Brainstem Syndromes

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Chapter 19 Brainstem Syndromes

Other chapters in this book that deal with symptoms emphasize history as the starting point for generating possibilities for the differential diagnosis. This list of diagnostic considerations is then refined during the examination. This chapter calls for a different approach. When the neurologist evaluates a patient with a brainstem disorder, often the most effective method of diagnosis is to organize the differential diagnosis around the objective physical findings, particularly in patients with an altered mental status such as coma. The symptoms are still integrated in the approach, but the physical findings take center stage.

Organization around physical findings is efficient because very specific neurological localization, which limits the diagnostic alternatives, often is possible. The long tracts of the nervous system traverse the entire brainstem in the longitudinal (rostrocaudal) plane. Cranial nerve nuclei and their respective cranial nerves originate and exit at distinct levels of the brainstem. This arrangement allows for exquisite localization of function based on the findings of the neurological examination.

The chapter begins with a discussion of the brainstem ocular motor syndromes, followed by descriptions of miscellaneous brainstem, brainstem stroke, diencephalic, and thalamic syndromes.

Ocular Motor Syndromes

Combined Vertical Gaze Ophthalmoplegia

Combined vertical gaze ophthalmoplegia is defined as paresis of both upward and downward gaze. Vertical gaze ophthalmoplegia is an example of a brainstem syndrome in which the objective physical findings dictate the diagnostic approach to the problem. Symptoms of vertical gaze ophthalmoplegia, when present, are relatively nonspecific and usually occur in patients who have difficulty looking down, as required in reading, eating from a table, and walking down a flight of stairs. In addition, the patient’s report of symptoms may be unobtainable because of mental status changes caused by dysfunction of the reticular formation that lies adjacent to the vertical gaze generator in the rostral midbrain (see Chapter 35).

The neurological examination discloses associated signs of the disorders listed in the differential diagnosis (see Box 19.1) (Graff-Redford et al., 1985). Coma may be associated with reticular system involvement. Long-tract signs and loss of pupillary reflexes are commonly associated. The syndrome of combined vertical gaze ophthalmoplegia is diagnosed when the ocular findings occur in isolation from long-tract signs.

With combined vertical gaze ophthalmoplegia, vertical saccades and pursuit are lost. This gaze limitation may be overcome by the oculocephalic (doll’s head or doll’s eye) maneuver, which tests the vestibulo-ocular reflex (VOR) (see Chapter 35). It is demonstrated by having the patient focus on an object and rotating the patient’s head; a conjugate eye movement in the opposite direction is the expected response with this maneuver. The Bell phenomenon (reflex movement of the eyes up and out in response to forced eye closure) often is absent. Skew deviation (vertical malalignment of the eyes) may occur. Absence of convergence and loss of pupillary reactions to light are common.

The location of the lesion of combined vertical gaze ophthalmoplegia is the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) for loss of vertical pursuit and saccades (Leigh and Zee, 2006). Box 19.1 lists the disorders involving the rostral mesodiencephalic region (for the differential diagnosis) that cause combined vertical gaze ophthalmoplegia (see Chapter 35). The most common causes of isolated combined vertical gaze ophthalmoplegia are stroke and progressive supranuclear palsy (PSP). In cortical-basal ganglionic degeneration, ocular motility findings are similar to those in PSP but are less severe. Whereas the supranuclear vertical gaze ophthalmoplegia may be prominent early in the course of PSP, obvious vertical and horizontal gaze restriction usually is a late finding in cortical-basal ganglionic degeneration (Rottach et al., 1996).

