of the course introduction to neurology. Copinion vertigo in brainstem and cerebellar strokes. Central tegmental tract palatal myoclonus accompanied by rhythmic movements of pharynx, larynx, face, eyes, or respiratory muscles.
The brainstem and cerebellum were removed by sectioning at the rostral midbrain. Any attempt to over simplify things runs the risk of. These physicians practiced in an era when disorders such as tuberculoma, syphilitic gumma, and tumor were seen much more often than today. If there are signs of both a lateral and a medial paramedian brainstem syndrome, then one needs to consider a basilar artery problem, possibly an occlusion. Intra brainstem connectivity is impaired in chronic fatigue syndrome. Epidemiology although many different brainstem stroke syndromes have. Aica involves ipsilateral facial weakness and hearing loss. Lacunar syndromes due to brainstem involvement include pure motor stroke, dysarthriaclumsy hand syndrome, and ataxic hemiparesis homolateral ataxia and crural paresis.
Upgaze paresis dorsal midbrain or parinaud syndrome another brainstem syndrome that often occurs without symptoms is the dorsal midbrain syndrome. Brainstem syndromes are most commonly due to occlusion of the posterior circulation or mass effect from intrinsic spaceoccupying lesions. It is characterized by four bumps, the paired superior and inferior colliculi on its dorsal posterior surface, and by the large cerebral peduncles containing the descending corticospinal and corticopontine tracts on. The presentation of a patient with brainstem symptoms and signs invokes a number of common and less common differential diagnoses, and accurate diagnosis can be challenging. There is a lot of overlap between brainstem syndromes, presented here are the main characteristic. Brain stem syndrome can result in abnormal functioning of cranial nerves which may lead to visual disturbances, pupil abnormalities, changes in sensation, muscle weakness, hearing problems, vertigo, swallowing and speech difficulty, voice change, and coordination problems. Gates the geelong hospital, barwon health, geelong, victoria, australia the rule of 4 is a simple method developed to help students of neurology to remember the anatomy of the brainstem and thus the features of the various brainstem vascular syndromes. There are two syndromes that combine an mlf lesion and contralateral.
Brainstem rules of 4 litfl neurology clinical exmaination. Choose from 500 different sets of brainstem syndromes spinal flashcards on quizlet. In contrast to the generally poor outcome from brainstem infarction syndromes, with prompt diagnosis and optimal management, wallenberg syndrome has a favourable prognosis. The two deformations were then merged and applied to the 1100 epi images. Paraneoplastic disorders can be divided into four groups, roughly in order of their recognition. Brainstem lesions in clinically isolated syndromes neurology. Parinauds syndrome 1883, paralysis of conjugate vertical gaze and paresis of convergence together with additional neuroophthalmological signs and the koerber salus elschnig. Ill let you mull over these rules until the next brainstem post, where youll be able to test drive gates brainstem rules of 4. The brainstem is a very small component of the brain, making up only around 2. Kahn, mdb, introduction to stroke acute stroke care is acornerstone of emergency medicine practice owing to its significantmorbidityandmortality. Upper motor neuron damage can lead to symptoms, ranging from hemiparesis to the lockedin syndrome, which is typically characterized by. The second step will be recognising brain stem infarction syndromes in single brain stem vascular territories and the third step will be identifying the difficult type multiple level brain. These and other related syndromes were instrumental in establishing important principles in brainstem localization.
Mlf internuclear ophthalmoplegia failure of adduction in horizontal gaze but preservation of convergence. Brainstem stroke syndromes, also known as crossed brainstem syndromes, refer to a group of syndromes that occur secondary to lesions, most commonly infarcts, of the brainstem. The spectrum of immunemediated and inflammatory lesions. Wallenberg syndrome, for identification of vertebral artery dissection. Occasionally lateral brainstem syndromes are seen in unilateral vertebral occlusion. Waardenburg pj 1951 a new syndrome combining develop. The reticular formation of the brainstem has a role in arousal, sleep, attention, alerting, sensory gating, and habituation brown et al. However, since these two syndromes are accompanied by both ocular and neurological signs which indicate typical lesions of the brain stem, they are also mentioned here. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for brain stem cancer skip to main content u. Human hoxa1 syndromes are a group of very rare disorders with complex neurological and systemic symptoms. Brain stem cancer genetic and rare diseases information. A brainstem stroke syndrome falls under the broader category of stroke syndromes, or specific symptoms caused by vascular injury to an area of brain for example, the lacunar syndromes. Brainstem rather than cerebellar lesions may be responsible for poor prognosis. Explain the symptoms with regards to structures affected.
