A case of ophthalmoplegic migraine: swelling and Gd-DTPA enhancement of the oculomotor nerve on MRI

We report here a 9-year-old girl with ophthalmoplegic migraine. At the age of 2 years and 6 months she first developed left ptosis and ophthalmoparesis that resolved gradually within 2 weeks. She experienced similar episodes repeatedly. After 5 years of age, left periorbital pulsatile pain preceded ptosis and ophthalmoparesis, and after 7 years, she showed permanent left third nerve paresis even between the attacks. On cranial MRI the left oculomotor nerve showed swelling and contrast enhancement, the latter being more prominent in the ictal than interictal images. Ophthalmoplegic migraine should be considered in the differential diagnosis of opthalmoplegia in children even in the absence of headache. The diagnosis is strongly suspected when MRI demonstrates swelling and enhancement of the oculomotor nerve.     

MRI findings and effectiveness of cyproheptadine in two patients with ophthalmoplegic migraine

We reported the MRI findings and clinical course of two patients with ophthalmoplegic migraine. Both patients presented with unilateral oculomotor nerve palsy. Contrast enhanced MR imaging revealed unilateral enhancement and thickening of the oculomotor nerve in one patient. Prednisolone was effective in both patients, but only could transiently. On the other hand, cyproheptadine hydrochloride could completely prevent recurrent attacks of ophthalmoplegic migraine. Thus, MR imaging with of contrast enhancement is useful in the diagnosis of ophthalmoplegic migraine. Cyproheptadine hydrochloride is better than prednisolone to prevention recurrent attacks and to avoid adverse effects.     

Ophthalmoplegic migraine: MRI findings. Case report

Ophthalmoplegic migraine is a rare syndrome in which headache is associated with ophthalmoplegia and third, fourth or sixth cranial nerves palsy. It occurs most frequently in childhood and teenagers. At magnetic resonance imaging (MRI) with gadolinium (GD-DTPA) it may be observed a transitory enhancement of the affected nerve. We present the case of a male teenager, 16 years old, with typical medical history and enhanced signal at left oculomotor nerve in cisternal portion at MRI weighted in T1 with GD-DTPA. On the control exam, eighteen months later, there was no remarkable lesion. The enhancement of oculomotor nerve at MRI is always pathological and among the differential diagnosis we must include: neoplasia (lymphoma and leukemia), infections (AIDS, syphilis), inflammatory process (sarcoidose and Tolosa-Hunt syndrome) and vascular (posterior communicating artery aneurysm).     

Swelling of the third nerve in a child with transient oculomotor paresis: a possible cause of ophthalmoplegic migraine

Transient oculomotor nerve palsy is rarely observed in childhood and mostly is recognized to be a migraine equivalent. We report an 8-year old girl who presented with recurrent attacks of transient oculomotor paresis. The clinical profile of the patient suggested transient dysfunction in the context of an ophthalmoplegic migraine. A CT scan was normal, but MRI demonstrated a swollen right oculomotor nerve.     

Bilateral ptosis, bilateral upgaze and adduction paresis, and monocular downgaze paresis from a mesencephalic infarction]

We report a 57-year-old man with an ischemic lesion in the midbrain. In the acute stage, he presented with bilateral ptosis and markedly limited extraocular motion except for bilateral abduction and downward motion of the right eye. The pupillary reaction to light of his left eye also was impaired. He was admitted to our hospital, and brain MRI showed a small infarction extending from the left paramedian to the median tegmentum of the midbrain. Three weeks after admission, the ptosis and limited extraocular right eye motion had resolved. The pupillary reaction and downward motion of the left eye normalized gradually within 3 weeks. Two months after admission, ptosis and the limited left eye adduction were partially resolved, but the markedly limited upgaze of the left eye had not changed. Initial neuro-ophthalmologic findings suggested involvement of the caudal part of the oculomotor nucleus and the left oculomotor nerve within the midbrain. The pattern of neuro-ophthalmologic impairment seen in our patient led us to conclude that the caudal oculomotor nucleus and medial part of the fascicular fibers of the left oculomotor nerve probably recovered first, after which recovery of the fascicular fibers progressed laterally. The results of serial MRI were consistent with this interpretation.     

