Cases reported "Cranial Nerve Diseases"

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1/11. Neurological deterioration after coil embolization of a giant basilar apex aneurysm with resolution following parent artery clip ligation. Case report and review of the literature.

    The authors present the case of a patient who suffered from progressive cranial nerve dysfunction, radiographically documented brainstem compression, and peduncular hallucinosis after undergoing endosaccular coil placement in a giant basilar apex aneurysm. Symptom resolution was achieved following clip ligation of the basilar artery. The pathogenesis of aneurysm mass effect due to coil placement is discussed and the pertinent literature is reviewed.
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2/11. Garcin syndrome resulting from a giant cell tumor of the skull base in a child.

    Garcin syndrome is characterized by a progressive ipsilateral involvement of cranial nerves, culminating in paralysis of all or at least seven of them, without sensory or motor long-tract disturbance, with no intracranial hypertension, and with osteoclastic involvement in the skull base on radiographic computed tomography. Giant cell tumor is a primary bone tumor rarely affecting the skull base. An 8-year-old female presented with a 3-month history of increasingly worsening right otalgia, tinnitus, hearing loss, right facial numbness, and diplopia. She was admitted with a 2-week history of swallowing difficulties, voice change, and right shoulder pain. neurologic examination disclosed unilateral paralysis of the right fifth through twelfth cranial nerves, with no other abnormal neurologic findings. Skull radiographic computed tomography revealed lytic lesions in the right temporal petrous portion. Computed tomographic scan indicated a destructive mass involving the right greater wing of the sphenoid bone and temporal petrous apex. magnetic resonance imaging demonstrated a tumor arising from the temporosphenoidal region, infiltrating neither the brain nor the brainstem. No hydrocephalus was observed. biopsy revealed giant cell tumor. Posterior treatment consisted of radiotherapy. At an 8-year follow-up, the patient was well but with functional sequelae. There is no magnetic resonance imaging evidence of tumor growth. No other giant cell tumor presenting as Garcin syndrome is known to have been reported.
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3/11. Disseminated intraparenchymal microgranulomas in the brainstem in central nervous system sarcoidosis.

    We report a 70-year-old woman with sarcoidosis and multiple cranial nerve palsy. The patient suffered from dysarthria, dysphagia and weakness of the upper and lower extremities and died of sepsis. No abnormalities were noted in brain MRI. At autopsy, numerous epithelioid granulomas with Langhans giant cells were present in the bilateral lungs, including the hilar lymph nodes. The brain had a normal external appearance. Histologically, there were brainstem parenchymal lesions consisting of many microgranulomas, lymphocytic infiltration, activated microglias and astrocytosis. Perivascular lympocytic cuffing was also seen. Neither granulomas nor lymphocytic infiltration were seen in the leptomeninges. The present case was considered to be a peculiar type of neurosarcoidosis, that is, "sarcoid brainstem encephalitis".
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4/11. Acute monocular visual loss in carcinomatous hypertrophic pachymeningitis mimicking giant cell arteritis.

    This report describes a 69-year-old woman who presented with acute monocular visual loss, ipsilateral headache, and elevated sedimentation rate and c-reactive protein. Both temporal artery biopsies were negative. neuroimaging, dural biopsy, and breast biopsy all confirmed the diagnosis of carcinomatous hypertrophic pachymeningitis associated with metastatic breast carcinoma. After treatment with corticosteroids, her vision improved. Her clinical presentation initially mimicked the symptoms and signs of giant cell arteritis. Acute monocular visual loss without other cranial nerve palsies may be an uncommon presentation of hypertrophic pachymeningitis from metastatic breast carcinoma.
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5/11. Intracavernous carotid aneurysm--an unusual cause of isolated abducens nerve palsy.

    We discuss the case of a 59-year-old woman who presented to the emergency department with complaints of diplopia for five days. physical examination disclosed an isolated complete right abducens nerve palsy. Angiography revealed a giant aneurysm from the postcavernous portion of the right internal carotid artery. This case illustrates an unusual etiology of a unilateral abducens nerve palsy.
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6/11. Giant cholesterol cysts of the petrous apex.

