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.     

Isolated cranial nerve palsies due to brainstem lesions.

Isolated cranial nerve palsies are often attributed to lesions of the respective nerves along their extraaxial courses. There are a significant number of reports of individual patients with cranial nerve palsies, mostly of the 3rd and 6th nerves, as the sole manifestation of brainstem lesions proven by magnetic resonance imaging (MRI) or computer-assisted tomography (CT). An intraaxial basis may still be underestimated if based on MRI only, as electrophysiological abnormalities indicating brainstem lesions (masseter reflex, blink reflex, DC electrooculography) may be independent from MRI-documented morphological lesions. This article reviews the evidence that ischemic and demyelinating brainstem lesions are an important and underestimated cause of clinically isolated cranial nerve palsies. Especially in middle-aged and elderly people with 3rd and 6th nerve palsies, small pontine and mesencephalic infarctions seem to be more frequent than small-vessel ischemic infarctions of the extraaxial nerves.     

Spontaneous internal carotid artery dissection with lower cranial nerve palsy

BACKGROUND: Typical presentation of spontaneous internal carotid artery (ICA) dissection is an ipsilateral pain in neck and face with Horner's syndrome and contralateral deficits. Although rare, lower cranial nerve palsy have been reported in association with an ipsilateral spontaneous ICA dissection. CASE STUDIES: We report three new cases of ICA dissection with lower cranial nerve palsies. RESULTS: The first symtom to appear was headache in all three patients. Examination disclosed a Horner's syndrome in two cases (1 and 2), an isolated XIIth nerve palsy in two patients (case 1 and 3) and IX, X, and XIIth nerve palsies (case 2) revealing an ipsilateral carotid dissection, confirmed by MRI and angiography. In all cases, prognosis was good after a few weeks. CONCLUSIONS: These cases, analysed with those in the literature, led us to discuss two possible mechanisms: direct compression of cranial nerves by a subadventitial haematoma in the parapharyngeal space or ischemic palsy by compression of the ascending pharyngeal artery.     

Isolated cranial nerve palsy secondary to carotid dissection

Cranial nerve palsy has a variety of causes such as cerebral ischemia, nerve ischemia in diabetes, infectious and noninfectious meningitis, subarachnoid hemorrhage, malignant tumors of the skull base, neck, or upper mediastinum, aortic aneurysm, surgery of the thyroid,and many more. We report two cases of spontaneous carotid dissections leading to cranial nerve palsies, which is an uncommon cause of isolated cranial nerve palsies.ICA dissection must therefore be included in the differential diagnosis of lower cranial nerve palsy and should be assessed by duplex ultrasound and MRI as is demonstrated in our cases.     

Cranial nerve palsies in spontaneous carotid artery dissection.

Two patients had isolated unilateral cranial nerve palsies due to spontaneous internal carotid artery (ICA) dissection without ischaemic cerebral involvement. One had acute glossopharyngeal and vagal, the other isolated hypoglossal nerve palsy. Reviewing all reported cases of angiographically confirmed ICA dissection in the literature, 36 additional cases with unequivocal ipsilateral cranial nerve palsies were analysed. While an isolated palsy of the IXth and Xth has not been reported previously, palsies of the XIIth nerve or the IXth to XIIth nerves were most frequently found. In these patients, lower cranial nerve palsies are probably the result of compression by an enlarging ICA due to mural haematoma. Symptoms and signs indicative of carotid dissection were concurrently present only in some reported cases. This raises the question of unrecognised carotid dissection as a cause of isolated cranial nerve palsies. When the dissection occurs in the subadventitial layer without relevant narrowing of the arterial lumen and when an aneurysm is thrombosed, angiography does not reliably yield the diagnosis. Therefore, carotid dissection might have been underestimated as a cause of isolated lower cranial nerve palsies before the advent of MRI. MRI demonstrates directly the extension of the wall haematoma in the axial and longitudinal planes. Some arteriopathies such as fibromuscular dysplasia and tortuosity make a vessel predisposed to dissection.     