Claude syndrome is one of the brainstem stroke syndromes in which there is infarction of the dorsomedial aspect of the midbrain. These syndromes can have subtle imaging findings that may be missed by a radiologist unfamiliar with the anatomy or typical manifesting features. The red circle indicates that the paramedian pontine arteries are affected in this syndrome. A clue to that component is found on the opening line of the paper. The descriptions of brain stem disorders are consistently structured and are supported by many mr images and short case reports. Classical brainstem syndromes many of the early neurologic pioneer clinicians described the clinical findings due to a focal process affecting the brainstem. Foville syndrome is a rare inferior medial pontine syndrome first. The rule of 4 is a simplified method to localize brainstem vascular syndromes specific to a vascular territory. Brainstem dysfunction in critically ill patients critical care full text.
These syndromes were first described among a few american indian tribes such as the navajo and apaches, who are related to athabaskan indians of northern canada. Pdf work out where the problem is in the brainstem using the rule. Brainstem syndromes typically cause ipsilateral cranial nerve lesions and contralateral long tract signs. When symptoms do occur, the patient has difficulty looking up and may have blurry distant vision caused by accommodative spasm. The syndromes of walleyed monocular internuclear ophthalmoplegia and. The signs of a right paramedian brainstem syndrome reproduced from gates 4. As the brainstem contains numerous cranial nuclei and white matter tracts, a.
Case historya 78 yr old woman presenting with brainstem signs following a seizure. In addition to primary brainstem lesions, such as vascular brainstem syndromes, other disorders are considered that do not exclusively show brainstem symptoms or signs, e. Brainstem disorders 2011th edition pdf download medical. Pontine disorders comprise a broad spectrum of clinical conditions ranging from monosymptomatic presentations to complex phenotypes. Keibel f, mall fp eds manual of human embryology,vol 2. Brainstem stroke syndromes radiology reference article. This presentation was created without any commercial support. Brainstem and multiple cranial nerve syndromes neupsy key. This paper describes a simple technique to aid in the understanding of brainstem vascular syndromes. If you continue browsing the site, you agree to the use of cookies on this website. Differential diagnosis and management of brainstem and cerebellar infarctions combined sections meeting, february 7, 2015.
In a study of 37 patients with acute infarcts mainly involving the base of the pons, pure motor hemiparesis was present in 17, sensorimotor stroke in 3, ataxic hemiparesis in 4. Thus a medial brainstem syndrome will consist of the 4 ms and the relevant motor cranial nerve, and a lateral brainstem syndrome will consist of the 4 ss and either the 911th cranial nerve if in the medulla, or the 5th, 7th and 8th cranial nerve if in the pons. After the formation of the basilar artery via the merging of vertebral arteries. Parinauds syndrome 1883, paralysis of conjugate vertical gaze and paresis of convergence together with additional neuroophthalmological signs and the koerber salus elschnigs syndrome 1903, 1910, 19, nystagmus. Materials and methods the brains of three patients who died without known neurologic disease were obtained at autopsy and fixed in 1 0% buffered formaldehyde. Antihuassociated brainstem encephalitis journal of. The rule of 4 is a simple method developed to help students of neurology to remember the anatomy of the brainstem and thus the features of the various brainstem vascular syndromes. Learn brainstem syndromes spinal with free interactive flashcards. They are commonly due to brainstem ischaemia, but can also be caused by neoplasia, demyelination, infective and hamartomatous lesions. Brainstem stroke syndromes there are many brainstem stroke syndromes some of the clinical features seen are. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Pdf on feb 16, 2010, robin a hurley and others published the brainstem. This article will provide an overview of the causes of the various brainstem vascular. Imaging, ideally with mri rather than ct, is obligatory and only then.