The diagnostic formulation varies with the age of the patient. Isolated combined vertical gaze ophthalmoplegia usually is due to infarction of the rostral dorsal midbrain. When onset is gradual instead of abrupt or if the patient is young, other disorders should be considered (see Box 19.1). In the elderly, PSP (see Chapter 66) is likely if the onset is gradual. PSP can be mimicked by the treatable Whipple disease (Averbuch-Heller et al., 1999). For Whipple disease, the movement disorder, oculomasticatory myorhythmia, is pathognomonic. Laboratory investigations used to evaluate combined vertical gaze ophthalmoplegia include computed tomography (CT) scan or, preferably, magnetic resonance imaging (MRI). Care should be taken not to overlook lesions inferior to the floor of the third ventricle. Lumbar puncture (LP), syphilis serology, erythrocyte sedimentation rate, and an antinuclear antibody test complete the evaluation when the cause is not obvious. Small-bowel biopsy should be considered if Whipple disease is a possible diagnosis. A polymerase chain reaction (PCR) assay of small-bowel biopsy specimen, cerebrospinal fluid (CSF), or other tissues for the 16S ribosomal ribonucleic acid (RNA) gene of Tropheryma whippelii appears to have both sensitivity and specificity for the diagnosis of Whipple disease (Lee, 2002).

Upgaze Paresis (Dorsal Midbrain or Parinaud Syndrome)

Another brainstem syndrome that often occurs without symptoms is the dorsal midbrain syndrome. When symptoms do occur, the patient has difficulty looking up and may have blurry distant vision caused by accommodative spasm.

The tetrad of findings in the dorsal midbrain syndrome are (1) loss of upgaze, which usually is supranuclear (loss of pursuit and saccades with preservation of the VOR); (2) normal to large pupils with light-near dissociation (loss of the light reaction with preservation of pupilloconstriction in response to a near target) or pupillary areflexia; (3) convergence-retraction nystagmus, in which the eyes make convergent and retracting oscillations after an upward saccade; and (4) lid retraction.

The location of the lesion causing the upgaze paresis of the dorsal midbrain syndrome is the posterior commissure and its interstitial nucleus (Leigh and Zee, 2006). The presence of the full syndrome implies a lesion of the dorsal midbrain (including the posterior commissure), a bilateral lesion of the pretectal region, or a large unilateral tegmental lesion.

The differential diagnosis is presented in Box 19.2. Other than the mild upgaze limitation that occurs with age, the most common cause of loss of upgaze is a tumor of the pineal region. The next most common causes are stroke and trauma. The upgaze palsy portion of the syndrome can be mimicked by any of several conditions: double elevator palsy, PSP, orbital causes such as thyroid ophthalmopathy and the bilateral Brown superior oblique tendon sheath syndrome, pseudo–dorsal midbrain syndrome secondary to myasthenia gravis (MG) or Guillain-Barré syndrome, and congenital upgaze limitation. Forced ductions (see Chapter 16) may be performed by grasping anesthetized sclera with forceps and moving the globe through its range of motion. The presence of restriction of movement with forced ductions implies a lesion within the orbit, as distinct from a midbrain lesion.

The diagnostic formulation for the dorsal midbrain syndrome varies with age. In children and adolescents, pineal region tumors usually are the cause. In young and middle-aged adults, the disorder is uncommon, and the cause may be trauma, multiple sclerosis (MS), or arteriovenous malformation (AVM). In the elderly, stroke and PSP are the most common causes.

The laboratory investigation needed to evaluate dorsal midbrain syndrome is MRI. If no tumor is present and an infectious or inflammatory cause is suspected, an LP should be performed.

Internuclear Ophthalmoplegia

Internuclear ophthalmoplegia (INO) is characterized by paresis of adduction of one eye, with horizontal nystagmus in the contralateral eye when it is abducted. It is due to a lesion of the MLF ipsilateral to the side of the adduction weakness.

Surprisingly, most patients with INO have no symptoms. The symptoms that may be associated with INO are diplopia, oscillopsia of one of the two images, and blurred vision. When diplopia is present, it is due to medial rectus paresis (horizontal diplopia) or skew deviation (vertical diplopia).

The MLF carries information for vertical pursuit and the vertical VOR. Consequently, other associated findings with MLF lesions are abnormal vertical smooth pursuit and impaired reflex vertical eye movements (doll’s eye maneuver, Bell phenomenon). Voluntary vertical eye movements (pursuit and saccades) are unaffected. Gaze-evoked vertical nystagmus (usually on upgaze) and skew deviation may be present with the higher eye usually present on the side of the lesion. Skew deviation is a pure vertical ocular deviation that is not due to a cranial nerve palsy, orbital lesion, or strabismus but is caused by disturbed supranuclear input to the third and fourth cranial nerve nuclei. It is thought to be due to unilateral damage to the otolith-ocular pathways or the pathways mediating the VOR (Zwergal et al., 2008).

