1/80. Chronic inflammatory demyelinating polyneuropathy with multiple hypertrophic nerves in intracranial, and intra- and extra-spinal segments.Hypertrophic nerves have occasionally been seen in chronic inflammatory demyelinating polyneuropathy (CIDP), but most are in the cauda equina. We report a case with CIDP in whom magnetic resonance imaging (MRI) with gadolinium diethylene triamine penta-acetic acid (Gd-DTPA) enhancement demonstrated hypertrophy of various peripheral nerves including multiple cranial nerves. Interestingly, none showed neurological signs corresponding to the lesions, except for clinical signs consistent with CIDP. MRI can be useful for the detection of silent, but abnormal nerve involvement in CIDP.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/80. Revascularization of occluded internal carotid arteries by hypertrophied vasa vasorum: report of four cases.OBJECTIVE AND IMPORTANCE: The vasa vasorum are involved in the pathophysiological development of carotid artery atherosclerosis, providing vascular support to the thickened intima and plaque. When advanced atherosclerosis causes carotid artery occlusion, the vasa vasorum may serve as a means of revascularization. CLINICAL PRESENTATION: We studied four patients with internal carotid artery occlusion who exhibited revascularization, distal to the occlusion, by small vascular channels that were inconsistent with recanalization through the thrombus. The channels had an angiographic appearance consistent with their being hypertrophied vasa vasorum. Significant collateral circulation was provided by the revascularization. INTERVENTION: All four patients exhibited adequate collateral circulation and were treated with antiplatelet or anticoagulation medication. CONCLUSION: The vasa vasorum have not been previously reported to contribute to the revascularization of occluded arteries. The four cases presented in this report suggest that the vasa vasorum can be a source of collateral circulation after carotid artery occlusion secondary to atherosclerotic disease.- - - - - - - - - - ranking = 0.11960501956275keywords = canal (Clic here for more details about this article) |
3/80. Complex repetitive discharges: cause or effect of neurogenic muscle hypertrophy?We report a patient with adult-onset spinal muscular atrophy (SMA) of the scapulohumeral type with neurogenic muscle hypertrophy (NMH) in markedly weakened biceps muscles in association with continuous complex repetitive discharges (CRDs). This is an apparently unique case due to the bilaterality of the NMH associated with CRDs as well as the well-circumscribed symmetric upper extremity distribution of the hypertrophy. The possible mechanisms of NMH in association with spontaneous motor activity are discussed.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
4/80. An asymptomatic hypertrophic pacchionian granulation simulating osteolytic lesion of the calvaria.Osteolytic lesions can be seen in various diseases. We present a rare case of symptomatic hypertrophic pacchionian granulation mimicking bone tumor in the calvaria. A 50-year-old woman suffered from a previous VII cranial nerve peripheral paresis accompanied by headache. A plain radiograph revealed a punched-out paramedial occipital lesion. Precontrast-enhanced computed tomographic scans demonstrated a hypodense mass, with a defect of both tables of the left occipital bone. magnetic resonance imaging (MRI) demonstrated a hypointense mass on the T1-weighted image and isointense to cerebrospinal fluid on the T2-weighted image, with capsule-like contrast enhancement by gadolinium. A biopsy was performed. Histologically, hypertrophic pacchionian granulation was diagnosed. The patient has had no growth for 2 years. This case suggests the need to include hypertrophic pacchionian granulation in the differential diagnosis of punched-out lesions.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
5/80. Hypertrophic inflammatory neuropathy involving bilateral brachial plexus.BACKGROUND: The present case is an example of hypertrophic inflammatory neuropathy (HIN). This entity is a rare tumor-like, chronic inflammatory, focal or multifocal, mainly demyelinating neuropathy of unknown origin, most frequently involving the brachial plexus. CASE DESCRIPTION: The authors describe a 67-year-old man presenting with a nodular mass in his right supraclavicular fossa. A nodular mass grossly resembling a schwannoma originating from a single nerve fascicle was surgically removed from the right C6 spinal nerve. Histologically, endoneurial edema, fibrosis, focal chronic inflammation, and extensive "onion bulb" formation were seen. Electron microscopy studies and immunohistochemistry proved that the onion bulb-forming cells were schwannian in nature and that the whorls of onion bulbs surrounded a generally demyelinated axon. Three months following surgery the patient developed acute painless paralysis of his right biceps brachii muscle that rapidly reversed; after that he remained neurologically asymptomatic. MRI revealed multiple fusiform mass lesions involving the brachial plexus bilaterally. Electrophysiologic studies demonstrated a bilateral, asymmetrical, mainly demyelinating neuropathy involving the brachial plexus; they failed to reveal any abnormality suggestive of generalized neuropathy. CONCLUSION: HIN is different from other focal tumor-like neuropathies and in particular from localized hypertrophic neuropathy (LHN).- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
6/80. Solitary neurofibroma of the anal canal: report of two cases.Neurofibromas are benign nerve sheath tumors commonly found in patients afflicted with neurofibromatosis-1. In the absence of multiple neurofibromas or a diagnosis of neurofibromatosis-1, neurofibromas are referred to as solitary and have been reported to involve the skin, subcutaneous tissue, deep soft tissue, and viscera of almost all areas of the body. A neurofibroma involving the anal canal is a rare entity, with only one report in the literature. We present two cases in which large masses involving the anal canal of elderly females were locally resected and proved to be neurofibromas by histopathology. Although rare, these tumors should be considered in the differential diagnosis of patients presenting with an anal mass, because resection alone is the treatment of choice.- - - - - - - - - - ranking = 0.71763011737651keywords = canal (Clic here for more details about this article) |
