Bilateral subclavian steal: a review of an unusual twist in a common disorder

The authors present two cases of bilateral subclavian steal syndrome, a rare condition that does not commonly cause neurovascular symptoms. Lateralizing hemispheric events occur usually with carotid lesions. Vertebral-basilar insufficiency is three times more common in bilateral than in unilateral subclavian steal syndrome. Arm-exercise-induced brain-stem dysfunction is rare, and is seen only in bilateral subclavian steal syndrome.     

Carotid artery dissection at the cervical level presenting as unilateral paresis of lower cranial nerves

Internal carotid artery dissection at the cervical level is a known cause of stroke in young patients. The usual clinical presentation is strong ipsilateral cephalea and oculosympathetic paresis or ischemic symptoms in the affected artery. Paresis of the lower cranial nerves due to local compression in the space behind the parotid is rarely found and may complicate the diagnosis by leading physicians to look for anomalies in the vertebro-basilar territory. We present a patient with internal carotid artery dissection at the cervical level diagnosed by angiography. Symptoms at presentation were hemicranial cephalea accompanied by Villaret's syndrome. We point out the importance of keeping this diagnostic possibility in mind when looking for the etiology of subacute paresis of the lower cranial nerves.     

Optimized method for the determination of organophosphorus pesticides in meat and fatty matrices

A female patient with an ischaemic focal lesion in the non-dominant cerebral hemisphere due to stenosis of the right carotid artery is presented. Besides a mild left hemiparesis the clinical symptomatology included a pronounced hemi-neglect syndrome in the form of unilateral limb akinesia, a failure to respond to stimuli presented to the left of the midline as well as anosognosia and anosodiaphoria. Despite the 6-week rehabilitation training, the neglect syndrome was rather stabilized with only a slight tendency for improvement.     

Relationship between genotype of angiotensin converting enzyme gene and left ventricular hypertrophy in Chinese hypertensives

Incontinentia pigmenti is an uncommon neurocutaneous syndrome characterised by skin lesions, dental and ocular abnormalities and central nervous system involvement. We report the cranial MRI findings in two sisters with this condition. These include hypoplasia of the corpus callosum, enlargement of the lateral ventricles and periventricular white-matter lesions. One girl also had unilateral microphthalmia and rostral agenesis of the corpus callosum, a feature not previously described.     

Long-term astigmatic changes after clear corneal cataract surgery

Ramsay Hunt's syndrome is an infectious cranial polyneuropathy caused by varicella zoster, the herpetic virus that also causes chickenpox and shingles. Its symptoms include facial paralysis, ear pain, and an auricular rash. Oral lesions are also present in most cases. This syndrome can affect any cranial nerve and usually affects multiple nerves, causing central, cervical, and peripheral effects. This article reports the case of a 35-year-old white female who was treated by the oral surgery service of a large urban hospital, after first reporting to the emergency clinic. Her reported symptoms of unilateral left-side facial paralysis, auricular pain, and trigeminal hyperesthesia were confirmed by clinical examination. An initial short low-dose steroid regimen was unsuccessful. A second daily dosage of 50 mg of prednisone was successful in 21 days. No permanent sequelae were evident or reported after treatment.     

Low incorporation of soleus tendon: a potential diagnostic pitfall on MR imaging

Head pain is one of the main presenting symptoms of internal carotid artery (ICA) dissection, usually in association with ischemic and/or local signs such as Horner's syndrome, lower cranial nerve palsies, or tinnitus. In rare cases, head pain remains isolated and mimics other conditions. We report a patient who suffered isolated prolonged orbital pain as the only sign of intrapetrous ICA dissection. Early recognition of such unusual facial pain may be crucial in decreasing the risk of secondary cerebral or retinal ischemia.     

Basal skull fracture with traumatic polycranial neuropathy and occluded left carotid artery: significance of fractures along the course of the carotid artery

A patient who survived with traumatic multiple cranial nerve palsies and occluded internal carotid artery associated with a basal skull fracture that involved the carotid canal is described. A literature review indicates that a wide variety of injuries can occur to the carotid artery as it passes through a fractured skull base. Basal fractures involving the course of the carotid artery reflect a significant risk of carotid injury.     

