Wouter I. Schievink

Headache and neck pain in spontaneous internal carotid and vertebral artery dissections

We studied the characteristics of headaches in 161 consecutive symptomatic patients with spontaneous dissections of the internal carotid artery (n equals 135) or the vertebral artery (n equals 26).For patients with internal carotid artery dissection (ICAD), the mean age was 47 years and for those with vertebral artery dissection (VAD), 40.7 years. A history of migraine was present in 18% of the ICAD group and in 23% of the VAD group. Headache was reported by 68% of the patients with ICAD and by 69% of those with VAD, and, when present, it was the initial manifestation in 47% of those with ICAD and in 33% of those with VAD. Ten percent of patients with ICAD had eye, facial, or ear pain without headache. The median interval from onset of headache to development of other neurologic manifestations was 4 days for the ICAD group and 14.5 hours for the VAD group. For all dissections, headaches typically were ipsilateral to the side of dissection. In the ICAD group, headaches were limited to the anterior head in 60% of patients and were steady in 73% and pulsating in 25%. In the VAD group, headaches were distributed posteriorly in 83% of patients and were steady in 56% and pulsating in 44%. Neck pain was present in 26% of patients with ICAD (anterolateral) and in 46% of those with VAD (posterior). The median duration of the headache in patients with VAD and ICAD was 72 hours, but headaches became prolonged, persisting for months to years, in four patients with ICAD. NEUROLOGY 1995;45: 1517-1522

Neurovascular manifestations of heritable connective tissue disorders. A review.

Background Heritable disorders of connective tissue are recognized in a small minority of patients with neurovascular diseases. In this report, we review the neurovascular manifestations of four heritable connective tissue disorders: Ehlers‐Danlos syndrome, Marfans syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum, as well as two other systemic disorders with potential vascular manifestations: neurofibromatosis and polycystic kidney disease. Summary of Review Typical neurovascular complications of Ehlers‐Danlos syndrome are carotid‐cavernous fistulae, intracranial aneurysms, and cervical artery dissections. Arterial dissections and intracranial aneurysms cause the majority of neurovascular symptoms in Marfans syndrome. Neurovascular disease is uncommon in osteogenesis imperfecta, although carotid‐cavernous fistulae and vertebral artery dissections have been reported. Neurovascular disease in pseudoxanthoma elasticum is characterized by intracranial aneurysms and cerebral ischemia caused by premature arterial occlusive disease. Intracranial occlusive arterial disease is the most common neurovascular manifestation of neurofibromatosis, followed by cervical arteriovenous fistulae and aneurysms and intracranial aneurysms. Intracranial aneurysms are the hallmark of polycystic kidney disease. Conclusions Recognition of an underlying generalized connective tissue disorder may be of considerable importance, although marked phenotypic heterogeneity often complicates the diagnosis of these disorders. Conversely, the association of certain neurovascular anomalies with generalized connective tissue disorders and recognition of their basic molecular defect may offer clues to the etiology and pathogenesis of these neurovascular diseases in general. (Stroke. 1994;25:889‐903.)

Genetics of Intracranial Aneurysms

The etiology and pathogenesis of intracranial aneurysms are clearly multifactorial, with genetic factors playing an increasingly recognized role. Intracranial aneurysms have been associated with numerous heritable connective tissue disorders, which account for at least 5% of cases. Of these disorders, the most important are Ehlers-Danlos syndrome Type IV, Marfans syndrome, neurofibromatosis Type 1, and autosomal dominant polycystic kidney disease; the association with intracranial aneurysms, however, has been firmly established only for polycystic kidney disease. Familial intracranial aneurysms are not rare but account for 7 to 20% of patients with aneurysmal subarachnoid hemorrhage and are generally not associated with any of the known heritable connective tissue disorders. First-degree relatives of patients with aneurysmal subarachnoid hemorrhage are at an approximately fourfold increased risk of suffering ruptured intracranial aneurysms, compared to the general population. Various possible modes of inheritance have been identified in families with intracranial aneurysms, suggesting genetic heterogeneity. Although the benefits have never been quantified, screening for asymptomatic intracranial aneurysms should be considered in families with two or more affected members. The yield of such a screening program may approximate 10%. Although it is unlikely that there is a single gene with major effect, much effort is currently being directed at locating intracranial aneurysm genes.