Familial recurrent cranial nerve palsies

Family cases of recurrent cranial nerve palsies are seldom reported. This paper presents a family with recurrent facial and ocular nerve palsies in 2 brothers. Their father and his sister had Bell's palsies. Examinations provided no explanation. Six previous reports of families with recurrent cranial nerve palsies are summarized. The pedigrees speak in favour of an autosomal dominant mode of inheritance of predisposing factors. The pathogenetic mechanism might be vascular or autoimmune, but is still unknown.     

Recurrent cranial nerve palsies, midbrain infarction and hydrocephalus due to megadolichobasilar artery

A 67-year-old man presented four recurrent, alternating facial palsies, two right abducens palsies and eventually a right extrinsic third nerve palsy due to brain-stem infarction in a 37-year time-span. Neuroradiological examinations showed hydrocephalus and an elongated, tortuous, estasic basilar artery. This patient presented in his lifetime the whole clinical spectrum of the dolichoectasic basilar artery complications.

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Sommario Viene descritto un paziente di 67 anni che, dai 30 anni, presentò 4 paralisi ricorrenti, alternanti del nervo facciale, 2 paralisi del nervo abducente destro ed infine una paralisi estrinseca del terzo nervo cranico di destra secondaria ad infarto mesencefalico. Gli esami neuroradiologici evidenziarono un'arteria basilare allungata, tortuosa, ectasica ed idrocefalo. Tale paziente ha presentato nella sua vita l'intero spettro clinico delle complicazioni legate alla megadolicobasilare.

     

Facial nerve palsy secondary to internal carotid artery dissection

We report facial palsy as the sole cranial neuropathy complicating an ipsilateral internal carotid artery dissection. A previously healthy 44-year-old man developed retro-orbital and temporal headache with associated nausea while engaged in modest physical exercise. On the following morning he noticed a left ptosis and miotic pupil. One week later he woke with a left facial weakness. On the same day he had a 90-minute episode of expressive dysphasia. Magnetic resonance imaging and angiography demonstrated left internal carotid artery dissection. The temporal association between our patient's facial nerve palsy and typical features of spontaneous internal carotid artery dissection suggests a common aetiology. We suggest that involvement of the VII cranial nerve in isolation followed disruption of an anomalous nutrient artery. The delay in clinical manifestation may imply extension of the dissection.     

Optic nerve MRI enhancement in posterior ischaemic optic neuropathy due to internal carotid artery dissection

Posterior ischaemic neuropathy (PION) is characterized by infarction in the retrobulbar optic nerve. A 73-year-old man suddenly experienced blurred vision in his left eye and intermittent weakness in his right hand. He had visual defects of superior lateral quarter and inferior medial quarter areas in the left eye. Gadolinium-enhanced magnetic resonance imaging (MRI) revealed segmental enhancement in the left optic nerve. A cerebral angiogram showed a left internal carotid dissection (ICD). He did not have fever, and his laboratory and cerebrospinal fluid tests were normal. These findings were suggestive of PION associated with ICD. No reports of PION caused by ICD has been reported and his is the first case in which MRI showed optic nerve enhancement due to ICD.     