Oculomotor ophthalmoplegic migraine: is it really migraine?

Oculomotor ophthalmoplegic migraine is a rare episodic childhood condition in which a unilateral oculomotor palsy is preceded by headache. I describe six new cases that had magnetic resonance imaging signal abnormalities during the acute phase, consisting of a thickened and enhancing ipsilateral oculomotor nerve at its exit from the midbrain. During the quiescent phase, when the headache had resolved, the signal abnormalities were still present but less dramatic. Seventeen similar cases have been previously reported. The pathophysiology may be a trigeminovascular migraine epiphenomenon that is dependent on the unique oculomotor nerve anatomy and porous blood-nerve barrier at the emergence of the oculomotor nerve from the brainstem and the sequelae of demyelination. Early high-dose corticosteroid treatment is recommended to rapidly resolve an acute episode and to potentially prevent permanent abnormal oculomotor nerve signs.     

Isolated crossed superior rectus palsy in a midbrain infarction]

A 61-year-old man suddenly heard tinnitus and diplopia at night during watchinng television. A few days later he visited at our hospital. Neurologically he exibited marked isolated right superior rectus palsy which was also indicated by the Hess test. No other neurological abnormalities were found such as other ocular muscle paresis, cranial nerve palsies, hemiparesis, sensory impairement or cerebellar ataxia. MRI showed a left medial thalamic infarction extending to a rostral part of the midbrain anterolateral to the cerebral aqueduct at the superior colliculi level. Unilateral superior rectus palsy can rarely be caused by a contralateral midbrain infarction, because fibers from the subnucleus subserving the superior rectus decussate within the oculomoter nerve complex. In this case the crossing fibers toward the contralateral superior rectus may have been selectively involved by a tinny lesion in the area of the oculomotor nucleus. The patient had a slightly narrowed right palpebral fissure. It is indicated that crossing fibers toward the contralateral levator muscle of the eyelid may be also involved. The patient's diplopia completely resolved two months later after the onset.     

Incomplete oculomotor nerve palsy in the subarachnoid space caused by traumatic brain injury

A patient with traumatic brain injury showed incomplete oculomotor nerve palsy in the subarachnoid space. A 12-year-old girl was hospitalized after a head injury. Neuro-ophthalmic examination showed that the left eye had a ptosis and pupillary involvement. An MRI indicated an intracranial hematoma at the basilar portion of the left temple. The ptosis and pupillary involvement improved after elimination of the hematoma. The presentation patterns are best explained by topographic organization of the third nerve fiber within the subarachnoid space. This case suggests that the topographic organization of the third nerve should be considered in diagnosis of oculomotor nerve palsy.     

MRI findings in pediatric ophthalmoplegic migraine: a case report and literature review

Ophthalmoplegic migraine (also recognized as a cranial neuralgia) is a form of migraine characterized by recurrent episodes of headache with ophthalmoplegia related to paresis of cranial nerves III, IV, or VI, with onset typically in childhood. These symptomatic episodes may persist for several hours or for several weeks, months, or permanently. To date, the exact etiology of ophthalmoplegic migraine remains unknown. In previous case reports, ophthalmoplegic migraine may or may not be associated with changes seen on magnetic resonance imaging. Contrast-enhanced magnetic resonance imaging performed during symptomatic and postsymptomatic periods in patients with ophthalmoplegic migraine may hold great value in identifying the pathophysiologic features of oculomotor nerve palsies. Of cases demonstrating abnormal magnetic resonance imaging, a majority show improved but persistent changes on repeat imaging. The present report describes a case of recurrent ophthalmoplegic migraine in a 16-year-old girl. Although the patient presented with ophthalmoplegic migraine during this episode in the same manner as her prior episodes, enhancement of the cranial nerve III on magnetic resonance imaging was evident during the eighth episode whereas previous imaging had been normal. Complete resolution of enhancement of the oculomotor nerve on repeat imaging adds to the few cases that have shown such findings in patients with recurrent ophthalmoplegic migraine. A review of previous reported cases of ophthalmoplegic migraine is offered.     