Sudden death from aneurysmal subarachnoid hemorrhage

Article abstract—Some patients with aneurysmal subarachnoid hemorrhage (SAH) never reach the hospital alive (“sudden death”) and, although their numbers are significant, they are not included in most studies of SAH. To clarify the clinical profile of sudden death from aneurysmal SAH, we reviewed the epidemiology and clinicopathologic features of patients with aneurysmal SAH who never reached medical attention. Using the medical record linkage system employed for epidemiologic studies for the population of Rochester, Minnesota, we identified all patients who were diagnosed with aneurysmal SAH between 1960 and 1989. There were 80 women and 33 men with a mean age of 55 years. Of these 113 patients, 13 (12%) died without reaching medical attention. The proportion of those with sudden death remained stable during the study period. In comparing patients with sudden death with those who reached medical attention, the only significant variable was the frequency of posterior circulation aneurysms that was found in 38%, compared with 14% in those who received medical attention (p = 0.042). At autopsy, intraventricular hemorrhage was present in 12 patients (92%) with sudden death, and intracerebral hemorrhage was present in two (15%). Twelve patients (92%) had acute pulmonary edema. In our community, the frequency of sudden death from aneurysmal SAH has not changed during the last three decades in spite of the advances in medical care. The typical clinical profile of sudden death in SAH includes intraventricular hemorrhage, pulmonary edema, and a ruptured posterior circulation aneurysm. Intracerebral hemorrhage is uncommonly associated with sudden death from aneurysmal SAH.

Internal carotid artery dissection in a community. Rochester, Minnesota, 1987-1992.

Background and Purpose Cervical internal carotid artery dissections are diagnosed with an increasing frequency, but reliable epidemiologic data are not available. The aim of this study was to determine the incidence rate of spontaneous cervical internal carotid artery dissection in a defined population. Methods Using the medical record linkage system used for epidemiologic studies in Rochester, Minn, all patients diagnosed with spontaneous cervical ICA dissection for 1987 through 1992 were identified. Results A total of 10 patients with spontaneous cervical internal carotid artery dissection (6 women and 4 men; mean age, 44 years) were identified. For the period 1987 through 1992, the average annual incidence rate for all ages was 2.6 per 100 000 (95% confidence intervals, 0.9 to 4.2). Conclusions This study, for the first time, provides incidence rates for spontaneous cervical internal carotid artery dissections. No diagnoses were made before 1987, probably reflecting an increased awareness of the disorder among physicians. (Stroke. 1993;24:1678-1680.)

Surgical treatment of spontaneous spinal cerebrospinal fluid leaks

OBJECT Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension and may require neurosurgical intervention. In the present report the authors review their experience with the surgical management of spontaneous spinal CSF leaks. METHODS Between 1992 and 1997, 10 patients with spontaneous spinal CSF leaks and intracranial hypotension were treated surgically. The mean age of the seven women and three men was 42.3 years (range 22-61 years). Preoperative imaging showed a single meningeal diverticulum in two patients, a complex of diverticula in one patient, and a focal CSF leak alone in seven patients. Surgical exploration in these seven patients demonstrated meningeal diverticula in one patient; no clear source of CSF leakage could be identified in the remaining six patients. Treatment consisted of ligation of the diverticula or packing of the epidural space with muscle or Gelfoam. Multiple simultaneous spinal CSF leaks were identified in three patients. CONCLUSIONS All patients experienced complete relief of their headaches postoperatively. There has been no recurrence of symptoms in any of the patients during a mean follow-up period of 19 months (range 3-58 months; 16 person-years of cumulative follow up). Complications consisted of transient intracranial hypertension in one patient and leg numbness in another patient. Although the disease is often self-limiting, surgical treatment has an important role in the management of spontaneous spinal CSF leaks. Surgery is effective in eliminating the headaches and the morbidity is generally low. Surgical exploration for a focal CSF leak, as demonstrated on radiographic studies, usually does not reveal a clear source of the leak. Some patients may have multiple simultaneous CSF leaks.

Cranial nerve palsy in spontaneous dissection of the extracranial internal carotid artery

Cranial nerve palsy was present in 23 of 190 consecutive adult patients (12%) with spontaneous dissection of the extracranial internal carotid artery.Ten patients (5.2%) had a syndrome of lower cranial nerve palsies (with invariable involvement of cranial nerve XII with or without additional involvement of cranial nerves XI, X, and IX), seven (3.7%) had palsy of cranial nerve V, and five (2.6%) had a syndrome of ocular motor palsies. Palsy of cranial nerve VIII and ischemic optic neuropathy occurred in one patient each. Three patients had dysgeusia without other cranial nerve involvement, presumably due to involvement of the chorda tympani nerve. Headache or face pain (often unilateral) was present in 83% of patients. Other associated manifestations were cerebral ischemic symptoms, bruits, or oculosympathetic palsy. In one patient, cranial nerve palsy was the only manifestation of internal carotid artery dissection, and in another patient, the disease presented only as a palsy of cranial nerve XII and oculosympathetic palsy. In six patients, a syndrome of hemicrania and ipsilateral cranial nerve palsy was the sole manifestation of internal carotid artery dissection. Cranial nerve palsy is not rare in internal carotid artery dissection. Compression or stretching of the nerve by the expanded artery may explain some but not all of the palsies. An alternative mechanism is likely interruption of the nutrient vessels supplying the nerve. NEUROLOGY 1996;46: 356-359