Upper cranial nerve palsy resulting from spontaneous carotid dissection

Introduction Upper cranial nerve palsy has a variety of causes such as cerebral and nerve ischemia, diabetes, infectious and non–infectious meningitis, subarachnoid hemorrhage and intracranial aneurysm. Case 1 A 45–year–old man suffered from holocephalic headaches and a rightsided neck pain for two weeks. He presented to our emergency department because of a sudden ptosis of the right eye. On admission neurological examination revealed a right sided Horners syndrome and hypesthesia of the right side of the face. Magnetic resonance angiography identified a circumscribed dissection of the right extracranial internal carotid artery originating from the carotid bifurcation. Conventional angiography 2 weeks later showed a nearly recanalized artery. Case 2 A 55–year–old previously healthy man without cardiovascular risk factors developed right sided neck pain when loading a seeder with several sacks of crop. A few hours later he noticed a left–sided weakness. On admission a severe left sided hemiparesis and a mild neglect were present. Duplex sonography revealed a right–sided distal internal carotid artery (ICA) occlusion. The next morning the patient complained of double vision; he had a right–sided pupil–sparing oculomotor palsy. The diagnosis of ICA dissection was confirmed by conventional angiography, at that time showing a partially recanalized ICA without involvement of the cavernous region by the dissection. Conclusion ICA dissection must be included in the differential diagnosis of upper cranial nerve palsy and should be assessed by duplex ultrasound and magnetic resonance imaging. A possible explanation is nerve ischemia due to a transient or permanent interruption of the blood supply by compression of the vasa nervorum originating from the intracranial carotid artery.     

Lower cranial nerve palsy produced by internal carotid artery dilatation. Report of two cases

We report two cases of lower cranial nerve palsies (XII in case 1, IX–X–XII in case 2) associated with abnormalities of the internal carotid artery at the base of the skull. In case 1 a limited dissection of the carotid wall produced both paresis of the hypoglossal nerve and Horners syndrome by compression of the nerve trunk against the base of the skull and stretching of the periarterial sympathetic fibres respectively. In case 2 we speculate that a narrow angled kinking of the internal carotid artery may have damaged cranial nerves IX, X and XII by interfering with the blood supply to the nerve trunks. In both cases the outcome was favorable with almost complete regression of the initial symptoms. We conclude that the association between lower cranial nerve disturbances and internal carotid artery abnormalities is probably more common than was thought. We suggest that the pathogenesis of the damage to the cranial nerves may differ from one case to the next.

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Riassunto Gli Autori riportano due casi di paralisi dei nervi cranici bulbari (XII nel caso 1, IX–X–XII nel caso 2) causate da anomalie dell'arteria carotide interna alla base cranica. Nel caso 1 la paresi del XII nervo cranico, associato con sindrome di Claude Bernard Horner ipsilaterale, è state determinata da un anuerisma dissecante della carotide con conseguente compressione del tronco nervoso contro la base cranica e stiramento delle fibre simpatiche periarteriose. Nel caso 2 viene ipotizzato che la lesione del IX, X e XII nervo cranico sia stata di origine ischemica per la presenza di un'ansa ad angolo acuto dell'arteria carotide interna. In entrambi i casi il decorso è stato favorevole, con regressione pressochè completa dei sintomi iniziali. L'associazione fra anomalie della carotide interna e disturbi dei nervi cranici bulbari è probabilmente più frequente di quanto si ritenesse in precedenza e la patogenesi della lesione nervosa può essere variabile da caso a caso.

     

Isolated cranial nerve palsies in multiple sclerosis

During a 10 year period 24 patients with definite multiplesclerosis with isolated cranial nerve palsies were studied (third andfourth nerve: one patient each, sixth nerve: 12 patients, seventhnerve: three patients, eighth nerve: seven patients), in whom cranialnerve palsies were the presenting sign in 14 and the only clinical signof an exacerbation in 10 patients. MRI was carried out in 20 patientsand substantiated corresponding brainstem lesions in seven patients(third nerve: one patient, sixth nerve: four patients, eighth nerve:two patients). Additional abnormal findings of electro-oculography, ormasseter reflex, or blink reflex, or combinations of these were foundin 20 patients and interpreted in favour of a brainstem lesion at thelevel of the respective cranial nerve. In 11 of 14 patients withisolated cranial nerve palsies as the presenting sign of multiplesclerosis, dissemination in space was documented by MRI, and in theremaining three by evoked potentials. In patients with multiplesclerosis with isolated cranial nerve palsies, MRI is the mostsensitive method of documenting dissemination in space andelectrophysiological testing the most sensitive at disclosing brainstem lesions.