Internuclear ophthalmoplegia, discussed further in Chapter 35, may occur as a false localizing sign. Brainstem compression due to subdural hematoma with transtentorial herniation and cerebellar masses may cause INO. Myasthenia gravis and Guillain-Barré syndrome also may simulate INO.

The diagnostic considerations are many and varied. Examination can differentiate a lesion of the MLF from a partial third cranial nerve palsy, MG, strabismus, or thyroid ophthalmopathy. The common causes of INO are stroke (including vertebral artery dissection) in older age groups and MS in the young. Keane’s series (Keane, 2005) from a large inner-city hospital reveals that approximately one third of INO cases are due to stroke, one third to MS, and one third to other causes. The less common causes include trauma, herniation, infections, tumor, vasculitis, and surgical procedures.

Laboratory investigations that are performed to elucidate the cause include MRI. Thin cuts are often needed to find the lesion when INO is isolated. An edrophonium (Tensilon) test should be performed to evaluate for MG unless there are associated signs of obligatory brainstem dysfunction.

The diagnostic formulation for INO first necessitates accurate localization of the lesion. Limitation of adduction initially is formulated simply as an adduction deficit. It may be due to (1) a lesion of the midbrain or third cranial nerve disrupting innervation, (2) a disorder of the neuromuscular junction (MG), or (3) a lesion directly involving the medial rectus muscle.

Horizontal Gaze Paresis

Although there are no common symptoms of horizontal gaze paresis, this condition seldom occurs in isolation. Patients may complain of inability to see or to look to the side. Because supranuclear gaze pareses are conjugate by definition, diplopia does not occur.

On examination, with unilateral isolated involvement of the paramedian pontine reticular formation (PPRF), loss of ipsilateral saccades and pursuit is evident. However, full horizontal eye movements are demonstrated with the oculocephalic maneuver.

Lesions of the sixth cranial nerve nucleus cause horizontal gaze paresis with inability of the oculocephalic maneuver to overcome the gaze limitation. Although an associated ipsilateral peripheral facial palsy is usually associated from involvement of the fascicle of the seventh cranial nerve coursing over the sixth cranial nerve nucleus, cases of isolated horizontal gaze paresis caused by sixth nerve nuclear lesions have been reported (Miller et al., 2002). With bilateral lesions, loss or limitation of horizontal saccades and (usually) pursuit in both directions is characteristic. Gaze-paretic nystagmus may be present. In the acute phase, transient vertical gaze paresis and vertical nystagmus or upgaze paresis can occur. In the chronic phase, vertical eye movements are full, although nystagmus may be noted on upgaze.

The location of the lesion for horizontal gaze paresis is the frontopontine tract, mesencephalic reticular formation, PPRF, and sixth cranial nerve nucleus. The explanation of gaze palsy occurring with a nuclear lesion is given later in the chapter (see Syndromes Involving Ocular Motor Nuclei).

The diagnostic possibilities are varied. As with other ocular motility disorders, MG may cause gaze limitation that simulates a central nervous system (CNS) lesion. The diagnostic formulation varies with age, rapidity of onset, and associated clinical findings. For patients with an acute onset who are older than 50 years of age, cerebrovascular disease, ischemic or hemorrhagic, is a likely cause. With a subacute onset before the age of 50 years, a diagnosis of MS should be considered. Congenital cases usually are due to Möbius syndrome. Systemic lupus erythematosus (SLE), syphilis, and Wernicke encephalopathy should be considered for any acquired cases.

Laboratory investigations for horizontal gaze paresis should include MRI. If there are no obligatory signs of CNS dysfunction, MG has to be considered.

