Cases reported "Muscular Atrophy"

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1/19. Postinjection vastus lateralis atrophy: 2 case reports.

    We report two cases of postsurgical intramuscular meperidine injection with injury to the femoral nerve and subsequent vastus lateralis atrophy. The first case is a patient who had arthroscopic anterior cruciate ligament reconstruction; the second, a patient with a C6-C7 anterior fusion. photographs, radiography, and electrodiagnostic studies clearly depict the nature of the injuries, and their etiology is discussed. These case reports describe a unique neuropathic injection injury that, to our knowledge, has never before been described in the literature.
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2/19. A case of Dejerine-Sottas disease with schizophrenic symptoms. A clinical and pathological study.

    A case of hypertrophic interstitial neuritis with a disturbance of intelligence and schizophrenic symptoms was reported. The patient, a 41-year-old male, showed monologia at the age of 14 and auditory hallucination at the age of 21. He was diagnosed as schizophrenia. Torsion of the head to the right side and muscular atrophy were also noticed at 21 years. Atrophy of the muscles progressed gradually, particularly in the distal parts of the upper and lower extremeties. Psychiatric examination revealed schizophrenic symptoms such as auditory hallucination, thought disorder, lack of spontaneity, autism, apathy, etc. There was also a disturbance of intelligence (imbecility). In neurological examination, the torsion of the head to the right side, slight exaggeration of the peripheral reflexes, muscular atrophy in the distal parts of the extremeties were noted. The bilateral ulnar nerves were markedly hypertrophied. The biopsy of the right ulnar nerve revealed a remarkable thickening of the endoneuria and "onion bulb" formations. A large amount of the interstitial accumulation of PAS positive substance, which showed metachromasia in cresyl violet staining, was also noted. Schwann cell processes were proved electron-microscopically to surround the myelin sheaths and some of them meandered and extended and into the metachromatic substance. The basement membrane of the sheath cell was hypertrophic in some portions and elongated into interstitium. The nucleus of the sheath cell was hyperchromic and showed marked atrophy. Histological examination of the biopsy specimen obtained from the gastrocnemius muscle suggested neurogenic muscular atrophy. The relationship among the hypertrophic interstitial neuritis, schizophrenic symptoms, disturbance of intelligence and torsion of the head was also discussed.
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3/19. Cervical amyotrophy caused by hypertrophy of the posterior longitudinal ligament.

    STUDY DESIGN: A case report. OBJECTIVES: To report a case of cervical amyotrophy caused by hypertrophy of the posterior longitudinal ligament (HPLL). SETTING: Department of Neurological Surgery, Aichi Medical University, Aichi, japan. methods: The patient had severe muscular atrophy in the deltoid and triceps with slight localized hypesthesia in the C5 area and severely unstable gait due to diminished vibration sense in the knees and ankles. magnetic resonance imaging (MRI) showed expanded cord compression from C4 to C6 with intramedullary high-signal intensity due to HPLL. Transverse image MRI was useful to identify the HPLL. RESULTS: Resection of HPLL was achieved by an anterior approach. Histological findings of the surgical specimens showed thickening of the ligamentous tissue with proliferation of chondrocytes. CONCLUSIONS: HPLL should be included as a causative pathology of cervical spondylotic amyotrophy. Careful neurological examination including sensory examination of the lower limbs should be performed to avoid confusion with motor neuron disease.
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4/19. Bilateral suprascapular nerve entrapment.

    Bilateral suprascapular nerve entrapment syndrome is very rare. It presents with shoulder pain, weakness and atrophy of the supraspinatus and infraspinatus muscles. We present a twenty-year old man having a history of bilateral shoulder pain associated with weakness. Electromyographic studies revealed signs of a lesion that caused a neupraxic state of the left suprascapular nerve, moderate axonal loss of the right suprascapular nerve and denervation of the right suprascapular muscle. The patient was treated with physical and medical therapy. Due to worsening of the symptoms, a surgical operation was performed by the excision of the transverse scapular ligaments bilaterally. His pain, weakness and atrophy had diminished on examination six weeks later. Suprascapular nerve entrapment should be considered in patients with shoulder pain, particularly those with weakness and atrophy of the supraspinatus and infraspinatus muscles.
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5/19. Morphological changes in late onset acid Maltase deficient patients with splicing gene mutation.

