Cases reported "Cranial Nerve Diseases"

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1/15. Spontaneous carotid dissection presenting lower cranial nerve palsies.

    Cranial nerve palsy in internal carotid artery (ICA) dissection occurs in 3--12% of all patients, but in 3% of these a syndrome of hemicranias and ipsilateral cranial nerve palsy is the sole manifestation of ICA dissection, and in 0.5% of cases there is only cranial nerve palsy without headache. We present two cases of lower cranial nerve palsy. The first patient, a 49-year-old woman, developed left eleventh and twelfth cranial nerve palsies and ipsilateral neck pain. The angio-RM showed an ICA dissection with stenosis of 50%, beginning about 2 cm before the carotid channel. The patient was treated with oral anticoagulant therapy and gradually improved, until complete clinical recovery. The second patient, a 38-year-old woman, presented right hemiparesis and neck pain. The left ICA dissection, beginning 2 cm distal to the bulb, was shown by ultrasound scanning of the carotid and confirmed by MR angiogram and angiography with lumen stenosis of 90%. Following hospitalisation, 20 days from the onset of symptoms, paresis of the left trapezius and sternocleidomastoideus muscles became evident. The patient was treated with oral anticoagulant therapy and only a slight right arm paresis was present at 10 months follow-up. Cranial nerve palsy is not rare in ICA dissection, and the lower cranial nerve palsies in various combinations constitute the main syndrome, but in most cases these are present with the motor or sensory deficit due to cerebral ischemia, along with headache or Horner's syndrome. In the diagnosis of the first case, there was further difficulty because the cranial nerve palsy was isolated without hemiparesis, and the second case presented a rare association of hemiparesis and palsy of the eleventh cranial nerve alone. Compression or stretching of the nerve by the expanded artery may explain the palsies, but an alternative cause is also possible, namely the interruption of the nutrient vessels supplying the nerve, which in our patients is more likely.
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2/15. A clinicopathological study of a patient with anti-Hu-associated paraneoplastic sensory neuronopathy with multiple cranial nerve palsies.

    Only a few cases of paraneoplastic neurologic syndrome with multiple cranial palsies have been reported. This is the case report of a patient with small-cell lung cancer and a high titer of anti-Hu antibodies who developed a tonic left pupil and multiple cranial nerve palsies, including palsies of the left fifth through tenth nerves and both twelfth nerves, as in Garcin syndrome showing at least more than seven ipsilateral cranial nerve palsies, in the course of paraneoplastic sensory neuronopathy (PSN). Pathologic examination revealed no metastasis or direct invasion of malignancy with gliosis and perivascular inflammation throughout the brainstem, indicating paraneoplastic encephalomyelitis (PEM). The numbers of EBM11 cells (probably reactive microglia), CD8 cells, and CD4 cells increased. Intracellular adhesion molecule-1 and lymphocyte function associated molecule-1 were expressed intensely on the endothelia of microvessels and were found to have infiltrated mononuclear cells around microvessels in the brainstem. Multiple cranial nerve palsies and their effects including the tonic pupil are likely due to the paraneoplastic effect of the primary systemic malignancy.
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3/15. Percutaneous intracranial stent placement for aneurysms.

    OBJECT: Intracranial stent placement combined with coil embolization is an emerging procedure for the treatment of intracranial aneurysms. The authors report their results using intracranial stents for the treatment of intracranial aneurysms. methods: A prospectively maintained database was reviewed to identify all patients with intracranial aneurysms that were treated with intracranial stents. Ten lesions, including eight broad-based aneurysms and two dissecting aneurysms, were treated in 10 patients. Four lesions were located in the cavernous segment of the internal carotid artery, two at the vertebrobasilar junction, two at the basilar trunk, one at the basilar apex, and one in the intracranial vertebral artery. Attempts were made to place stents in 13 patients, but in three the stents could not be delivered. Altogether, intracranial stents were placed in 10 patients for 10 lesions. Results that were determined to be satisfactory angiographically were achieved in all 10 lesions. Two patients suffered permanent neurological deterioration related to stent placement. In two patients, the aneurysm recurred after stent-assisted coil embolization. In one case of recurrence a second attempt at coil embolization was successful, whereas in the second case of recurrence parent vessel occlusion was required and well tolerated. CONCLUSIONS: Intracranial stents can be a useful addition to coil embolization by providing mechanical, hemodynamic, and visual benefits in the treatment of complex, broad-based aneurysms.
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4/15. Progressive bulbar dysfunction caused by a predominantly venous vascular malformation of the medulla oblongata.

    We describe a 58-year-old patient with a rare predominantly venous vascular malformation of the medulla oblongata, which caused a progressive bulbar dysfunction consisting of hiccoughs, dysphagia, hoarseness, dysarthria, gait ataxia and dysuria over a period of 11 months. On autopsy, a large dilated vein with focal marked intimal fibroelastic thickening was present on the ventral surface of the medulla. Microscopically, moderate proliferation of capillaries and veins was observed which was confined primarily to the medulla. The veins displayed abnormal dilatation and tortuosity; prominent thickening of vessel walls was also present in the veins and capillaries. The venous abnormalities were prominent in the parenchyma of the medulla, but much less apparent in its subarachnoid space. Multifocal neuronal loss and gliosis were observed, most prominently in the inferior olives, hypoglossal, dorsal vagal and ambiguus nuclei. The histopathologic findings suggested that abnormal venous drainage within the parenchyma of the medulla was the most critical factor for the pathogenesis of this patient's neurologic symptomatology.
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5/15. Use of the Amplatzer vascular plug as an anchoring scaffold for coil-mediated parent vessel occlusion: technical case report.

