Cases reported "Muscular Atrophy"

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1/120. MRI findings of benign monomelic amyotrophy of lower limb.

    We report here magnetic resonance imaging (MRI) findings of two patients with benign monomelic amyotrophy of lower limb. Both subjects showed unilateral amyotrophy of the lower limb with a benign clinical course, and the affected muscles demonstrated neurogenic changes. On T1- and T2-weighted MRI, marked atrophy and increased signal intensity were found mainly in gastrocnemius and soleus muscles. Moreover, MRI examination also revealed that thigh muscles including semitendinosus, semimembranosus, and vastus intermedius and lateralis muscles were involved in one of the patients. We concluded that muscle MRI is very useful for detecting affected muscles, especially deep skeletal muscles in patients with benign monomelic amyotrophy of lower limb.
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2/120. Hereditary neuropathy and vocal cord paralysis in a man with childhood diphtheria.

    We present the case of a 37-year-old Afghani man with a history of childhood diphtheria, who was diagnosed with bilateral vocal cord paralysis at age 15 years. At about this time he developed progressive muscular wasting and distally predominant weakness, and subsequently developed respiratory insufficiency, necessitating nocturnal ventilatory support. His examination suggested a distal symmetric sensorimotor neuropathy, and his brother was similarly affected, although to a lesser degree. electromyography (EMG) and nerve conduction studies revealed this process to be purely axonal. A diagnosis of possible hereditary motor and sensory neuropathy (HMSN) type IIc, hereditary axonal polyneuropathy with vocal cord paralysis, is proposed, although the question of early diphtheritic involvement of the vocal cords and peripheral nerves is also considered.
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3/120. ulnar nerve injuries of the hand producing intrinsic muscle denervation on magnetic resonance imaging.

    Muscle and nerve injuries in the hand may be difficult to detect and diagnose clinically. Two cases are reported in which magnetic resonance imaging showed ulnar nerve injury and intrinsic hand muscle denervation. The clinical, anatomical and radiological features of injury to the deep motor branch of the ulnar nerve and associated muscle denervation are discussed and illustrated.
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4/120. Intravenous immunoglobulin therapy for diabetic amyotrophy.

    A 49-year-old woman with diabetes mellitus developed progressive weakness and atrophy of both thighs rendering her wheelchair-bound within two months. The neurological findings and electrophysiological test results were compatible with diabetic amyotrophy (DA). Immediately after intravenous immunoglobulin (IVIg) therapy (20 g x 3 days), she became able to walk with a cane. After the next course of the therapy, she could walk without assistance. This dramatic effect of IVIg therapy together with the recent observation of vasculitic neuropathy in DA indicates an inflammatory process in this condition, and gives support to this treatment for DA.
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keywords = neuropathy
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5/120. Isolated brachialis wasting: an unusual presentation of neuralgic amyotrophy.

    Although neuralgic amyotrophy can selectively affect discrete components of the brachial plexus including individual peripheral nerves, involvement of an individual nerve fascicle is rare. Discrete fascicular musculocutaneous neuropathy as a manifestation of neuralgic amyotrophy has not previously been reported to our knowledge. We report two cases of otherwise typical neuralgic amyotrophy with isolated brachialis muscle wasting. Abnormal spontaneous activity, motor unit remodeling, or both, was observed only in the brachialis muscle. Lateral antebrachial cutaneous nerve conduction studies were normal. These cases serve to broaden the spectrum of the clinical presentation of neuralgic amyotrophy.
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keywords = neuropathy
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6/120. Osteoid osteoma presenting with focal neurologic signs.

    Osteoid osteomas are benign bone tumors, most commonly located in the femur or tibia. In young children, these tumors can be extremely difficult to diagnose. They commonly present nonspecifically with gait disturbance and pain and characteristically respond well to mild pain relievers. We report two patients who presented with neurologic signs, including atrophy, weakness, and diminished deep tendon reflexes of the affected limb. These two patients demonstrate that osteoid osteomas of the lower extremities can present with neurologic signs, and proper diagnosis requires a detailed history and clinical awareness of this phenomenon.
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7/120. Myotonic pupils in charcot-marie-tooth disease. Successful relief of symptoms with 0.025% pilocarpine.

