1/102. Hypoglycemic coma masquerading thyrotoxic storm.A 59-year-old woman was hospitalized in hypoglycemic coma. Although hypoglycemia was promptly reversed, she was in a somnolent, restless state with tachycardia, tremor, profuse sweating, and high body temperature. Thyrotoxic storm was highly suspected and vigorous antithyroid regimens gradually brought her up to normal mental and cardiovascular states in several days. However, profound generalized myopathy necessitated the maintenance with a respirator. One month later, an episode of angina pectoris was followed by generalized convulsion, coma, and death in a few days. neuroimaging study disclosed posterior leukoencephalopathy syndrome. This case is instructive in that hypoglycemic coma may masquerade the major symptomatology of thyrotoxic storm, and that profound myopathy and angiopathic or angiospastic processes of the brain and the heart may interfere with the outcome.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/102. Diaphragmatic cramp as a possible cause of noncardiac chest pain and referred mandibular pain.The initial assumption that sudden acute chest pain may be of cardiac origin is justifiable, but when this proves not to be the case the patient is left with little explanation of the cause. It is suggested here that diaphragmatic cramp may be a cause of some undiagnosed noncardiac chest pains associated with mandibular referred pain. The phrenic nerve provides both motor and sensory innervation to the diaphragm, while the trigeminal nerve carries sensation from the mandibular teeth. Both nerves originate in separate nuclei close together in the lower medulla. Interconnections between these nuclei and others higher up in the brain may provide one explanation for this problem.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/102. Type II neurofibromatosis presenting as quadriceps atrophy.A young woman aged 26 years presented with atrophy of the left quadriceps progressing over one year. magnetic resonance imaging (MRI) showed a large lesion of the lumbar plexus compatible with neurinoma. Cerebral MRI revealed a lesion in the right eighth cranial nerve also compatible with neurinoma. On further questioning of the patient, it was learned that her mother had undergone surgery twice for acoustic neurinoma. Type II neurofibromatosis was diagnosed from the basis of clinical picture, neuroimaging findings and family history. This case is unusual for the lack of central nervous system (CNS) symptoms. Expansive radicular lesions compatible with neurinoma should prompt cerebral MRI. Regular examination of family members at risk and early diagnosis can decrease the high mortality associated with this condition.- - - - - - - - - - ranking = 8.5953050079419keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/102. cysticercosis of the triceps--an unusual pseudotumor: case report and review.cysticercosis is a disease caused by encysted larvae of the tapeworm taenia solium. Most muscular disease presents with central nervous system involvement or multiple cysts or both. The authors report a 25-year-old woman with a rare single muscle cyst presenting as a tender hard triceps mass. Results of clinical examination and magnetic resonance imaging were indicative of a soft tissue tumor. Excision of the mass revealed purulence, and the histologic diagnosis was cysticercosis. In the differential diagnosis of isolated muscular masses, a rare isolated cysticercus presenting as a pseudotumor should be considered.- - - - - - - - - - ranking = 8.5953050079419keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/102. central nervous system disease in patients with macrophagic myofasciitis.Macrophagic myofasciitis (MMF), a condition newly recognized in france, is manifested by diffuse myalgias and characterized by highly specific myopathological alterations which have recently been shown to represent an unusually persistent local reaction to intramuscular injections of aluminium-containing vaccines. Among 92 MMF patients recognized so far, eight of them, which included the seven patients reported here, had a symptomatic demyelinating CNS disorder. CNS manifestations included hemisensory or sensorimotor symptoms (four out of seven), bilateral pyramidal signs (six out of seven), cerebellar signs (four out of seven), visual loss (two out of seven), cognitive and behavioural disorders (one out of seven) and bladder dysfunction (one out of seven). brain T(2)-weighted MRI showed single (two out of seven) or multiple (four out of seven) supratentorial white matter hyperintense signals and corpus callosum atrophy (one out of seven). evoked potentials were abnormal in four out of six patients and CSF in four out of seven. According to Poser's criteria for multiple sclerosis, the diagnosis was clinically definite (five out of seven) or clinically probable multiple sclerosis (two out of seven). Six out of seven patients had diffuse myalgias. deltoid muscle biopsy showed stereotypical accumulations of PAS (periodic acid-Schiff)-positive macrophages, sparse CD8 T cells and minimal myofibre damage. Aluminium-containing vaccines had been administered 3-78 months (median = 33 months) before muscle biopsy (hepatitis b virus: four out of seven, tetanus toxoid: one out of seven, both hepatitis b virus and tetanus toxoid: two out of seven). The association between MMF and multiple sclerosis-like disorders may give new insights into the controversial issues surrounding vaccinations and demyelinating CNS disorders. deltoid muscle biopsy searching for myopathological alterations of MMF should be performed in multiple sclerosis patients with diffuse myalgias.- - - - - - - - - - ranking = 12.927559462031keywords = nervous system (Clic here for more details about this article) |
