Case Review - A 45 year old male with headache
Anastasia Markopoulou, MD
HISTORY
A 45 year-old male presented to the emergency department with a two week history of a severe headache. The pain was located in his neck and occipital area, was diffuse bilaterally, and radiated to the top of his head. The headache started suddenly without any precipitating factors and quickly worsened. The pain was severe, throbbing in quality, and continuous in nature. He denied any fever, chills, chest pain, nausea, vomiting, aura, photophobia, or other changes in his vision. There was no history of trauma, weakness, numbness or tingling in his extremities, bladder or bowel dysfunction, or difficulty ambulating. He denied changes in his handwriting or problems using his hands for simple tasks such as opening jars, zipping zippers, or buttoning buttons. The patient did report a metallic taste over the last couple of days.
He had sought medical attention, in different hospitals, for his headache over the course of the previous two weeks. An MRI of his brain and a lumbar puncture, done two days prior to his admission, were both negative for any pathology. A cervical spine MRI performed three days ago demonstrated cervical stenosis.
His past medical history was significant for hypertension, gastroesophageal reflux disease, and surgery on an esophageal stricture. His medications included hydrochlorothiazide and acetaminophen/hydrocodone. The patient’s family history was significant only for congestive heart failure. He smoked one pack of cigarettes every week and drank alcohol occasionally.
PHYSICAL EXAMINATION
On physical examination his vital signs were significant for an elevated blood pressure of 190/140 mmHg. He was in moderate distress, awake, alert and oriented to person, place and time. His pupils were equal, round, and reactive to light, and conjunctivae were normal. There was mild postnasal drip and no sinus tenderness. His neck was supple without carotid bruits. His lungs were clear to auscultation bilaterally and the heart and abdominal exam were normal. No skin abnormalities were noted. The neurological examination including cranial nerves, reflexes, gait, sensory function, and cerebellar function were normal. The motor examination revealed normal strength bilaterally and the patient ambulated with a normal gait. Hoffman’s sign was negative bilaterally.
HOSPITAL COURSE
A Computed Tomography (CT) of the head was performed which ruled out any acute intracranial pathology but did reveal subtle findings of mucosal thickening in a few of the ethmoidal air cells. Due to the presence of postnasal drip on examination and the CT findings, the possibility of sinusitis was further evaluated by maxillo-facial computed tomography imaging.
Figure 1: Maxillofacial CT revealed unremarkable paranasal sinuses, normal mastoid air cells, and findings suspicious for bilateral carotid dissection.
Figure 2: Magnetic Resonance Angiography of the neck confirmed the diagnosis of bilateral internal carotid artery dissections
The Neuroendovascular service was consulted and a recommendation to anti-coagulate with a target INR of 2-3 was made. Follow-up imaging was also recommended and a CT-angiography three months later revealed disappearance of all of the abnormalities related to bilateral carotid dissection seen previously. His physical examination remained essentially normal.
DISCUSSION
Epidemiology
Carotid and vertebral dissection are such difficult diagnoses that until recently a diagnosis was not made until the postmortem examination. Although a high index of suspicion is required to make the diagnosis, the advent of noninvasive imaging has increased the number of diagnoses. Both conditions occur most frequently in the fifth decade of life and account for approximately five percent of ischemic strokes in young patients (thirty to fifty years-of-age) occurring most commonly on the right side (eighty percent).
The annual incidence in the US of symptomatic spontaneous internal carotid artery dissection ranges from 2.5-3 per 100,000. The actual incidence may be higher as some dissections are asymptomatic or cause only minor transient symptoms and remain undiagnosed.
Pathogenesis
The pathologic process begins with an intimal tear. The blood under arterial pressure dissects along the artery to create an intramural hematoma, which can either narrow the carotid artery lumen or cause an aneurysmal dilatation. Both processes can cause stenosis or thromboemboli.
The intimal tear can be caused by minor or major trauma, or can be spontaneous in nature. Minor trauma as a precipitating event is associated with hyperextension or rotation of the neck. Common situations engendering this include: practicing yoga, painting a ceiling, coughing, vomiting, sneezing, the receipt of anesthesia, and medical resuscitation. Such neck movements, particularly when they are sudden, may injure the artery as a result of mechanical stretching. On the other hand, major trauma involves motor vehicle accidents and certain sports injuries.
In spontaneous cases, genetic, familial and/or heritable disorders must be considered as likely etiologies. Connective tissue disorders that are associated with an increased risk of spontaneous dissections of the carotid and vertebral arteries are Ehlers-Danlos syndrome type IV, Marfan’s syndrome, autosomal dominant polycystic kidney disease, and osteogenesis imperfecta type I. One fifth of patients have a clinically apparent but as yet undiagnosed connective-tissue disorder. In addition, five percent of patients have at least one family member with a history of a spontaneous dissection of the aorta or its main branches, including the carotid and vertebral arteries.