7/80. P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature.An autopsy case of hypertrophic pachymeningitis and multiple cranial neuropathies is reported. A 53-year-old woman with paraplegia and various neurological signs which developed over a 2 year period was diagnosed as having an epidural mass with thickened dura mater extending from the lower cervical to the thoracic spinal cord. In addition, bilateral episcleritis, blephaloptosis, and blindness of the right eye with various cranial nerve deficits were found to be caused by the mass lesions involving the paranasal sinuses, orbit, and the cavernous sinus. Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) was positive, but cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) was negative by enzyme-linked immunosorbent assay. The partially removed epidural mass with hypertrophied dura mater and biopsy of the paranasal lesions showed chronic granulomatous inflammation with vasculitis. The remaining lesions resolved with steroid therapy with remarkable neurological improvement. The positive p-ANCA test, paranasal involvement, the report of a similar histopathological case and a review of the literature on granulomatous pachymeningitis suggest the presence of p-ANCA-positive Wegener's granulomatosis with central nervous system involvement characterized by hypertrophic pachymeningitis and/or multiple cranial neuropathies.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
8/80. Idiopathic hypertrophic pachymeningitis: a report of two patients and review of the literature.PURPOSE: We report the treatment and follow-up, including MRI, of two patients with idiopathic hypertrophic pachymeningitis and review the English language literature, with emphasis on management and outcome in this rare disorder. methods AND MATERIALS: The files of two patients were reviewed, with relevant histopathology and imaging (MRI). The first patient has been followed for sixteen years (the longest MRI-documented postoperative course reported for this condition) and the second for two years. The English language literature was reviewed, including a summary of all reported patients that have been followed with MRI or CT imaging. RESULTS: Despite extensive investigation, no underlying etiology was determined in either patient. Histopathological studies revealed a chronic inflammatory dural infiltrate in both patients, with granulomas in the first but not the second patient. The first patient underwent surgery twice and has remained stable for sixteen years, despite persistent neurologic deficits. The second patient was managed with dexamethasone after a surgical biopsy, and experienced complete resolution of all neurological deficits and abnormalities seen with MRI. CONCLUSIONS: Although prompt and extensive surgery has been recommended for this condition, the results from our second patient indicate that complete remission can be achieved in some patients with biopsy and steroid therapy. This also supports the view that autoimmune mechanisms underlie idiopathic hypertrophic pachymeningitis. The first patient illustrates that extensive laminectomies may be an effective therapeutic option but chronic discomfort may result. If extensive surgery must be performed, laminoplasty should be done because of the potential for reduced pain and improved long-term spinal stability.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
9/80. Hypertrophic chronic pachymeningitis as a localized immune process in the craniocervical region.Hypertrophic chronic pachymeningitis (HCP) is a rare disorder that causes intracranial or spinal thickening of the dura mater. This report describes a patient with progressive HCP in the craniocervical region associated with signs of rheumatic disease. A ventricular-atrial shunt had to be inserted because of increased intracranial pressure. The patient improved after suboccipital craniotomy, C1 to C6 laminectomy, and removal of the thickened dura. Additional therapy with methotrexate stopped progression, which was documented by MRI and PET.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
10/80. Primary papillary adenocarcinoma confined to the middle ear and mastoid.Primary adenocarcinoma is a rare tumor of the middle ear and temporal bone; its most frequent symptoms are hearing loss, otalgia, and facial paralysis. Otoscopic examination of a 27-year-old man revealed purulent discharge in the ear canal, diffuse edema, and hypertrophy of the right tympanic membrane. He presented with a grade III (House-Brachman) facial paralysis and right conductive hearing loss with a history of aural discharge for 6 months, otalgia, and facial weakness for 2 days. Computed tomography of the temporal bone showed an opacity filling the tympanic cavity, antrum, and aditus. Tympanotomy revealed diffuse edema of the middle ear mucosa, and granulation tissue was encountered during mastoidectomy filling the antrum and periantral cells and eroding the fallopian canal at the level of the oval window. After the histopathological examination revealed papillary adenocarcinoma, a subtotal temporal bone resection, facial nerve segmenter resection, and end-to-end anastomosis of the facial with the hypoglossal nerves were performed. The importance of histopathological examination in all cases of chronic otitis media with granulation tissue is stressed.- - - - - - - - - - ranking = 0.2392100391255keywords = canal (Clic here for more details about this article) |
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