Ophthalmoplegic migraine: from questions to answers.

INTRODUCTION: The International Classification of Headache Disorders classifies ophthalmoplegic migraine (OM) under "cranial neuralgias and central causes of facial pain." OM is diagnosed when all the following criteria are satisfied: A. At least two attacks fulfilling criterion B. B. Migraine-like headache accompanied or followed within four days of its onset by paresis of one or more of the III, IV and/or VI cranial nerves. C. Parasellar orbital fissure and posterior fossa lesions ruled out by appropriate investigations. In children the syndrome is rare and magnetic resonance (MR) shows strongly enhancing thickened nerve at the root entry zone (REZ). METHOD: The authors review the literature focusing on pathogenesis theories. RESULTS: The authors suggest that ischemic reversible breakdown of the blood-nerve barrier is the most probable cause of OM and to include MR findings in the hallmarks of the disease. CONCLUSION: OM is the same disease in adulthood and childhood, even if in adults the MR imaging findings are negative. In the authors' opinion, OM should be classified as migraine.

Cavernous sinus apoplexy presenting isolated sixth cranial nerve palsy: case report.

The clinical and radiological findings of a patient affected by apoplexy of an adenoma located within cavernous sinus and presenting isolated Cranial Nerve (CN) VI palsy are reported. The differential diagnosis with other pathologies is debated, and pathogenesis of isolated abducens nerve palsy is investigated. The pertinent literature of this uncommon case of apoplexy is reviewed. A 59-year-old female presented sudden appearance of CN VI palsy associated to violent headache. Endocrinological assessment did not show pituitary insufficiency; MRI depicted an adenoma located within the left CS. She underwent an endoscopic endonasal procedure with extensive opening of the cavernous sinus, occupied by an ischemic adenoma. Afterward, neurological symptoms promptly resolved and the patient was discharged after 5 days. At 3 months follow-up MRI radical tumor removal was assessed, and the patient resulted neurologically intact. The review of the pertinent literature shows that apoplexy of pituitary tumor exclusively located within the Cavernous Sinus is a very uncommon event. The distinctive clinical presentation is represented by the sudden abducens nerve palsy and the absence of pituitary insufficiency and it could be explained by the vulnerability of the nerve along its course within the cavernous sinus, and by the sparing of the sellar content by the adenoma.

Wall motion at 4D-CT angiography and surgical correlation in unruptured intracranial aneurysms: a pilot study.

BACKGROUND: Unruptured intracranial aneurysms (UIAs) are reported more frequently nowadays but their management is not well established; it depends on different structural features of the aneurysms and on multiple patient's risk factors. With this prospective observational study we report our preliminary experience by using four-dimensional (4D) computed tomographic (CT) angiography with ECG-gated reconstructions in the evaluation of dynamic modifications of the aneurysm wall, as a potential predicting factor of growth or rupture. The novelty of this study consists in the correlation between Angio 4D-CT angiography images and surgical findings; only few scientific papers, in fact, have studied this issue. METHODS: Thirty-one patients (1 male, 30 females; 59.0±12.7 years old) with 43 unruptured aneurysms (medium size: 5.2±3.0 mm) were studied. ECG-triggered 4D-CT angiography was performed with a 320-detector CT system (Aquilion ONE; Toshiba Medical Systems Corporation, Otawara, Japan); ECG-gated reconstruction was performed for visualizing local displacement of the aneurysmal wall over a heart cycle. In the aneurysms surgically treated in our institution we were able to compare the macroscopic features of the aneurysm wall with 4D-CTA findings. We performed long-term follow-up on untreated patients. RESULTS: Pulsation was detected in 13 of 43 unruptured aneurysms. Eighteen aneurysms were treated: thirteen were surgically treated and five underwent embolization with detachable coil placement. In nine aneurysms surgically treated in our institution (5 with pulsation and 4 without) we observed a significant correlation between 4D-CTA findings and macroscopic features of the aneurysms wall, in particular the pulsations detected at 4D-CTA study have shown to correspond to dark-reddish thinner wall at surgery. CONCLUSIONS: Our data confirm the previous reports concerning the reliability of 4D-CT angiography with ECG-gated reconstructions in defining the dynamic and structural features of the aneurysm wall. Moreover, optimal correlation rate between the findings provided by the 4D-CTA and the macroscopic surgical evaluation support a possible role of this technique to identify aneurysms with a higher risk of rupture.

Anterior spinal artery syndrome complicated by the ondine curse.

BACKGROUND: Anterior spinal artery (ASA) syndrome results in motor palsy and dissociated sensory loss below the level of the lesion, accompanied by bladder dysfunction. When the cervical spine is involved, breathing disorders may be observed. OBJECTIVE: To describe the polysomnographic findings in a patient with cervical ASA syndrome complicated by a sleep breathing disorder. SETTING: Unit of neurology at a sleep center. Patient A 30-year-old man had an ischemic lesion that affected the anterior cervical spinal cord (C2-C6) bilaterally because of an ASA thrombosis. He developed ASA syndrome associated with respiratory impairment during sleep. RESULTS: The polysomnographic study during sleep showed a severe sleep disruption caused by continuous central apneas that appeared immediately after falling asleep. Treatment by intermittent positive pressure ventilation normalized the respiratory pattern and sleep architecture. CONCLUSIONS: The sleep breathing pattern was compatible with central alveolar hypoventilation due to automatic breathing control failure caused by a lesion of the reticulospinal pathway, which normally activates ventilatory muscles during sleep. This autonomic sleep breathing impairment resembles that found as a complication in patients who undergo spinothalamic tract cervical cordotomy for intractable pain. This surgical complication is known as the Ondine curse.

Computed tomography and magnetic resonance imaging findings in ophthalmoplegic migraine.

A patient with ophthalmoplegic migraine is described, and his computed tomography and magnetic resonance imaging findings are discussed. According to our results, triad migraine, third nerve palsy, and focal enhancement of an enlarged third cranial nerve at the root exit zone should be considered pathognomonic of the disease, and further examinations should be avoided. Pathogenetic theories of the disease are discussed, and we suggest a new pathogenetic theory.

A combined a-EEG and MR spectroscopy study in term newborns with hypoxic-ischemic encephalopathy.

OBJECTIVES: Brain damage following a perinatal hypoxic-ischemic (HI) insult has been documented by different diagnostic techniques. The aim of the present study was to relate a-EEG time course during the first 24h of life to brain metabolic changes detected by proton MR spectroscopy ((1)H-MRS) at 7-10days of life and to evaluate their correlation with outcome. METHODS: Thirty-two patients with any grade HI encephalopathy were studied. Thirty-one out of 32 patients survived and underwent (1)H-MRS examination at 7-10days of life; a-EEG was recorded during the first 24h of life in 27/32 newborns; 26 patients underwent both examinations. Griffiths test, evaluation of motor skills, visual and hearing function were performed at regular intervals until the age of 2years. RESULTS: a-EEG at 6, 12 and 24h of life showed a significant correlation with outcome. N-acetyl-aspartate/creatine (Cr), Lactate/Cr and myo-inositol differed significantly between patients with normal or poor outcome. a-EEG time course during the first 24h of life showed improvement in newborns with normal (1)H-MRS and good outcome and a deterioration in those with abnormal (1)H-MRS and poor outcome. CONCLUSIONS: a-EEG time course may be able to document the severity and the evolution of the cerebral damage following an HI event. a-EEG is related to the severity of cerebral injury as defined by (1)H-MRS and both examinations showed a good correlation with outcome. These data, obtained in non-cooled infants, may represent reference data for future investigations in cooled infants.