(Stroke. 1997;28:2400-2404.)
© 1997 American Heart Association, Inc.
Articles |
Causes and Mechanisms of Cerebellar Infarction in Young Patients
From the Stroke Clinic, Instituto Nacional de Neurologia y Neurocirugía, Manuel Velasco Suárez, Mexico City, Mexico.
Correspondence to Fernando Barinagarrementeria, MD, Stroke Clinic, Instituto Nacional de Neurologia y Neurocirugía, Manuel Velasco Suárez, Insurgentes Sur 3877, Tlalpan 41269, Mexico City, Mexico.
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Abstract |
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Background and Purpose The incidence of cerebellar infarction in a series of patients with stroke is approximately 1.5%. The average patient age in most reported series is 62 years. The most common etiologies in this age group are atherosclerosis and cardiac embolism. The aim of this study was to determine the causes and mechanisms of cerebellar infarction in patients younger than 40 years.
Methods We analyzed retrospectively the clinical and radiological data from 21 men and 16 women with cerebellar infarction admitted to our hospital from January 1986 to December 1996. The patients had been studied extensively to determine the etiology of the cerebellar infarction.
Results In the 37 patients (mean age, 30 years), 29 infarcts were limited to one territory (15 in the posteroinferior cerebellar artery [PICA]; 14 in the superior cerebellar artery); 8 had nonterritorial infarctions. The most common stroke mechanisms in each territory were as follows: PICA: nonatherosclerotic vasculopathic (67%), cardioembolic (20%), and hematologic and cryptogenic (each 7%); superior cerebellar artery: cardioembolic (42%), cryptogenic (31%), migrainous (21%), and nonatherosclerotic vasculopathic and hematologic (each 7%); and mixed territory: nonatherosclerotic vasculopathic (50%), cryptogenic (25%), cardioembolic (12%), and hematologic (12%).
Conclusions The most common mechanism of cerebellar infarctions was arterial occlusion as a result of intracranial vertebral artery dissection (40%), mainly with PICA involvement. Embolism from a cardiac source resulted primarily from patent foramen ovale and rheumatic valvular disease. Hematologic disturbances and migraine were responsible for a few cases.
Key Words: cardioembolic stroke • cerebellar infarction • dissection • procoagulant • young adults
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Introduction |
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The incidence of cerebellar infarction in a series of patients with stroke is approximately 1.5%.1 The average patient age in most reported series is 62 years.2 3 4 5 The most common etiologies in this age group are atherosclerosis and cardiac embolism.2 3 4 6 In recent years, although numerous series of cerebral infarction in young patients have been reported, none has presented a detailed discussion of the primary causes of cerebellar infarction in this age group.7 8 9 10 The goal of the present study was to determine the causes and mechanisms of cerebellar infarction in patients younger than 40 years.
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Subjects and Methods |
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Among 1657 consecutive patients admitted to our stroke clinic from January 1986 to December 1996, 332 patients with brain infarction were younger than 40 years. We analyzed retrospectively the clinical and radiological data from those patients with neuroimaging evidence of cerebellar infarction.
The inclusion criteria were age younger than 40 years at the time of the cerebellar infarction; availability of detailed clinical information relating to risk factors, clinical manifestations, hospital course, and final outcome (clinical evaluation performed at hospital discharge); performance of a detailed neurological examination; rheumatologic profile and hematologic tests; documentation of the cerebellar infarction by means of CT or MRI; and investigation of the mechanism of cerebellar infarction by means of transthoracic or transesophageal echocardiography, transcranial Doppler, and sonography of vessels of the neck.
In each case the presence of associated brain infarctions in other regions (brain stem, thalamus, occipital), the presence of hydrocephalus, and the mass effect were analyzed. Cerebellar infarction was classified as territorial (PICA, SCA) and nonterritorial (mixed), ie, those that did not correspond to an arterial territory as defined by Amarenco et al.11 Clinical outcome was determined at hospital discharge as follows: (1) total recovery; (2) mild sequelae, in which the patients were able to work, although they might have mild complaints; (3) severe sequelae, in which patients had physical limitations and serious neurological symptoms that made them unable to engage in everyday activities; and (4) death.
Differences between groups were tested for statistical significance
with the 
2 test and Fisher's exact test.
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Results |
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We studied 37 patients: 29 with territorial cerebellar infarcts (15 PICA, 14 SCA) and 8 with nonterritorial infarction. The series included 21 men (57%) and 16 women (43%) (mean age, 30 years; range, 16 to 40 years). Risk factors included tobacco use in 4 patients (11%), rheumatic valve disease in 3 (8%), migraine in 3 (8%), hypertriglyceridemia in 2 (5%), arterial hypertension in 2 (5%), and alcohol intoxication in 1 (3%). The most common signs and symptoms in each territory are shown in Table 1
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Territorial Infarctions
PICA
All 15 patients (40%) had unilateral PICA territory infarcts: 6
had infarcts that involved partial vascular territories (4 medial PICA;
2 border-zone medial and lateral territories of the PICA), and 9
involved the full PICA territory (Table 2). A dorsal medullary infarct was
detected by MRI in 6 patients, 4 of whom had full PICA territorial
infarctions.
