Cases reported "Hypertrophy"

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1/133. hypertrophy and pseudohypertrophy of the lower leg following chronic radiculopathy and neuropathy: imaging findings in two patients.

    Enlargement of the ipsilateral muscle compartment is an exceptional finding in patients with chronic radiculopathy, peripheral nerve injury, anterior horn cell diseases, or acquired peripheral neuropathy. We report radiographic, ultrasonographic, CT and MRI findings in a patient with chronic S1 radiculopathy and another with chronic neuropathy of the common fibular nerve (L4-S2), both presenting with painless enlargement of the calf muscles.
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2/133. 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.
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3/133. Hippocampal sclerosis with hypertrophy of end folium pyramidal cells.

    Mesial temporal lobectomy for the treatment of intractable temporal lobe seizures may show dual pathologies for example hippocampal sclerosis (HCS) combined with a malformation. In a lobectomy specimen from a 40-year-old female with typical radiological and pathological features of HCS, an additional histopathological finding was the presence of hypertrophic pyramidal cells in the dentate hilus, in which cytoplasmic accumulations of phosphorylated neurofilament were demonstrated. Although these cells closely resembled dysplastic nerve cells of cortical dysplasia, we argue that the cytoskeletal abnormalities observed are a result of ongoing alterations to hippocampal circuitry in an evolving HCS.
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4/133. 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.
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5/133. 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).
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6/133. Idiopathic hypertrophic cranial pachymeningitis: clinicoradiological spectrum and therapeutic options.

    OBJECTIVE: Idiopathic hypertrophic cranial pachymeningitis is a rare disease, of undetermined pathogenesis, that is characterized by inflammation and fibrosis of the dura mater. methods: We encountered six patients with idiopathic hypertrophic cranial pachymeningitis and analyzed their clinical presentations, radiological findings, and treatment. RESULTS: In the six patients, the main manifestations were cranial nerve palsies and headache. Three associations were present, namely optic neuropathy, tolosa-hunt syndrome, and diabetes insipidus. gadolinium-enhanced magnetic resonance imaging was diagnostic, showing intense dural enhancement in a linear or nodular pattern. The responses to corticosteroid therapy were better for patients who exhibited linear, rather than nodular, dural enhancement. For one patient, surgical decompression of the superior orbital fissure provided lasting relief. The course of the disease followed one of three patterns, i.e., sustained remission, relapse with corticosteroid independence, or relapse with corticosteroid dependence. pulse corticosteroid therapy provided significant relief, while reducing the daily corticosteroid requirement and avoiding side effects, for a corticosteroid-dependent relapsing patient. CONCLUSION: Idiopathic hypertrophic cranial pachymeningitis exhibits varied clinical courses. It is important to prevent irreversible cranial neuropathy during the active phase of the disease, using daily administration of corticosteroids, pulse corticosteroid therapy, or surgical decompression.
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7/133. Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.

    OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.
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8/133. 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.
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9/133. Muscle hypertrophy due to scarring of the S1 nerve root.

    Segmental muscle enlargement occurs in a variety of neurogenic conditions. We present a patient with calf hypertrophy, likely produced by continuous neuromuscular irritability and compensatory type 1 and type 2 muscle fiber hypertrophy. The underlying lesion of the S1 nerve root was caused by scarring, which could be demonstrated by Gadolinum enhanced, fat saturated magnetic resonance imaging. Thus, the application of this technique is recommended in otherwise etiologically unclear cases of neurogenic muscular lesions in order to detect chronic nerve root pathology.
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10/133. 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.
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