    Clinical and morphological features have been studied in 11 late onset Acid Maltase Deficient (AMD) patients. All patients have been diagnosed on biochemical evidence of acid maltase deficiency on muscle biopsy. Molecular studies showed a heterozygous mutation (IVS1-13 T > G transversion resulting in aberrant splicing) in the GSDII gene, which is the most common mutation in late onset AMD patients. Morphological features in muscle biopsy showed a vacuolar myopathy and golgi apparatus proliferation within fibres. The peripheral areas of autophagic vacuoles were positive for caveolin-3 and dystrophin, documenting an extensive membrane turnover. The ultrastructural study of muscle biopsy showed randomly distributed or isolated vesicles sometimes derived from the golgi apparatus. In subsarcolemmal region, lipofucsin bodies and abnormal mitochondria with crystalline inclusions were observed. Primary and secondary lysosomes were typically filled with glycogen. These data suggest a predominant role of Golgi in vesicle proliferation and extensive intra-fibral membrane remodelling. The pathological changes observed are selective for muscle fibres (mostly in type 1) and for muscle groups (mainly proximal). An attractive hypothesis for the variability of clinical phenotype in adult and infantile onset AMD patients is that in the former, an aberrant transcript of smaller size may have originated from alternative splicing (exon 2 skipping). A residual enzyme activity is detectable in muscle, but the intracellular processing of the enzyme precursor from Golgi to the mature form in lysosomes might be blocked.
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6/19. Familial spastic paraplegia with peroneal amyotrophy. A family with hypersensitivity to pyrexia.

    We describe 4 siblings with spastic paraparesis and peroneal amyotrophy who were prone to severe pain and painful dysesthesias, tetraparesis and pyramidal signs during pyrexial episodes of variable etiology. These symptoms cleared almost completely in 10-20 days. Nerve conduction velocity was reduced more markedly during the spells of fever. Muscle biopsy specimen was normal. Some transient functional disturbance of membrane equilibrium of the nervous pathways of both central and peripheral nervous systems was probably responsible for the attacks during pyrexial episodes.
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7/19. Neonatal form of nemaline myopathy, muscle immaturity, and a microvascular injury.

    An infant with a neonatal form of nemaline myopathy showed ultrastructural features of muscle immaturity. Immaturity was characterized by an abnormal presence of myotubes, as well as cells in clusters within a common basement membrane and a great number of satellite cells adhering to very small muscle fibers. In addition, degenerative changes and a severe microvascular lesion were observed. The pathologic findings in the muscle of this patient were those of neonatal nemaline myopathy complicating severe microvascular injury, possibly induced by an unknown toxic agent.
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8/19. Posterior longitudinal ligament ossification myelopathy and hypothyroidism.

    Two elderly white women with a long history of hypothyroidism and myelopathy showed ossification in posterior longitudinal ligament at their cervical spine. Case histories are presented and the reported association of PLLO with other conditions discussed.
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9/19. A new myopathy with tubulomembranous inclusions.

    A muscle biopsy performed on a 16-year-old boy with progressive myopathy revealed hitherto unrecognized peculiar inclusions which consisted of 3 types of structures. The first type consisted of laminated tubulomembranous structures and most of the inclusions belonged to this type. The lamellae were regularly spaced with a periodicity of 8.5--9 nm and curving a little, and were observed as concentric lamellae according to the plane of sectioning. The second type of inclusions consisted of curvifilamentous material. The third type had the appearance of moderately electron-dense granular material surrounded by a single unit membrane. The origin and nature of these inclusions is obscure, but the diagnosis of some kind of storage myopathy was suspected in this case.
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10/19. Inclusion body myositis. Clinical, biological and ultrastructural study.

    A 48-year-old patient presented for the past 4 years an amyotrophy of the quadriceps and moderate involvement of the truncal and pelvic girdle muscles. The CK level was elevated (10 times the normal rate) and the EMG revealed a fibrillation pattern on relaxation, myotonic bursts on needle insertion and reduced activity during contraction. The histological study of the muscle biopsy showed nuclear cytoplasmic inclusion bodies and pseudo-myelinic membranes. The case was classified in the inclusion body myositis group. Analysis of the other published cases underlines the variety of the clinical, biological and electromyographical aspects and abnormalities.
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