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The most common signs and symptoms in this group were vertigo, gait instability, and headache. Ten patients had a normal level of consciousness, 4 patients had somnolence, and 1 was stuporous since the onset of the disease. Six patients had clinical findings of Wallenberg's syndrome.
Cerebral arteriography was performed in 12 patients, and the findings were abnormal in 10 patients (83%). All patients had angiographic findings compatible with vertebral arterial dissection: 2 patients had extracranial vertebral dissection, and 8 had intracranial vertebral dissection. The cardiac evaluation disclosed patent foramen ovale, rheumatic valve disease, and nonrheumatic valvular disease related to antiphospholipid syndrome in 1 patient each.
MRI was performed in 14 patients and showed slight brain stem compression in 3 patients, 2 of whom had full PICA territorial involvement. Three patients developed hydrocephalus that did not require shunting.
The most common mechanism of stroke was arterial occlusion
resulting primarily from nonatherosclerotic vasculopathy (40%). This
etiology was more prevalent in patients with PICA involvement
(P=.03) (versus non-PICA) and less common in patients with
SCA involvement (P=.03) (versus non-SCA). The most probable
stroke mechanisms in this group were as follows: vertebral artery
dissection mainly from an intracranial location (n=10),
cardioembolism from rheumatic mitral valvular
disease (n=1), patent foramen ovale (n=1), protein S deficiency (n=1),
and antiphospholipid syndrome with evidence of nonrheumatic nodular
valvular disease (n=1). One patient had no identifiable cause
of stroke (Table 3).
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Fourteen patients (93%) had good recovery: 8 patients (53%) recovered
totally, and 6 (40%) had mild sequelae and functioned independently.
One patient died from the mass effect of the stroke (pseudotumoral
infarct) (Table 4).
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SCA
Of the 14 patients in this group, 8 had full SCA territorial
involvement, and 3 each had medial SCA branch involvement and lateral
SCA branch involvement (Table 2
). The main signs and symptoms were gait
instability, limb ataxia, and vertigo (Table 1).
Cerebral arteriography was performed in 12 patients. The findings were abnormal in 2 (17%): 1 patient had fibromuscular dysplasia of the extracranial portion of the vertebral artery, and the other had evidence of basilar stenosis of unknown origin. The cardiac evaluation disclosed patent foramen ovale in 3 patients, rheumatic valvular disease in 2, and mitral valve prolapse with myxomatous degeneration in 1. A hematologic workup disclosed a combined protein S deficiency and antiphospholipid syndrome in 1 patient.
The stroke mechanisms in this group were as follows:
cardioembolism (n=6), nonatherosclerotic vasculopathy
(n=1), migraine (n=3), protein S deficiency (n=1), and cryptogenic
stroke (n=3) (Table 3).
Ten of 14 patients (71%) had a complete neurological recovery, 3
(21%) had mild neurological sequelae, and 1 (7%) had severe sequelae
(Table 4).
Nonterritorial Infarctions
All 8 patients in this group had SCA involvement. Two patients had
cerebellar infarcts that included the AICA and SCA territories, 3 had
infarcts that included the PICA and SCA territories, and 3 had
compromised PICA-AICA and SCA territories (Table 5). Six patients had a brain infarction
in other locations (Table 5). The main signs and symptoms were vertigo,
gait instability, and limb ataxia. A decreased level of alertness was
present in 6 (75%) (P=.005) (Table 1).
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MRI was performed in all patients, and compression of the brain stem was found in 6. Cerebral arteriography was performed in 8 patients and was abnormal in 4 (3 with primary central nervous system vasculitis and 1 with vascular changes related to neurofibromatosis). Echocardiographic evaluation, which was performed in 6 patients, disclosed patent foramen ovale in 1 patient.
The stroke mechanism in this group included nonatherosclerotic
vasculopathy (n=4, 3 with cerebral vasculitis and 1 with
neurofibromatosis), cardioembolism (n=1, patent foramen
ovale), protein S deficiency (n=1), and cryptogenic stroke (n=2) (Table 3).
Only 1 patient (12%) had a complete neurological recovery, 6 (76%) had mild sequelae, and 1 (12.5%) had severe sequelae.