    The giant cholesterol cyst is a clinical entity distinct from cholesterol granulomas and other destructive lesions of the petrous bone. Preoperative assessment by computed tomographic scan and magnetic resonance imaging is extremely helpful. Attempts at total resection of the lesion are not necessary. Adequate surgical drainage may be established through the mastoid or middle fossa.
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7/11. Familial granulomatous synovitis, uveitis, and cranial neuropathies.

    A family is presented that had what is believed to be a previously undescribed syndrome of granulomatous synovitis, bilateral recurrent uveitis, and cranial neuropathies. Affected members included the proband, his brother, father, and probably the decreased paternal grandmother. disease onset was in childhood. Each had symmetric, boggy polysynovitis of the hands and wrists, resulting in nearly identical boutonniere deformities. hand radiography in the proband and his brother revealed no erosions or joint destruction despite more than 20 years of disease. Synovectomy specimens in the proband and his brother showed granulomatous inflammation with giant cells. Recurrent, nongranulomatous, acute iridocyclitis with visual impairment afflicted the proband, brother, and father. Apparently corticosteroid-responsive bilateral neurosensory hearing loss occurred in the proband, and a transient sixth cranial nerve palsy in his brother. All members of the family were antinuclear antibody-, rheumatoid factor-, and HLA-B27-negative. serum angiotensin-converting enzyme levels were within normal limits in all family members. The inheritance pattern of this syndrome is most consistent with an autosomal dominant mode.
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8/11. Isolated accessory nerve palsy of unusual cause.

    We report the case of a 23 year old male patient who presented for an isolated left accessory nerve palsy which had appeared one year before. Neuroradiological investigations showed that the causative pathology was a giant saccular aneurysm of the intracranial left vertebral artery. Three months after diagnosis, signs of bulbar palsy rapidly developed. An emergency intra-arterial embolization was then attempted, which led to complete recovery except for the accessory nerve palsy which remained unchanged. We conclude that, in cases of apparently isolated accessory nerve palsy, neuroradiological investigations should include the posterior fossa.
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9/11. Angiocentric T-cell lymphoma presenting with multiple cranial nerve palsies and retrobulbar optic neuropathy.

    Angiocentric T-cell lymphoma (lymphomatoid granulomatosis) may present with prominent central nervous system (CNS) findings with variable radiographic features. We describe a patient who presented with multiple cranial nerve palsies involving the left optic nerve, left facial nerve, left ocular motor nerves, and bilateral acoustic nerves. Enhancement of the right temporal meninges and a cavernous sinus mass were noted on magnetic resonance (MR) scan. A right temporal craniectomy and meningeal biopsy were performed. Meningeal biopsy revealed atypical angiocentric granulomatous lymphoid infiltrates without associated necrosis, giant cells, or granuloma formation. Morphologic and T-cell, receptor gene rearrangement findings were diagnostic of an angiocentric T-cell lymphoma. Retrobulbar optic neuropathy and multiple cranial nerve palsies may be the presenting features of angiocentric T-cell lymphoma. The neurologic and unique radiographic changes in our case expand the previously reported findings in CNS angiocentric T-cell lymphoma.
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10/11. Eight-and-a-half syndrome: one-and-a-half syndrome plus cranial nerve VII palsy.

    Three cases of isolated one-and-a-half syndrome with facial nerve palsy related to infarction are presented. magnetic resonance imaging in cases 1 and 2 was unremarkable, whereas magnetic resonance angiography demonstrated pathophysiologically significant vertebral basilar disease. Case 3 is unique due to its association with giant cell arteritis. Ipsilateral adduction improved to a greater extent than abduction in each case, perhaps providing insight into the exact localization of these lesions or selective vulnerability of the ocular motor structures within the pons. This combination of clinical findings, termed the 8-1/2 syndrome (cranial nerve 7 1-1/2), allows precise localization, and magnetic resonance angiography appears to be the imaging study of choice.
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