Global Paralysis of Gaze

The characteristic symptom of global paralysis of gaze is an inability to look voluntarily (saccades and pursuit) in any direction. Global paralysis of gaze rarely occurs in isolation, however, and signs and symptoms of involvement of other local structures usually are present.

The location of the lesion is the frontopontine tract for saccades, and the parieto-occipitopontine tract for pursuit, where they converge at the subthalamic and upper midbrain level (Thurtell and Halmagyi, 2008). The differential diagnosis for total ophthalmoplegia is given in Box 19.3. The common causes for this presentation are diseases outside the CNS, such as Guillain-Barré syndrome, MG, and chronic progressive external ophthalmoplegia (CPEO); for intraaxial lesions, considerations include stroke, Wernicke encephalopathy, and PSP.

The diagnostic formulation usually is concerned with extraaxial (cranial nerve, neuromuscular junction, or muscle) pathology, because isolated complete ophthalmoplegia is rarely caused by a brainstem lesion. Myasthenia gravis (sometimes in combination with thyroid ophthalmopathy), bilateral cavernous sinus metastases (Ebert et al., 2009), and Guillain-Barré syndrome are more likely possibilities if the onset is subacute. If the presentation is long-standing, slowly progressive, and accompanied by eyelid ptosis, the CPEO syndromes, such as Kearns-Sayre syndrome, should be considered. In these extraaxial disorders, oculocephalic reflexes do not overcome the gaze limitations. PSP is a diagnostic possibility in the elderly, whereas Wernicke encephalopathy should be considered in alcoholics and nutritionally deprived patients. Whipple disease also can cause this rare clinical presentation.

Laboratory investigations for patients with global paralysis of gaze should include MRI. An edrophonium test is performed when MG is suspected. When botulism is suspected, electromyography with repetitive stimulation and serum assay for botulinum toxin should be performed.

Syndromes Involving Ocular Motor Nuclei

Patients with lesions of the third or sixth cranial nerve nucleus not only present with accompanying long-tract signs but also show different ocular motility disturbances than with lesions of the third or sixth cranial nerve.

Third Cranial Nerve Nucleus

The common manifestations of nuclear third cranial nerve palsies are diplopia and ptosis. The signs present on the side of the lesion are weakness of the inferior and medial recti and the inferior oblique muscles. Upgaze limitation is present in both eyes because the superior rectus subnucleus is contralateral, and the axons cross within the nuclear complex. In addition, ptosis and dilated unreactive pupils may be present on both sides because the levator subnucleus and Edinger-Westphal nuclei are bilaterally represented.

To localize a lesion to the third cranial nerve nucleus, both eyes must have some involvement because of the bilateral representation. The superior rectus and levator of the eyelid, however, are bilaterally represented and thus cannot demonstrate single muscle involvement. In addition, because the medial rectus subnucleus is in the most ventral portion of the nucleus and all of the dorsal subnuclei send axons through it, single muscle involvement of the medial rectus may not be possible. The eyelid levator subnucleus may be spared because it is located at the dorsocaudal periphery of the nuclear complex.

Main considerations in the differential diagnosis are stroke (either ischemic or hemorrhagic), metastatic tumor, and MS. Of these diagnoses, only ischemic stroke is common. Disorders that simulate nuclear third cranial nerve palsy are MG, CPEO, thyroid ophthalmopathy, and Guillain-Barré syndrome.

The pertinent laboratory investigation for this syndrome is MRI, which usually demonstrates the ischemic cerebrovascular lesion. Once the proper localization has been made, the diagnostic formulation is straightforward.

Other Brainstem and Associated Syndromes

Tectal Deafness

The symptoms associated with tectal deafness are bilateral deafness associated with other related CNS symptoms such as poor coordination, weakness, or vertigo. The main considerations in the differential diagnosis for the deafness are conduction-type hearing loss, cochlear disorders, bilateral eighth cranial nerve lesions, tectal deafness, and pure word deafness.