Ophthalmoplegic "migraine" or recurrent ophthalmoplegic cranial neuropathy: new cases and a systematic review

Ophthalmoplegic migraine is a poorly understood neurologic syndrome characterized by recurrent bouts of head pain and ophthalmoplegia. By reviewing cases presenting to our centers in whom the phenotype has been carefully dissected, and systematically reviewing all published cases of ophthalmoplegic migraine in the magnetic resonance imaging (MRI) era, this review sets out to clearly define the syndrome and discuss possible etiologies. We found that in up to one-third of patients, the headache was not migrainous or associated with migrainous symptoms. In three-quarters of the cases involving the third nerve, there was focal nerve thickening and contrast enhancement on MRI. Observational data suggest systemic corticosteroids may be beneficial acutely. The etiology remains unclear, but may involve recurrent bouts of demyelination of the oculomotor nerve. "Ophthalmoplegic migraine" is a misnomer in that it is probably not a variant of migraine but rather a recurrent cranial neuralgia. A more appropriate name might be "ophthalmoplegic cranial neuropathy."     

Ophthalmoplegic migraine

Ophthalmoplegic migraine is a rare presentation of migraine complicated by an isolated oculomotor paresis. Vasodilation of extracranial vessels is believed to underlie the headache, and vasoconstriction to account for the ophthalmoplegia. Whether the vascular insult involves the central or peripheral portions of the oculomotor nerve is still uncertain. We describe a child who presented with ophthalmoplegic migraine and was demonstrated to have a deficiency of the near triad documented by eye movement and pupillary recordings. Voluntary conjugate eye movements--saccades, smooth pursuit, and optokinetic nystagmus--were normal. Vergence amplitudes appropriate to fixation distance were elicited for Snellen optotypes but not to a point source of light. Concurrent measures of pupillary diameter failed to reveal significant modulation to either stimulus. Brain magnetic resonance imaging scan was normal, and there was no contrast enhancement of the oculomotor nerve at its exit from the midbrain. Both the oculomotor paresis and concurrent presence of a deficiency of the near triad localized the vascular insult to the oculomotor nerve complex in the brainstem.     

Mononeuritis des N. oculomotorius bei infektiöser Mononukleose

A 19-year-old immune-competent patient developed right-sided headache and, subsequently, subacute diplopia. On clinical examination he had incomplete right oculomotor palsy. Cranial MRI showed pathologic contrast enhancement of the right oculomotor nerve at its exit point from the mesencephalon, and the CSF displayed slight pleocytosis. The following relevant differential diagnoses were not supported by additional examinations: neurosarcoidosis, Lyme neuroborreliosis, neurosyphilis, tuberculous meningitis, viral meningitis (HIV, VZV, CMV), CNS lymphoma, vasculitis associated with rheumatic disease, Tolosa-Hunt syndrome, and diabetic neuropathy. However, on the basis of blood lymphocytosis, positive heterophile antibody test (Paul-Bunnell test), the presence of IgM antibodies against Epstein-Barr virus capsid antigen, and elevated transaminases, infectious mononucleosis was diagnosed. Isolated neuritis of the oculomotor nerve is a rare parainfectious manifestation of infectious mononucleosis.     

A patient with reversible pupil-sparing Weber's syndrome

This is a case report of a lady who presented with pupil-sparing Weber's syndrome. She had left oculomotor nerve palsy with normal pupil and right hemiparesis. The patient subsequently made a good recovery. An ischemic lesion of the lower mid-brain was demonstrated on the MRI scan of the brain, which corresponds to the motor nucleus of the oculomotor nerve. The article also describes the neuroanatomy of the oculomotor nerve and how its partial lesions lead to sparing of the pupil. In addition, this case report documents that a pupil-sparing Weber's syndrome could be reversible.     