Clinical Presentation
The clinical manifestations can be subtle. Some dissections remain asymptomatic, but most patients have at least two symptoms. The typical patient presents with head, face, or neck pain ipsilateral to the dissection accompanied by a partial Horner’s syndrome and followed hours or days later by cerebral or retinal ischemia. This classic triad is found in less than one third of patients, but the presence of any two elements of this triad provides an opportunity to recognize and treat patients before cerebral ischemia occurs.
The initial and occasionally isolated manifestation can be headache and facial or orbital pain ipsilateral to the dissection. The onset of pain is usually gradual, but it can also be an instantaneous, excruciating, “thunderclap” headache that mimics a subarachnoid hemorrhage. It may resemble a migraine but most patients consider the headache or facial pain to be unique and unusual, compared with previously experienced headache or neck pain episodes. The median time to the appearance of other symptoms, if they do appear, is four days.
The oculosympathetic palsy, or Horner’s syndrome, consisting of miosis and ptosis, may not be detected clinically and is found in less than half of patients. Facial anhidrosis is not present because the facial sweat glands are innervated by the sympathetic plexus surrounding the external carotid artery. Dissection may mimic a cluster headache as it is associated with severe orbital pain. Even in the absence of other symptoms oculosympathetic palsy should be considered to be due to carotid-artery dissection until proven otherwise.
Cranial nerve palsies are found in about 12 percent of patients. The vulnerability of the affected nerves is usually attributed to mechanical compression by the locally expanding internal carotid artery mural hematoma, rather than to ischemia. The lower cranial nerves are the most commonly affected, particularly the hypoglossal nerve, though the involvement of various combinations of nerves has been described. Of note, among the possible etiologies of the 12th cranial nerve palsy, a dissection of the ICA should be considered in the differential diagnosis. Impairment of taste, noted in 10% of patients, may be the presenting symptom and is due to the involvement of the chorda tympani. The dysgeusia usually resolves spontaneously. Pulsatile tinnitus is reported in one fourth of patients, and an objective bruit may be present on auscultation.
Cerebral or retinal ischemic symptoms were reported in fifty to ninety-five percent of patients although this percentage has decreased over the years as the condition is diagnosed in more patients with less obvious manifestations. Transient ischemic attacks or transient monocular blindness usually precedes the ischemic stroke and only about one fifth of patients have an ischemic stroke without any warning signs.
Diagnostic tests
Magnetic resonance techniques are replacing conventional angiography as the gold standard in the diagnosis of dissections of the carotid and vertebral arteries. This shift is made possible by the fact that the resolution of magnetic resonance angiography now approaches that of conventional angiography and can display the intramural hematoma itself. MRA imaging is superior to angiography in the diagnosis of dissection without associated luminal abnormalities or in cases resulting in no specific occlusion. MRA imaging is also useful for evaluating other intra- or extracranial cervical arterial dissections.
Doppler ultrasonography can currently be used for the initial assessment and an abnormal blood flow pattern can be seen in up to ninety percent of patients with carotid artery dissection. Due to the limited ability of ultrasonography to evaluate past the carotid bulb, the actual site of injury is usually not seen.
It is important to remember that dissections of the carotid and vertebral arteries are dynamic processes and the radiographic findings may change dramatically within hours.
Treatment
To prevent thromboembolic complications, anticoagulation with intravenous heparin followed by oral warfarin has been recommended for all patients with acute dissections, regardless of the type of symptoms, unless there are contraindications such as intracranial extension of the dissection. Anticoagulation with a target INR of 2-3 is generally used for a duration of three to six months. Endovascular therapy with stent placement and surgical repair has also been used to treat dissection, mainly for patients who have recurrent ischemia despite antithrombotic therapy.
Eventually, ninety percent of stenoses resolve, two thirds of occlusions are recanalized, and one third of aneurysms decrease in size. These improvements take place largely within the first two to three months after the dissection and are seldom noted past six months. In rare cases, persistent extracranial aneurysms may cause thromboembolic complications years after the dissection, but they do not tend to rupture.
REFERENCES
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- Kistler JP, Furie KL and Ay H. Treatment for specific causes of ischemic stroke and transient ischemic attack. Uptodate 2006.
- Pakdemirli E, Usal D and Tali ET. Spontaneous bilateral internal carotid artery dissection with hypoglossal nerve palsy. Computerized Medical Imaging and Graphics 2001; 25:373-378.
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- Wouter I. Schievink, MD Spontaneous Dissection of the Carotid and Vertebral arteries. New England Journal of Medicine 2006; 344:898-905.