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Discussion |
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TopAbstractIntroductionSubjects and MethodsResultsDiscussionReferences |
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Cerebellar infarction in young patients has been poorly studied. Interestingly, the first reported case of a fatal cerebellar infarction occurred in a 27-year-old woman with syphilis.12 In 1975, Sypert and Alvord13 published a clinicopathologic analysis of 28 patients with acute cerebellar infarction. In this series, all patients were older than 40 years. Among the reported causes of cerebellar infarction in young people the following have been reported: migraine,14 fibromuscular dysplasia,15 vertebral dissection,16 and embolism from various cardiac sources.6 17 18
As in other series,2 3 4 5 men were affected more often than women. More than half of the women with cerebellar infarction were younger than 30 years compared with 38% of male patients. In the present series, the most commonly affected territory was the PICA, which agrees with the results of recently published clinicoradiologic and clinicopathologic series.2 3 4 5 6 17
Classically, the primary mechanisms of cerebellar infarction included embolism from a cardiac source and arterial occlusion resulting from atherosclerosis. In the present series, the frequency of affected cerebellar arteries in young patients was similar to that in other age groups, ie, a high frequency of PICA involvement followed by SCA involvement. There was no case of pure AICA involvement or "very small cerebellar infarcts."19
Particular etiologies in this group included vertebral arterial dissection in 67% (10/15) of patients. Interestingly, only 2 patients had extracranial vertebral artery dissection; the rest had intracranial vertebral dissection. This finding contrasts with the frequency of vertebral dissection reported by Caplan et al20 and Mas et al.21 Intracranial vertebral dissection can cause cerebellar infarction because of the development of intraluminal thrombi, which can be the source of intra-arterial emboli,22 or arterial occlusion involving the intracranial portion of the vertebral artery facing the PICA ostium and the origin of the PICA.23 Other etiologies included granulomatous vasculitis in 3 patients, all with multiple compromised vessels of the vertebrobasilar system. In 2 patients, diagnosis was reached by leptomeningeal biopsy; in the remaining patients, the angiographic findings and response to cyclophosphamide-prednisone therapy were considered diagnostic.24
Intracranial arterial occlusive disease associated with neurofibromatosis was responsible for cerebellar infarction in 1 patient with multiple cerebellar artery involvement. Uncommonly, neurofibromatosis can produce occlusion of the posterior circulatory arteries related to eccentric, fibrous intimal proliferation, smooth muscle fibrosis, and elastica fragmentation.25
Fibromuscular dysplasia, a segmental nonatheromatous stenotic disease involving small to medium-sized arteries,26 occasionally has been reported as a cause of cerebellar infarction in young patients. In 1 patient in the present study, fibromuscular dysplasia (type I) produced a cerebellar infarction in the SCA artery that was probably related to an intra-arterial embolism.
Cerebellar embolism from a cardiac source was the second most commonly found mechanism (27%). This finding agrees with findings from other series,2 4 18 ie, that the most commonly involved arteries are the SCAs. In this series, 42% of cases with cerebellar infarction resulting from cardioembolism affected the SCA territory. The most frequent causes included patent foramen ovale in 5 patients, rheumatic valvular disease in 3, nonrheumatic mitral valvular disease related to antiphospholipid syndrome in 1, and mitral valve prolapse in 1. In those patient with patent foramen ovale and mitral valve disease there were no other potential causes of stroke. Patent foramen ovale is a well-known cause of stroke, especially in young patients who have had cryptogenic stroke.27 28 Nater et al29 reported a high frequency of posterior circulatory stroke in patients with patent foramen ovale and atrial septum aneurysm. Paradoxical embolism from a venous source through a right-to-left shunt is usually incriminated,28 30 as was seen in one of our patients in whom there were coexistent antiphospholipid antibodies with deep venous thrombosis and patent foramen ovale.
Cryptogenic stroke was found in 6 patients (16%), which is similar to other reports of cerebral infarction in young patients.7 10 It must noted that not all patients underwent transesophageal echocardiography or an extensive hematologic workup.
Hematologic disturbances explained cerebellar infarction in 3 patients (8%). Specific causes included protein S deficiency in 2 patients and protein S deficiency with antiphospholipid syndrome in 1. Several articles recently have shown that hematologic disorders promote a prothrombotic state, which is a well-known cause of cerebral ischemia.31 Protein S deficiency is the most common type of prothrombotic state related to cerebral ischemia.32 33
Finally, migraine was responsible for cerebellar infarction in 3 cases (8%), which is slightly higher than reported in other series of brain infarction in young patients7 9 34 but is similar to that reported by Chaves et al.2 Migraine can cause cerebral infarction as a result of severe and prolonged vasoconstriction that leads to prolonged hypoperfusion and cerebral infarction.35 Caplan36 described 5 patients with cerebellar infarction related to migraine, 4 of whom had abnormal cerebral angiograms. All of our patients had SCA involvement with no evidence of a cardiac source of embolism.
In conclusion, cerebellar infarction in young patients has a wider etiologic spectrum than in older patients. The principal mechanism of stroke is arterial occlusion resulting from nonatherosclerotic vasculopathy, mainly vertebral artery dissection with a close relationship to PICA territory involvement, followed by cardioembolism related to patent foramen ovale and rheumatic valvular disease, mainly with SCA involvement. Hematologic disturbances and migraine were responsible for a very small number of cases.
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Selected Abbreviations and Acronyms |
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Received June 24, 1997; revision received September 9, 1997; accepted September 9, 1997.
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References |
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