Sequential injections of amobarbital sodium and lidocaine for provocative neurologic testing in the external carotid circulation

PURPOSE: To present the results of a protocol for embolization in the external carotid territory that includes provocative testing with amobarbital sodium and lidocaine. METHODS: Provocative testing with sequential intraarterial injections of amobarbital sodium and lidocaine was done before embolization of 66 vascular pedicles in 26 patients with the following pathologic disorders: 8 meningiomas, 7 paragangliomas, 5 dural arteriovenous fistulas, 3 angiofibromas, 1 endolymphatic sac carcinoma, 1 metastatic renal carcinoma, and 1 Rendu-Osler-Weber syndrome. Findings during provocative testing and any postembolization deficits were recorded. RESULTS: Of the 66 vascular pedicles tested, only 1 showed positive findings on an amobarbital test, in which the patient had transient numbness in the contralateral leg. Results of lidocaine tests were positive, in 7 cases, in which cranial nerve deficits were suspected on the basis of anatomic findings. All deficits resolved within 15 minutes. If results of amobarbital or lidocaine tests were positive, large particles or coils were used for embolization. Otherwise, small particles or liquid agents were used. There were no complications after embolization. CONCLUSIONS: Provocative testing with amobarbital can reveal dangerous anastomoses. Testing with lidocaine can show vascular supply to the cranial nerves. With the use of appropriate protocols, embolization in the external carotid territory can be done with minimal risk.     

Detection of restricted junctional diversity of peripheral T cells in SLE patients by spectratyping

Three patients presented with unilateral sensori-neural hearing disturbance as the initial symptom of cerebellar tumors: a 19-year-old female with a medulloblastoma (Case 1), a 45-year-old male with a cerebellar low-grade glioma (Case 2), and a 49-year-old female with a cerebellaer hemangioblastoma (Case 3). In Cases 1 and 2, the whole length of the eight cranial nerve was intact according to magnetic resonance imaging and intraoperative findings. In Case 3, the intracerebellar tumor had bulged into the cerebellopontine cistern, compressing the eighth cranial nerve near the brainstem. Auditory evoked brainstem responses showed only the first wave in all three patients, and the following waves could not be discriminated. Unilateral sensori-neural hearing disturbance occurs very rarely in patients with intramedullary cerebellar lesions because the auditory neural pathway is bilaterally innervated. Intramedullary tumors may cause unilateral sensori-neural hearing disturbance by infiltrating or causing edematous changes of the eighth cranial nerve or the cochlear nucleus in the brainstem, or by compressing the nerve in the cistern. The symptoms are the same as those of acoustic neurinoma, so intramedullary cerebellar tumors should be considered in the differential diagnosis of unilateral sensorineural hearing disturbance.     

Giant intracavernous aneurysm: rare cause of isolated sixth cranial nerve palsy in a child

A boy, aged 7 1/2 years, developed a sixth cranial nerve palsy for which no cause was found until 8 1/2 years later, when he developed a rapidly progressive ophthalmoplegia. A giant intracavernous aneurysm was then demonstrated on carotid angiography and successfully treated by carotid ligation. Radioisotope techniques were of value in diagnosis and follow-up.     

Asymptomatic carotid artery stenosis and stroke in patients undergoing cardiopulmonary bypass