On examination, deafness that usually spares pure tones is confirmed. Pure word deafness with lesions of the inferior colliculi has been reported (Vitte et al., 2002). Other brainstem signs, including the dorsal midbrain syndrome, often are associated. The location of the lesion is the inferior colliculi; the most common causes are trauma, stroke, or a tumor of the brainstem, cerebellum, or pineal region. The diagnostic formulation for hearing loss caused by lesions rostral to the cochlear nuclei is the presence of hearing loss characterized by sparing of pure tone, with marked deterioration when background noise distortion or competing messages are added. In addition, signs of damage to adjacent nervous system structures are present. Neuroimaging studies may confirm the diagnosis.

The pertinent laboratory investigations include MRI and an audiogram. Tests that reveal CNS auditory loss are distorted speech audiometry, dichotic auditory testing, and auditory brainstem evoked responses, although findings on the last test may be normal (Vitte et al., 2002).

Foramen Magnum Syndrome

Foramen magnum syndrome is characterized by upper motor neuron–type weakness and sensory loss in any modality below the head. Detecting this syndrome is important because it often is caused by benign tumors such as meningiomas or fibromas, which may be removed completely when detected early in their course. Its only manifestations may be those of a high spinal cord syndrome (see Chapter 24).

The common initial symptoms typically are neck stiffness and pain, which may radiate into the shoulder. Occipital headache also may be an early symptom. Other common symptoms are weakness of the upper or lower extremities, numbness (most commonly of hands or arms), clumsiness, and a gait disturbance.

Considerations in the differential diagnosis at this stage include cervical spondylosis, syringomyelia, MS, transverse myelitis, atlantoaxial subluxation, Chiari malformation, and foramen magnum or upper cervical cord tumor.

On examination, hemiparesis or quadriparesis and sensory loss are common. The loss of sensation may involve all modalities. It may be dissociated and capelike or may occur in a C2 distribution. Some patients have a hemisensory pattern below the cranium or involvement of only the lower extremities.

Pseudoathetosis resulting from loss of joint position sense may be an early sign. Atrophy of muscles of the upper extremities may occur at levels well below the lesion (e.g., intrinsic muscles of the hands). Electric shock–like sensations radiating down the spine, which may be transmitted into the extremities, may occur with neck flexion (Lhermitte sign). This phenomenon may occur with lesions of the posterior columns, most commonly MS. Lower cranial nerve palsies are less common. The presence of downbeat nystagmus in primary position or lateral gaze strongly suggests a lesion of the craniocervical junction. This sign may be missed unless the eyelids are manually elevated and the nystagmus is sought when the patient gazes laterally and slightly downward.

The differential diagnosis at this stage is focused on a foramen magnum or upper cervical cord tumor. The tumor type usually is meningioma, neurofibroma, glioma, or metastasis. Cervical spondylosis, MS, syringobulbia, and the Chiari malformation (often accompanied by a syrinx) are other diagnostic considerations. The definitive laboratory investigation for evaluation of the foramen magnum syndrome is MRI.

Patients with foramen magnum tumors may have a relapsing-remitting clinical course with features that simulate those of MS. Because many of these tumors are meningiomas, the clinician should be alert for patients at risk. Meningiomas occur with increased frequency in women in their childbearing years and increase in size during pregnancy. Cervical spondylosis usually is associated with a related radiculopathy and is not accompanied by downbeat nystagmus or lower cranial nerve abnormalities. Diagnosis requires a high index of suspicion early in the patient’s course. Foramen magnum tumors are known to present difficult diagnostic problems because signs may be minimal despite a large tumor.

Syringobulbia

Syringobulbia is a disorder of the lower brainstem caused by progressive enlargement of a fluid-filled cavity that involves the medulla and almost invariably the spinal cord (syringomyelia). The symptoms and signs are primarily those of a disorder of the central spinal cord region (see Syringomyelia in Chapter 73).

The common symptoms of syringobulbia and syringomyelia are lack of pain with accidental burns, hand numbness, neck and arm pain, leg stiffness, and headache, together with oscillopsia, diplopia, or vertigo. On examination, signs of lower brainstem dysfunction are evident. Lower motor neuron signs of the ninth through twelfth cranial nerves may be present. Nystagmus, if present, is horizontal, vertical, or rotatory. Signs of a spinal cord lesion characteristically coexist. In the upper extremities, dissociated anesthesia of an upper limb or forequarter (i.e., loss of pain and temperature sensation with sparing of other modalities) may be noted. The sensory loss also may be in a hemisensory distribution. Absence of or decreased deep tendon reflexes in the upper extremities are the rule.