    Twenty-seven members of a family with dominantly inherited charcot-marie-tooth disease (CMTD) were examined. Fifteen members had CMTD and 13 of these had varying amounts of myotonic pupillary abnormalities similar in some ways to Adie tonic pupil syndrome. Those with graver neurologic disease showed greater pupillary abnormalities. Ten of the 15 patients had pupillary constriction with methacholine chloride (Mecholyl) and some of these had extensive iris atrophy. Several affected patients received symptomatic relief from 0.025% pilocarpine. Seven other patients with CMTD who were not related to our initial family were checked for myotonic pupils; two had findings similar to our initial family. Pupillary abnormalities in certain patients with CMTD appear secondary to a parasympathetic denervation of the iris sphincter and ciliary muscle, as shown by a positive methacholine test, and probably represent part of the autonomic nervous system dysfunction associated with the polyneuropathy in CMTD.
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keywords = neuropathy
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8/120. Acute isolated suprascapular nerve palsy limited to the infraspinatus muscle: a case report.

    Suprascapular nerve palsy resulting in isolated weakness of the infraspinatus muscle is subtle at its onset and must be differentiated from cervical radiculopathy or bone and joint diseases of the shoulder, especially in the presence of pain. The literature suggests an association of cumulative trauma with ganglionic cyst formation and entrapment neuropathy of the suprascapular nerve in the spinoglenoid notch. Here we present clinical, electrodiagnostic, and radiologic features in a young patient who presented with a 2-week history of isolated, painless weakness of the right shoulder. His electromyography showed acute denervation of the right infraspinatus muscle. magnetic resonance imaging revealed a ganglionic cyst in the spinoglenoid fossa and edema in the infraspinatus muscle consistent with denervation. The patient recovered after removal of the cyst.
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ranking = 16702.155665342
keywords = neuropathy
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9/120. Multifocal motor neuropathy and asymptomatic Hashimoto's thyroiditis: first report of an association.

    Motor neuropathy with multifocal conduction blocks represents a recently identified autoimmune disorder of the peripheral nerve myelin. association of motor neuropathies or neuronopathies with thyroid disorders, such as hyperthyroidism, hypothyroidism or thyroid neoplasms has been rarely described. We studied a 61-year-old man with a 2-year-history of slowly progressive weakness of the left limbs with atrophy and fasciculations. Nerve conduction velocity studies revealed multifocal motor conduction blocks. serum IgM titer of antibodies against GM1 was elevated (1:1280; n.v. up to 1:640). Thyroid studies were compatible with Hashimoto's thyroiditis. Therapy with high dose intravenous immunoglobulins was followed by a prompt clinical recovery. Then the disease assumed an intravenous immunoglobulins dependent course with a full clinical, but transient, recovery. This is the first observation of an association of multifocal motor neuropathy with high titers of GM1 and Hashimoto's thyroiditis and reinforces the multifocal motor neuropathy autoimmune origin as well as the repeated clinical recoveries after intravenous immunoglobulins. This case also suggests to deeply investigate the thyroid function in patients with multifocal motor neuropathy.
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ranking = 133618.24532274
keywords = neuropathy, deep
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10/120. gyrate atrophy of the choroid and retina: clinical, ophthalmologic, and biochemical considerations.

    A case of gyrate atrophy of the choroid and retina associated with hyperornithinemia has been subjected to extensive clinical and biochemical investigation. The familial occurrence of the ocular disease and of abnormality of amino acid was unique to this 28-year-old male, being absent in parents and siblings. He presented with progressive visual loss, and was found to have cataracts and large areas of peripheral lacunar atrophy. Clinically there was no other abnormality. However, he was hyperuricemic and has an abnormal EEG. Despite otherwise normal biochemical indices of hepatic, renal, and muscle function; selective catheterization of an artery, the hepatic vein, the renal vein, and a deep forearm vein showed all of these circulatory beds to be producing ornithine according to arteriovenous difference measurements. cerebrospinal fluid and urine contained increased amounts of ornithine. Though electromyography was normal, the muscle biopsy was abnormal. Clinical tests including arginine loading, glucose tolerance testing, and other measurements of blood variables provided inferences as to the metabolic locus of the abnormality. The syndrome is a systemic multiorgan disorder in which the choroid and retina would appear to be target organs and the hyperornithinemia to be of, as yet, undetermined cause and pathogenic significance.
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