6/102. X-linked myopathy with excessive autophagy: a clinicopathological study of five new families.In 1988, Kalimo et al. (Ann Neurol 23 (1988) 258)described a new type of X-linked myopathy in a Finnish family. The clinical course was characterized by slow progression of muscle weakness without loss of ambulation in childhood and no evidence of cardiac, respiratory, or central nervous system involvement. Muscle fibers were not necrotic and showed excessive autophagic activity and exocytosis of the phagocytosed material. These authors proposed the name X-linked myopathy with excessive autophagy. Subsequently, only one French family has been reported with similar clinical and histopathological data. We report here five new families with a total of eight affected boys with the same clinical and histopathological features as reported in the original families. Histopathological findings of an asymptomatic mother are also reported. Vacuolar changes in muscle fibers result both from invaginations of the sarcolemma along with a variable component of basal lamina and from an autophagic process. The complement c5b-9 membrane attack complex associated with MHC class 1 antigen and calcium deposits is involved in muscle fiber damage. Among the X-linked myopathies, the identification of this new type is of great interest because of its favorable prognosis and unique morphological findings.- - - - - - - - - - ranking = 8.5953050079419keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/102. Respiratory flutter syndrome: an underrecognized cause of respiratory failure in neonates.We report the clinical and respiratory data of three neonates with flutter of the diaphragm and intercostal muscles, presenting soon after birth with respiratory failure. The breathing pattern was dirhythmic with superimposed frequencies, one regular and slow (60/min) representing the underlying respiratory rate, the other fast (> 300/min) and limited to inspiration. Nasal continuous positive airway pressure immediately normalized the breathing pattern in one infant, and improved ventilation in the two others. Pharmacologic therapy with chlorpromazine terminated the respiratory flutter and permitted weaning of ventilatory support within a few hours. Coexistent dysphagia suggested a disorder of brainstem function, although the children were otherwise developmentally normal at 8, 10, and 26 mo old. laryngomalacia and gastroesophageal reflux were also present. We propose that the occurrence of respiratory flutter, dysphagia, laryngomalacia, and gastroesophageal reflux in a neonate constitutes a distinct clinical entity, termed the "respiratory flutter syndrome." The diagnosis of three infants with this presentation during an 18-mo period suggests that this may be a more frequent cause of respiratory failure in newborns than previously recognized.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/102. Cavernous hemangioma of the temporal muscle.Intramuscular cavernous hemangiomas are often found in the brain, but they are uncommon in the muscles of the head and neck region, with the masseter being the most frequent, followed by the trapezius and sternocleidomastoideus. Such a lesion in the temporal muscle is an extremely unusual situation. A 55-year-old man presented with intermittent headaches and painless swelling of the right temple region brought on by stress and bending forward. On MRI, T1-weighted imaging with enhancement after contrast medium showed a low signal-intense, fat-free lesion restricted to the temporal muscle. T2 weighting showed a hyperintense, high fluid content, low-flow lesion. There was no progression within 2 years. No resection was performed. In planning a treatment approach, one must bear in mind that cavernous hemangiomas in the temporal muscle or other muscles of the head and neck can be clinically distinguished from the more aggressive capillary ones. Complications are extremely rare (hemorrhage or functional deficits). These cavernomas should simply be followed up and only resected in case of any problems (cosmetic, neurological deficits).- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/102. mulibrey nanism: review of 23 cases of a new autosomal recessive syndrome.Mulibrey (muscle, liver, brain, eye) nanism is probably an autosomal recessive condition characterized by progressive growth failure of prenatal onset, triangular face with hydrocephaloid skull, general thinness and muscular hypotonicity, peculiar voice, venous congestion caused by pericardial constriction, and pigment dispersion and yellowish dots in ocular fundi. Two thirds of the patients had cutaneous nevi flammei and one third cystic fibrous dysplasia of the tibia. Probably a substantial portion of the affected are lost by early abortion and others by infantile death. The physical capacity and life expectancy seem to vary depending on the degree of the cardiac affection.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/102. Cerebrotendinous xanthomatosis.A case of presumed cerebrotendinous xanthomatosis is described. The association of cataracts with central nervous system signs and tendon xanthoma is noted. Deposition of cholestanol appears to be the primary lesion in this disease.- - - - - - - - - - ranking = 8.5953050079419keywords = central nervous system, nervous system (Clic here for more details about this article) |
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