PURPOSE: This study was undertaken to assess the natural history of carotid artery stenosis in patients undergoing cardiopulmonary bypass (CPB) at a Veterans Administration Medical Center. METHODS: Between January 1989 and August 1993, all patients undergoing CPB were offered preoperative carotid artery ultrasound screening as part of an investigative protocol. Patients were monitored in-hospital for the occurrence of perioperative neurologic deficit. RESULTS: A total of 582 patients underwent carotid artery ultrasound screening. Greater than 50% stenosis or occlusion of one or both internal carotid arteries was present in 130 patients (22%), with 80% or greater stenosis or occlusion of one or both arteries present in 70 patients (12%). In-hospital stroke or death occurred in 12 (2.1%) and 36 (6.2%) patients, respectively. Of the 12 strokes, five were global and seven were hemispheric in distribution. Of the five patients who had global events, none had evidence of carotid artery stenosis. However, of the seven patients who had hemispheric events, five had significant 50% or greater stenosis or occlusion of the internal carotid artery ipsilateral to the hemispheric stroke. Therefore the presence of carotid artery stenosis or occlusion was significantly associated with hemispheric stroke (no stenosis 0.34% vs stenosis 3.8%; p = 0.0072). Furthermore, the risk of hemispheric stroke in patients with unilateral 80% to 99% stenosis, bilateral 50% to 99% stenosis, or unilateral occlusion with contralateral 50% or greater stenosis was 5.3% (4 of 75). No strokes occurred in patients with unilateral 50% to 79% stenosis (n = 52). CONCLUSIONS: It is concluded that carotid atherosclerosis is a risk factor for hemispheric stroke in patients undergoing CPB.     

Unilateral internal carotid arterial occlusion: special considerations

Cases of patients with unilateral internal carotid arterial occlusion and contralateral internal carotid arterial stenosis are reviewed. Forty-two percent presented with a fixed neurological deficit. The deficit was referable to the side of occlusion in 92% and to the side of stenosis in 8%. Eleven percent had a neurological complication following carotid endarterectomy on the side of the stenotic lesion. The neurological complication was referable to the side of stenosis in 67% and to the side of occlusion in 33%. Patients have been followed for an average of 19 months and have not developed any additional TIA's or strokes in the followup period. There may be a role for an extracranial-intracranial bypass (ECIC) on the occluded side prior to an endarterectomy on the stenotic side if a poor collateral situation exists. An ECIC should be done in patients who remain symptomatic following carotid endarterectomy on the stenotic side. These data do not support doing ECIC in asymptomatic patients with unilateral carotid arterial occlusion.     

Glomus tumor of the temporal bone

A case story describing the typical symptoms and course of a glomus tumour of the temporal bone is presented. The most frequent symptoms are pulsatile tinnitus, unilateral hearing loss, aural fullness and paresis of the vagal nerve or other lower cranial nerves. The tumour is frequently visible by otoscopy. Despite being histologically benign, the tumour is infiltrative and may affect the surrounding cranial nerves or spread into the cranial cavity. The early signs and findings are vague. Since the sequelae are fewer when the tumour is treated while it is small, an increased awareness will be of benefit to the patients.     

An analysis of slow phase velocity on congenital nystagmus-asymmetrical responses of optokinetic stimulation

Persistence of the hypoglossal artery is an unusual congenital abnormality of the carotid arterial system, and the simultaneous occurrence of atheromatous disease in the internal carotid artery and persistent hypoglossal artery is even more uncommon. Carotid surgery in this situation is challenging, and the surgeon must be aware of potential inherent pitfalls. A 74-year-old woman with asymptomatic stenosis of both internal carotid and hypoglossal arteries associated with occlusion of the contralateral internal carotid artery underwent successful revascularization. The anatomy of this unusual lesion and the surgical strategies, which included cranial nerve dissection and mandibular subluxation, are reviewed.     

Agenesis of the internal carotid artery associated with an aneurysm of the anterior communicating artery

We report a case of agenesis of the internal carotid artery which was revealed by a subarachnoid hemorrhage. Angiography showed a ruptured aneurysm of the anterior communicating artery and unilateral absence of the left internal carotid artery. Both the left anterior and middle cerebral arteries were perfused from the right carotid artery via the anterior communicating artery. Absence of the left carotid canal was proved on bone CT. Such an association is discussed. An hemodynamic stress on a congenital defect of the cerebral arterial wall could be the origin of the aneurysm development.     

Isolated vagal nerve palsy associated with a dissection of the extracranial internal carotid artery

A 40-year-old man had paralysis of the right vocal cord. Imaging showed a dissection of the extracranial internal carotid artery, and physical examination disclosed paresis of the right side of the soft palate. To our knowledge, this is only the second report of carotid dissection presenting as an isolated vagal neuropathy. Most often, multiple lower cranial nerves are involved. The CT, MR imaging, and MR angiographic findings are presented and the topic is reviewed.     