Spastic paraparesis, usually asymmetrical, may occur. Loss of facial sensation can occur in an onionskin pattern emanating from the corner of the mouth. Charcot (neuropathic) joints and trophic skin disorders may be features in long-standing cases. Horner syndrome and bowel and bladder disturbances are other occasional findings.

The lesion is located in a rostrocaudal longitudinal cavity from the medulla into the spinal cord. The cavity usually is located near the fourth ventricle or central canal of the spinal cord. The definitive laboratory investigation for syringobulbia is MRI, the most reliable and sensitive test to demonstrate a syrinx.

The main considerations in the differential diagnosis are an intrinsic central cord and lower brainstem lesion (syrinx, tumor, or trauma) and compressive foramen magnum syndrome caused by a tumor. Less likely causes are MS and spinal arachnoiditis.

The diagnostic formulation for syringobulbia is based on data from the history, examination, and laboratory evaluation. It usually is a disease of young adults, with a peak incidence in the third and fourth decades of life. Painless burns and dissociated segmental anesthesia of the upper extremities are of major diagnostic significance. A diagnosis of MS requires the presence of other noncontiguous lesions, oligoclonal bands in the CSF, and characteristic MRI findings. Tumors usually produce a more rapid course.

Brainstem Ischemic Stroke Syndromes

Vertebrobasilar ischemic lesions often have a rostrocaudal or patchy localization (Fig. 19.1), rather than the simplified transverse localization that usually is schematized (Kubik and Adams, 1946). In addition, all of the clinical features may not be explainable in anatomical terms; that is, clinicopathological correlation may not be precise.

image

Fig. 19.1 A postmortem examination of a patient with embolism of the basilar artery. Note the rostrocaudal extension of the infarction, along with its patchy nature.

(Reprinted with permission from Kubik, C.S., Adams, R.D., 1946. Occlusion of the basilar artery: a clinical and pathological study. Brain 59, 73-121.)

The cardinal manifestations of brainstem stroke are involvement of the long tracts of the brainstem in combination with cranial nerve deficits. Crossed cranial nerve and motor or sensory long-tract deficits are characteristic. The cranial nerve palsy is ipsilateral to the lesion, and the long-tract signs are contralateral—hence the term crossed. Coma, ataxia, and vertigo, which are common with vertebrobasilar stroke, are uncommon with internal carotid artery circulation stroke. INO, unreactive pupils, lower motor neuron cranial nerve impairment, and ocular skew deviation, when caused by stroke, occur only with posterior circulation lesions. The same is usually true for nystagmus and most other ocular oscillations.

Another characteristic of vertebrobasilar ischemia is bilateral involvement of the long tracts. This can result in locked-in syndrome. This syndrome, usually caused by a lesion of the basis pontis, is characterized by quadriplegia with corticobulbar tract involvement and loss of the ability to produce speech. The reticular activating system is spared, so consciousness is preserved. Eye movements or blinking may be all that is left under voluntary control.

Another manifestation of bilateral lesions of the long tracts is pseudobulbar palsy. The symptoms resemble those that occur with lesions of the medulla (bulb). Cranial nerve nuclei have been disconnected from cortical input, however, which causes dysarthria, dysphagia, bilateral facial weakness, extremity weakness, and emotional lability. A more descriptive term for this syndrome is supranuclear bulbar palsy.

Blindness occurs with bilateral posterior cerebral artery occlusion and concomitant occipital lobe infarction.

Ischemic stroke syndromes are outlined next. These syndromes occur in isolation, as presented here, and in combination. The combinations can be medial, with lateral or often rostrocaudal extension. Several classic stroke syndromes, such as Wallenberg later medullary syndrome and Weber cerebral peduncle syndrome, exist. However, an investigation that looked prospectively at 304 cases of brainstem stroke found 1 of 24 named syndromes in only 20. About 20% of cases showed different unnamed crossed brainstem syndromes (Marx and Thomke, 2009).