Oculomotor nerve schwannoma mimicking ophthalmoplegic migraine

Ophthalmoplegic migraine (OM) is a rare variant of migraine characterized by recurrent attacks of severe headache followed by oculomotor nerve palsy. The recent revision of the International Headache Classification has reclassified OM from a subtype of migraine, defined as a functional headache, to the neuralgia category. We describe a case of an 11-year-old girl with pathologically confirmed oculomotor nerve schwannoma who had been suffering from symptoms mimicking OM. For five years, she has been under treatment for OM, an initial diagnosis which was corroborated by brain magnetic resonance imaging (MRI). Usually, most OM attacks occur during one period in a lifetime and remit completely. In contrast, however, her attacks became more frequent and were not controlled by medication. After surgery, the frequency of OM attacks was reduced. From this experience, we hypothesize that optic nerve tumor is one condition that can mimic OM, without apparent signs suggestive of intracranial mass. To our knowledge, this is the first report to describe a pathologically confirmed case of oculomotor nerve schwannoma mimicking OM.     

Rendu-Osler-Weber syndrome presented paramedian thalamic and midbrain infarcts and primary medullary hemorrhage: a case report

We reported a 41-year-old male with paramedian thalamic and midbrain infarcts due to cerebral embolism from bilateral pulmonary arterio-venous fistula and primary medullary hemorrhage. The patient had an episode of sudden onset consciousness disturbance with left Weber's syndrome (right hemiplegia and left oculomotor palsy) and vertical gaze palsy at age of 23. He noticed numbness in the left hand and the left half body under clavicular when he had got up in a morning at age 41. He had headache and left tinnitus on second and third days, and on the 3rd and 4th days, he experienced nausea. He had severe hiccup persisting from the 6th to the 13th days. The 23rd days he was admitted to our hospital. He showed dysesthesia and paresthesia in left half body under clavicular, dysesthesia in left hand and vertical gaze palsy and convergence disturbance. MRI performed on the 18th and 24th days, disclosed hyperdense mass in T1 and T2-weighted images in dorsal site of medulla, but the 70th days MRI showed no abnormal lesions. Therefore we diagnosed the high intensity mass as primary medullary hemorrhage. Cerebral angiography showed no abnormal vasculature. Many members of his family had history of sever nasal bleeding. He had skin hemangioma and mucosal hemangioma in esophagus, stomach, colon and rectum, and bilateral pulmonary arterio-venous fistula which had been operated at age 39. His mother also had skin hemangioma and pulmonary arterio-venous fistula. Therefore this family was diagnosed Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia). MRI also disclosed multiple cerebral infarctions in bilateral thalamus, left cerebral peduncle and left cerebellar hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)     

Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient

Malignant peripheral nerve sheath tumors (MPNSTs) arising from cranial nerves are rare and usually affect adults. Here we report the clinicopathologic features of a young adult patient with a trigeminal nerve MPNST, in whom another tumor involving the oculomotor nerve on the contralateral side was evident. The patient, an 18‐year‐old woman, had suffered recurrent paroxysmal sharp stabbing pain over her cheek and forehead on the right side for 1 month. A brain MRI study disclosed a mass, 35 mm in diameter, in the right Meckel's cave, and another mass, 10 mm in diameter, involving the intracranial portion of the left oculomotor nerve. Following gadolinium administration, the former and latter tumors exhibited strong and weak enhancement, respectively. The patient had no clinical stigmata characteristic of neurofibromatosis type 1. Following a tentative diagnosis of schwannoma, total resection of the trigeminal nerve tumor was performed. Histologically, the tumor consisted of highly cellular, spindle‐shaped cells arranged in a fascicular pattern, with occasional mitotic figures, nuclear pleomorphism and necrosis. Immunohistochemically, the tumor cells showed variable intensities and frequencies of reactivity for S‐100 protein, myelin basic protein, CD34, podoplanin and p53, but no reactivity for Smarcb1. Thus, the tumor exhibited features of MPNST. This case appears to provide information that is useful for accurate diagnosis and surgical planning in patients with bilateral or multiple cranial nerve tumors.     

