A 56-year-old man presents with a one-week history of nausea, headache, dizziness, blurred vision, and feeling “off balance.” He denies a history of fevers, trauma, paresthesias, rash, neck pain, or stiffness. He has a history of hypertension, and had a middle cerebral artery hemorrhagic stroke three years earlier.
On examination, he has nystagmus, difficulty with conjugate gaze, spontaneous abnormal vertical eye movements, left facial droop, and ataxia (falls to the left). What conditions are you concerned about?
Diagnosis: Cerebellar Hemorrhage
The cerebellum (Latin for “little brain”) is the balance center for the body, and is responsible for coordinating movement. Interestingly, recent literature notes that the cerebellum also may play a role in language, motor learning, and sensory cognitive functions. (Cerebellum 2003; 2:114.) The cerebellum is a bihemispheric structure located just posterior to the pons and inferior to the occipital lobe. It is supplied by three major arteries: the superior cerebellar artery, the anterior inferior cerebellar artery, and the posterior inferior cerebellar artery. Because of its dense blood supply, relative containment within the bony skull, and proximity to the neural-pathway-dense pons, lesions caused by local tumors, infarction, ischemia, or hemorrhage in this area can result in myriad clinical presentations.
Approximately 20 percent of intracranial ischemic events occur within the posterior circulatory (vertebrobasilar system). Most cerebellar strokes are ischemic, with one- third caused by thromboembolism. Hemorrhagic stroke is most common in Asians and then blacks when compared with whites, with approximately 16 percent of intracerebral hemorrhages occurring in the cerebellum. (Stroke: Pathophysiology, Diagnosis, and Management. Philadelphia: Churchill Livingstone; 1998.) Extremely low birth weight infants (<750 g) are also high-risk for cerebellar hemorrhage. (Pediatrics 2005;116:717.)
The cerebellum is a common location of hypertension-related hemorrhage. Intracerebral hemorrhage is a potentially life-threatening condition that requires prompt recognition. The etiology of hemorrhage is fairly circumscribed, and includes hypertension (from trauma, tumor/mass, bleeding dyscrasia, amyloid angiopathy, infection, vasculitis, congenital malformation, and rarely post-spinal (Neurosurgery 2002;50:1361) or intracranial (Am J Neuroradiol 2006; 27:387) surgery.
Intracranial hemorrhage, including cerebellar hemorrhage, can result in brain injury by anoxia of local tissues due to compromised blood flow from hemorrhagic vessels and direct clot expansion. Secondary injury can result from tissue edema and subsequent increased intracranial pressure that can lead to herniation. Injured tissue is further damaged by inflammatory cascade mediators for a trifecta of insult to the brain tissue.
Cerebellar disease including hemorrhage, infarction, or metabolic-induced neuropathology can manifest in a variety of ways depending on the type and severity of the underlying condition. Diagnosis of posterior circulation cerebrovascular syndromes can be difficult. Patients with cerebellar ischemia or hemorrhage may have an acute or insidious onset of symptoms. Common complaints include vertigo, vomiting, blurred vision or diplopia, headache, face pain or paresthesias, referred pain in the neck or shoulder, neck stiffness, hiccups, dizziness, syncope, altered mental status, loss of balance, and the inability to walk.
Physical examination characteristics may vary. Patients may be ataxic, veer to one side (ipsilateral), have nuchal rigidity, gaze palsy, facial weakness with no hemiparesis, nystagmus, conjugate gaze paresis, ocular torsion, ocular bobbing, limb incoordination, decreased consciousness or unresponsive. Patients may also have neurogenic cardiopulmonary findings including bradycardia, arrhythmia, and hypertension. The differential diagnosis of cerebellar hemorrhage can be extensive depending on the patient's presentation, and can include the expansive differential of syncope, vertigo, and dizziness.
Patients with suspected cerebellar disease including hemorrhage should have head CT performed in the ED. Depending on the condition suspected or identified, further laboratory or radiological evaluation may be warranted. Coagulation studies, platelet count, and hematocrit should be performed for those suspected of an intracranial hemorrhage. If cerebellar tumor or hemorrhage is suspected or identified on CT, MRI or cerebral angiography may be warranted to further characterize the lesion and identify possible underlying etiologies such as vascular malformation. Treatment in the ED will depend on the underlying etiology. If intracerebellar hemorrhage is identified, prompt neurosurgical evaluation is warranted. Although immediate neurosurgical intervention was once the standard of care, early detection of small hemorrhage with today's improved diagnostic imaging has made “watchful waiting” an acceptable approach for some patients depending on the patient's clinical condition, comorbidities, and hemorrhage size.
Acute deterioration of a patient known or suspected to have an intracranial hemorrhage is due to an expanding hematoma until proven otherwise. The hemorrhage size and patient's clinical condition will guide the ED treatments and therapies, but standard resuscitation protocols should be followed. If hemorrhage is caused or exacerbated by pharmacological agents, including heparin or warfarin, reversal agents should be considered. All patients with intracranial hemorrhage should be admitted to critical care for close monitoring and frequent neurological checks.
Prior to 1960, the diagnosis of cerebellar hemorrhage was typically made on autopsy. (West J Med 1982; 136:198.) Today, the prognosis of patients with intracranial hemorrhage depends on the location and size of the hemorrhage, premorbidities, and contribution of anticoagulants. In general, the prognosis tends to be grim: 50 percent of patients die within 30 days, half within the first two days. Patients who present obtunded, with large volume hemorrhage, and have rapidly expanding hematomas also typically do poorly.
This patient was ultimately diagnosed with an ependymoma and arteriovenous malformation with hemorrhage. The patient did well with conservative management, and was discharged home two days later.
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