Arterial Territory Involved MEDIAL Common Symptoms Signs Arterial Territory Involved LATERAL AND POSTERIOR INTERNAL CAPSULE Common Symptoms Signs Arterial Territory Involved POSTEROLATERAL Common Symptoms Signs Arterial Territory Involved

Midbrain Stroke Syndromes

Ischemia of the midbrain is characterized by long-tract signs combined with involvement of the third and fourth cranial nerves (Bogousslavsky et al., 1994). Supratentorial (anterior circulation) stroke syndromes may manifest with midbrain signs when rostrocaudal deterioration occurs, causing transtentorial herniation. Classifications of the blood supply to the brainstem are numerous, and this variability is nowhere more apparent than in the midbrain.

Blood flows to the upper mesencephalon via perforating branches of the basilar communicating artery. The basilar communicating artery (P1 segment of the posterior cerebral artery or mesencephalic artery) connects the basilar artery with the posterior communicating artery. A simplified scheme used here divides the vascular territories into medial and lateral transverse regions.

The medial midbrain syndromes are characterized by an ipsilateral third cranial nerve palsy associated with a contralateral hemiparesis. Loss of the discriminative sensations (proprioception, vibration, and stereognosis) with involvement of the medial lemniscus may occur. The lateral syndromes are composed of contralateral loss of pain and temperature sensation and ipsilateral Horner syndrome and loss of facial sensation. Ataxia may occur on either side. Ischemic stroke syndromes of the mesencephalon are outlined in Table 19.2, and Figs. 19.4 and 19.5 show the territories involved with occlusive and ischemic stroke syndromes in this area. Eponymic designations are given later in the text in Table 74.1. Mossuto-Agatiello (2006) identified a caudal paramedian midbrain syndrome with a distinctive clinical picture: bilateral cerebellar (truncal and gait) ataxia, eye movement disorders (nuclear third nerve palsy, INO), and palatal myoclonus.

Table 19.2 Ischemic Stroke Syndromes of the Mesencephalon

MIDDLE MEDIAN MIDBRAIN SYNDROME
Common Symptoms

Signs Arterial Territory Involved MIDDLE LATERAL MIDBRAIN SYNDROME (see Fig. 19.4) Common Symptoms Signs Arterial Territory Involved INFERIOR MEDIAL MIDBRAIN SYNDROME (see Fig. 19.5) Common Symptoms Signs Arterial Territory Involved INFERIOR LATERAL MIDBRAIN SYNDROME (see Fig. 19.5) Common Symptom Signs Arterial Territory Involved
image

Fig. 19.4 Midbrain at the superior colliculus level, showing the medial and lateral territories involved with occlusive stroke syndromes in this area.

(Reprinted with permission from DeArmond, S.J., Fusco, M.M., Dewey, M.M., 1976. Structure of the Human Brain, second ed. Oxford University Press, New York.)

image

Fig. 19.5 Midbrain at the inferior colliculus level, showing the medial and lateral territories involved with ischemic stroke syndromes in this area.

(Reprinted with permission from DeArmond, S.J., Fusco, M.M., Dewey, M.M., 1976. Structure of the Human Brain, second ed. Oxford University Press, New York.)

Pontine Stroke Syndromes

The pons is supplied by numerous penetrating branches of the basilar artery. These arteries have little collateral supply; consequently, lacunar syndromes (see Chapter 51A) can occur (Table 19.3). These syndromes (Figs. 19.6, 19.7, and 19.8) may be clinically indistinguishable from lacunar syndromes due to lesions of the internal capsule. More extensive paramedian syndromes are accompanied by characteristic pontine findings. The findings may fluctuate (pontine warning syndrome) and in this situation may be treated by intravenous venous tissue plasminogen activator (Saposnik et al., 2008). Contralateral hemiparesis, ipsilateral ataxia, INO, and conjugate horizontal gaze paresis characterize the medial syndromes.