Spontaneous dissections of cervicocephalic arteries in childhood and adolescence

Among 263 consecutive patients with spontaneous cervicocephalic arterial dissections evaluated at the Mayo Clinic, 18 (6.8%) were 18 years of age or younger (mean age, 12 years). The dissection involved the cervical arteries in 11 patients and the intracranial arteries in seven. Extracranially, the internal carotid artery was involved in eight patients, the vertebral artery in two, and both arteries in one. Intracranially, only the anterior circulation was affected. All the patients had cerebral or retinal ischemic symptoms, usually preceded by headache. Death occurred in a 13-year-old boy with intracranial arterial dissection and coarctation of the aorta. For the 17 remaining patients, the follow-up period ranged from 1 to 21 years. A complete or good clinical recovery occurred in 10 of the 11 patients with cervical arterial dissection but in only four of the seven with dissection of intracranial arteries. Recurrent arterial dissection occurred in two patients with cervical arterial dissections and in one patient with intracranial arterial dissection.

Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study.

OBJECTIVEIntracranial hypotension attributable to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches. The cause of these leaks is poorly understood, but it is likely multifactorial and may involve a primary connective tissue disorder. We undertook a study to estimate the contribution of systemic connective tissue disorders to the development of spontaneous spinal CSF leaks. METHODSWe examined a group of 18 consecutive patients with spontaneous spinal CSF leaks for features of a connective tissue disorder. RESULTSThe mean age of the 15 female patients and 3 male patients was 38 years (range, 22–55 yr). Seven patients (38%) demonstrated stigmata of a systemic connective tissue disorder, and three distinct types of disorders could be identified, as follows. 1) The association of spontaneous spinal CSF leaks and minor skeletal features of Marfan syndrome was noted for three patients. 2) Ehlers-Danlos syndrome Type II was noted for two patients. 3) Joint hypermobility associated with marked attenuation of the dorsal muscular fascia, precluding proper wound closure, was noted for two patients. In addition, isolated small-joint hypermobility was observed for five patients (28%). Slit-lamp ocular examinations, echocardiographic evaluations, histopathological examinations of skin biopsy specimens, and renal scanning did not reveal any other features of a systemic connective tissue disorder. CONCLUSIONFindings suggesting connective tissue disorders are common among patients with spontaneous spinal CSF leaks, and manifestations may be subtle. A variety of disorders can be identified, probably reflecting genetic heterogeneity. Problems with wound healing may occur as a result of the systemic nature of the underlying connective tissue disorder.

Diagnostic Criteria for Spontaneous Spinal CSF Leaks and Intracranial Hypotension

BACKGROUND AND PURPOSE:Comprehensive diagnostic criteria encompassing the varied clinical and radiographic manifestations of spontaneous intracranial hypotension are not available. Therefore, we propose a new set of diagnostic criteria. MATERIALS AND METHODS: The diagnostic criteria are based on results of brain and spine imaging, clinical manifestations, results of lumbar puncture, and response to epidural blood patching. The diagnostic criteria include criterion A, the demonstration of extrathecal CSF on spinal imaging. If criterion A is not met, criterion B, which is cranial MR imaging findings of spontaneous intracranial hypotension, follows, with at least one of the following: 1) low opening pressure, 2) spinal meningeal diverticulum, or 3) improvement of symptoms after epidural blood patch. If criteria A and B are not met, there is criterion C, the presence of all of the following or at least 2 of the following if typical orthostatic headaches are present: 1) low opening pressure, 2) spinal meningeal diverticulum, and 3) improvement of symptoms after epidural blood patch. These criteria were applied to a group of 107 consecutive patients evaluated for spontaneous spinal CSF leaks and intracranial hypotension. RESULTS: The diagnosis was confirmed in 94 patients, with use of criterion A in 78 patients, criterion B in 11 patients, and criterion C in 5 patients. CONCLUSIONS:A new diagnostic scheme is presented reflecting the wide spectrum of clinical and radiographic manifestations of spontaneous spinal CSF leaks and intracranial hypotension.