    OBJECTIVE: Parent vessel sacrifice is a useful treatment strategy for fusiform intracranial aneurysms. Originally performed using the detachable silicone balloon, endovascular arterial occlusion is currently achieved using coils, a process which can be limited by coil mass migration. methods: We demonstrate the use of the Amplatzer vascular plug as a fixed anchor within the target parent vessel to facilitate coil-mediated occlusion, especially in vascular segments not encased by a bony canal. The technique was used successfully in two patients: a 90-year-old woman presenting with IIIrd and VIth cranial nerve palsy from a fusiform left cavernous internal carotid aneurysm and a 44-year-old man with distal thromboemboli from a fusiform dissecting-type right vertebral artery involving the origin of the posterior inferior cerebellar artery. RESULTS: Both patients were treated successfully with proximal parent vessel occlusion using coils after deployment of an Amplatzer vascular plug proximal to the target lesion. With the Amplatzer device acting as a fixed anchor in the parent vessel, coils were deployed proximally in a compact configuration. After deployment of the vascular plugs and coils, hermetic occlusion of the parent vessel was documented angiographically. CONCLUSION: The Amplatzer vascular plug can facilitate coil occlusion of large cervical vessels by acting as a focal coil and embolic material immobilizer, which can prevent coil mass migration and lead to improved packing density.
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6/15. Collet-Sicard syndrome caused by a coiling of the internal carotid artery.

    A Collet-Sicard syndrome was observed in a 53-year-old patient with a coiling of the left internal carotid artery just below the skull base. Although impairment of cranial nerves by tortuous vessel compression has frequently been reported, a combined palsy of the last four cranial nerves related to such a mechanism has never been described before.
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7/15. Isolated trigeminal sensory loss secondary to a distal anterior inferior cerebellar artery aneurysm: case report.

    A previously healthy 25-year-old woman suddenly developed right-sided facial numbness and a headache. The neurological examination was within normal limits with the exception of meningismus and right-sided facial sensory loss. A computed tomographic scan and a magnetic resonance imaging study demonstrated an acute hematoma in the right cerebellopontine angle. A 4-vessel cerebral angiogram revealed no abnormalities. Posterior fossa exploration disclosed a large, partially thrombosed, fusiform anterior inferior cerebellar artery aneurysm, which indented the pons at the trigeminal root entry zone. The aneurysm was excised, and the patient made an excellent recovery. She was left with a persistent trigeminal sensory deficit. Anterior inferior cerebellar artery aneurysms are rare lesions that generally present with a cerebellopontine angle syndrome; occasionally, facial sensory loss is also a feature. Isolated trigeminal sensory findings, as illustrated in this case, are extremely unusual in posterior fossa vascular lesions.
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8/15. mobius syndrome and transposition of the great vessels.

    A case of mobius syndrome was associated with transposition of the aorta and pulmonary artery, as well as acheiria. This combination of anomalies supports the hypothesis that mobius syndrome is caused by an intrapartum insult during the fourth to seventh week of gestation and is consistent with the vascular theory of embryopathogenesis.
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9/15. Microsurgical technique in the treatment of vascular compression of cranial nerves in the posterior fossa.

    The authors report on 2 cases: cerebello-pontine syndrome and a trigeminal neuralgia with homolateral facial spasm. In both patients the clinical syndrome was thought to be of vascular origin and the vessels accompanying the nerves showed to be tightly anchored to them by means of arachnoid bridges. microsurgery allowed for performing section of the arachnoid bridges in order to prevent the nerves from the continuous microtrauma due to the vascular pulsation, this being the pathogenic mechanism responsible for the suffering of the nerves themselves.
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10/15. Bilateral trigeminal amyloidoma: an unusual case of trigeminal neuropathy with a review of the literature. Case report.

    Isolated amyloidomas may, albeit rarely, involve the central nervous system. There are three previous reports of amyloidomas that involved the gasserian ganglion and caused unilateral trigeminal neuropathies. The authors report the case of a 49-year-old woman with apparently isolated amyloidomas that caused slowly progressive bilateral trigeminal neuropathies. magnetic resonance imaging of the brain revealed mild swelling of the left trigeminal nerve within the cavernous sinus and uniform enhancement with gadolinium throughout the length of the nerve. At craniotomy, the trigeminal nerve and ganglion were observed to be infiltrated by a tumor-like mass. biopsy showed extensive infiltration of the nerve and ganglion by amyloid. Immunocytochemical studies of the amyloid were negative for immunoglobulins, kappa and lambda light chains, beta-amyloid A4 protein, transthyretin, beta 2-microglobulin, cystatin c, and gelsolin, but weak focal immunoreactivity with antiamyloid AA antibody was seen in the amyloid in vessels and in some intraneural deposits. Extensive investigations failed to reveal evidence of either systemic amyloidoses or an underlying inflammatory disorder or malignancy.
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