Table 19.3 Ischemic Stroke Syndromes of the Pons

SUPERIOR MEDIAL PONTINE SYNDROME (see Fig. 19.6)
Common Symptoms

Signs Arterial Territory Involved SUPERIOR LATERAL PONTINE SYNDROME (see Fig. 19.6) Common Symptoms Signs Arterial Territory Involved MIDDLE MEDIAL PONTINE SYNDROME (see Fig. 19.7) Common Symptoms Signs Arterial Territory Involved MIDDLE LATERAL PONTINE SYNDROME (see Fig. 19.7) Common Symptoms Signs Arterial Territory Involved INFERIOR MEDIAL PONTINE SYNDROME (FOVILLE SYNDROME) (see Fig. 19.8) Common Symptoms Signs Arterial Territory Involved INFERIOR LATERAL PONTINE SYNDROME (ANTERIOR INFERIOR CEREBELLAR ARTERY SYNDROME) (see Fig. 19.8) Common Symptoms Signs Arterial Territory Involved
image

Fig. 19.6 Superior pontine level, showing the medial and lateral territories involved with occlusive stroke in this region.

(Reprinted with permission from Adams, R.D., Victor, M., 1993. Principles of Neurology, fifth ed. McGraw-Hill, New York.)

image

Fig. 19.7 Middle pontine level, showing the medial and lateral territories involved with ischemic stroke syndromes in this locality.

(Reprinted with permission from Adams, R.D., Victor, M., 1993. Principles of Neurology, fifth ed. McGraw-Hill, New York.)

image

Fig. 19.8 Inferior pons at the level of the sixth cranial nerve nucleus, showing the medial and lateral territories involved with occlusive stroke in this area.

(Reprinted with permission from Adams, R.D., Victor, M., 1993. Principles of Neurology, fifth ed. McGraw-Hill, New York.)

The lateral syndromes are distinguished by contralateral hemianesthesia and loss of discriminative sensation with ipsilateral Horner syndrome, facial hemianesthesia, and ataxia. Ipsilateral lower motor neuron–type facial paresis, sixth cranial nerve paresis, deafness, and vertigo occur with inferior pontine lesions.

Medullary Stroke Syndromes

Medial medullary ischemia (Fig. 19.9) can cause crossed hypoglossal hemiparesis syndrome (Table 19.4). In addition, patients may have loss of discriminative-type sensation (position sense, graphesthesia, and stereognosis) when there is associated medial lemniscus involvement. Kumral and associates (2002) have described four patterns of medial medullary stroke: (1) the most frequent, classic crossed hypoglossal hemiparesis syndrome, (2) sensorimotor stroke without lingual palsy, (3) pure hemiparesis, and (4) bilateral medial medullary stroke. Ocular motor findings often are prominent (Kim et al., 2005), with ocular contrapulsion, ocular tilt reaction, ipsilesional nystagmus (gaze-evoked, upbeating, and hemi-seesaw).

Table 19.4 Ischemic Stroke Syndromes of the Medulla

MEDIAL MEDULLARY SYNDROME (see Fig. 19.9)
Common Symptoms

Signs Arterial Territory Involved LATERAL MEDULLARY SYNDROME (WALLENBERG SYNDROME) (see Fig. 19.9) Common Symptoms Signs Arterial Territory Involved

Lateral medullary syndrome (Wallenberg syndrome; see Fig. 19.9) is one of the most dramatic clinical presentations in neurology (see Table 19.4). Long-tract signs (i.e., contralateral loss of pain and temperature sensation over half of the body, ipsilateral ataxia, ipsilateral axial lateropulsion [Eggers et al., 2009], Horner syndrome) are accompanied by involvement of the nuclei and fasciculi of cranial nerves V, VIII, IX, and X. Nystagmus often is present. The critical sign that distinguishes this from a lateral pontine syndrome is involvement of the nucleus ambiguus or its fasciculus and consequent weakness of the ipsilateral palate and vocal cord. It is increasingly found in the setting of vertebral artery dissection. (A more detailed discussion of stroke is presented in Chapter 51A.)

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