Extracranial carotid aneurysms

Extracranial carotid artery aneurysms occur very seldom. Here will be reported on 15 aneurysms (11 arteriosclerotic, 3 false and 1 dissecting aneurysm). Therapy of choice during the second half of this period was aneurysm exstirpation and PTFE interposition, twice with reinsertion of the external carotid artery. No patient died perioperatively, one permanent and one transient ischemic neurologic deficit occurred. Three lesions of the cranial nerves recovered completely. As to avoid cerebral embolisation each extracranial carotid artery aneurysm should be operated on if possible. This can be managed with tolerable risk and represents an effective embolic prophylaxis.     

Intracranial hemorrhage observed in the cases of cervical internal carotid artery occlusion associated with moyamoya disease-like vessels]

We report on two peculiar cases of intracranial hemorrhage due to the rupture of moyamoya disease-like vessels associated with unilateral internal carotid occlusion at its origin. The first case is 44-year-old male showing intraventricular hemorrhage associated with right internal carotid occlusion. The second case is 58-year-old female presenting sub-arachnoid hemorrhage associated with left internal carotid occlusion. Although both cases showed the unique appearance of collateral flow resembling moyamoya disease, they were not classified as moyamoya disease nor unilateral Moyamoya-like state. Vascular abnormality such as cerebral aneurysm and arteriovenous malformation were not at all detected. The etiology of hemorrhage for both cases is presumed as the rupture of moyamoya disease-like vessels, however, it is unable for us to determine why the one case showed IVH, yet the other showed SAH. The two cases showed hypoperfusion of ipsilateral cerebral hemisphere on SPECT that followed by the external-internal carotid revascularization surgery in the chronic stage. Post operative study proved the improvement of the CBF reserve. Nevertheless, whether the reconstructive vascularization prevent an occurrence of rebleeding needs the longer observation and the accumulation of the resembling cases.     

Craniofacial and dental characteristics of Silver-Russell syndrome

We found significant differences in a craniometric, cephalometric, and dental study of 19 Silver-Russell syndrome patients (13 without growth hormone treatment) with appropriate controls. Although head circumference was normal for age, head length was increased, while cranial and facial widths and facial heights were reduced. Posterior facial height, posterior cranial base length, cranial base height, and mandibular body size were significantly smaller than in healthy children of the same height. Articulatory speech disorders were common. Enamel defects pointed to an early prenatal insult. Delayed dental age and small mandibular and cranial base dimensions support the possibility of physiological growth hormone deficiency in many Silver-Russell syndrome children; however, facial soft tissue structures were strikingly different from those observed in classical growth hormone deficiency.     

Dumbbell type jugular foramen meningioma extending both into the posterior cranial fossa and into the parapharyngeal space: report of 2 cases with vascular reconstruction

Two cases with huge dumbbell type jugular foramen meningioma with extension into the parapharyngeal space are reported. A well co-ordinated surgical strategy for total resection to this high risk tumour with neurosurgeons, otolaryngologists and plastic surgeons is mandatory to minimise operative complications. Both of our patients presented with a cervical mass and lower cranial nerve palsies, and had huge dumbbell type masses extending from the posterior cranial fossa through the jugular foramen to the parapharyngeal space, encasing the cervical internal carotid artery. Gross total resection of the tumours was successfully achieved by basically a 2-stage operation. In the first stage, posterior fossa tumours were removed by the transjugular approach, combined with the petrosal approach in one case. In the second stage, cervical tumours were removed along with the cervical carotid artery by the transcervical and/or transmandibular approach, followed by vascular reconstruction from the ipsilateral carotid artery to the middle cerebral artery using saphenous vein graft. From these experiences, we recommend this 2-stage operation for large dumbbell type meningiomas extending to the infratemporal/parapharyngeal space. The intracranial tumour is removed at the first operation. The extracranial portion is resected at the second, and if necessary, the involved cervical carotid artery is resected and simultaneous revascularisation using saphenous vein graft is performed with a vascularised free muscle graft. This strategy could maximise the functional preservation on the one hand, and minimise the surgical risk, such as postoperative infection, on the other.