Fascicular arrangement within the oculomotor nerve MRI analysis of a midbrain infarct.

The fascicular arrangement of the oculomotor nerve within the midbrain is not adequately elucidated in humans. We treated a patient with a partial oculomotor palsy who had impaired adduction and supraduction on the left side, which were attributed to an ipsilateral lacunar infarct. CT and MRI revealed a discrete lesion in the centre of the midbrain tegmentum in the rostrocaudal plane. This case suggests that the oculomotor fibres for extraocular movement are located in the middle of the the midbrain, and supports the fascicular proximity of the superior and medial rectus muscles. The fascicular arrangement of the midbrain oculomotor nerve is speculated to be pupillary component, extraocular movement and eyelid elevation in that rostrocaudal order, based on the previous reports of neuro-ophthalmological impairment and MRI findings, which are analogous to the nuclear arrangement proposed by Warwick.     

Isolated nuclear oculomotor nerve palsy due to mesencephalic infarction

A patient with unilateral nuclear oculomotor palsy due to midbrain infarction is described. A 46-year-old man was admitted because of difficulty in opening right eye and double vision noticed when he awoke in that morning. On admission, neurological examination revealed total right oculomotor palsy with slight impairment of left upward gaze. There were no other neurologic abnormalities at all. Brain CT and cerebral angiograms were also normal. Magnetic resonance imaging (MRI) performed on the ninth day, however, demonstrated high signal intensity in the right tegmentum of the mesencephalon on T2-weighted images, which was shown more clearly after the administration of Gadolinium-DPTA. He was diagnosed as nuclear third nerve palsy caused by midbrain infarction. The majority of isolated oculomotor nerve palsy has been reported to be caused by extraaxial lesion. When the oculomotor palsy is caused by intraaxial ischemic lesion, it is usually accompanied by other brain stem signs, because abundant nuclei and fibers are present adjacent to the oculomotor nucleus and nerve in the mesencephalon. The present case clarified that such a small infarct disclosed only by MRI can cause isolated oculomotor nerve palsy. It is emphasized that the intraaxial ischemic lesion should be ruled out by using the sophisticated diagnostic aid before making diagnosis of peripheral lesion. This is the first report of the isolated third nerve palsy resulting from mesencephalic ischemic lesion in the Japanese.     

Isolated oculomotor nerve palsy in lymphoma

We report a patient with non-Hodgkin's lymphoma who developed a unilateral left oculomotor nerve palsy. Only eyelid lifting and vertical gaze were involved. Lateral gaze or sizes and light reactions of pupils were not involved. Magnetic resonance imaging revealed an enhancement of an upper part of left cavernous sinus and the posterior clinoid process. It was conceivable that lymphoma invaded the upper branch of oculomotor nerve. Such neurological symptoms in cases of oculomotor nerve palsy by lymphoma have not been reported previously. Because cranial neuropathy could occur as the first sign of lymphoma, lymphoma is an important differential diagnosis for the partial oculomotor palsy such as our present case.     

Successful steroid pulse therapy for acute unilateral oculomotor nerve palsy associated with norovirus infection

We report a 4-year-old boy who developed acute unilateral oculomotor nerve palsy following Norovirus infection. He visited our hospital because of diplopia three weeks after Norovirus gastroenteritis. Physical examination showed only the left oculomotor nerve palsy. Enhanced MRI of the brain and cerebrospinal fluid examination revealed no abnormality. Anti GQlb antibody was negative. Because blepharoptosis and ophthalmoplegia progressed rapidly, we performed three courses of steroid pulse therapy (methylpredonisolone 30 mg/kg x 3 day/course) combined with vitamin B6. Autonomic dysfunction (isocorea, light reflex) began to improve in several days and subsequently extraocular movements (blepharoptosis, infraduction supraduction, adducent in order) resolved completely in one month. Idiopathic oculomotor paralysis is usually believed to be selflimited, but steroid pulse therapy should to be considered in cases ocular paralysis is so severe or progressive that immune-mediated mechanism was presumed.