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Biomedical subjects

M P Merchut

Publications and source records attributed to M P Merchut.

17 recordsLinked to original sources

Segmental zoster paresis of limbs.

Segmental zoster paresis (SZP) is the focal, asymmetrical neurogenic weakness which may occur in a limb affected by cutaneous zoster. We have summarized the features of this syndrome, based on a retrospective review of 8 personal and 96 published cases. Limb SZP becomes apparent in at least 3-5% of patients with cutaneous zoster, who are usually over the age of sixty and weak proximally (C5,6,7 or L2,3,4 innervated muscles). Functional motor recovery occurs in about 75% of cases, generally by 1-2 years. Limb weakness is probably due to a lesion of the ventral nerve root, in close proximity to the initiating dorsal ganglionitis. The electrodiagnostic findings, scarce in the literature, typically consist of absent compound sensory nerve action potentials in the involved limb, with less frequent reduction or loss of compound muscle action potentials. Fibrillations and positive sharp waves become detectable within 1-4 months in limb and related paraspinal muscles, decreasing or disappearing later. In addition to this radiculopathy, peripheral nerves may also occasionally become involved, manifest as mononeuropathies of the median, ulnar, long thoracic, recurrent laryngeal, and phrenic nerves. The zoster infection or consequent inflammatory response appears able to affect motor axons distally as well as proximally.

Action Potentials↗

Quantitative sensory threshold testing in patients with multiple sclerosis.

Cutaneous thresholds for vibratory and thermal stimuli were quantitated at the index finger and great toe of twelve patients with definite multiple sclerosis. Nine of 12 (75%) patients had abnormalities to either modality, especially at the great toe, where 48% of vibratory and 17% of thermal thresholds were abnormal. Of ten patients undergoing somatosensory evoked potential (SSEP) testing, 8 (80%) had abnormal median and 9 (90%) had abnormal tibial studies. While more experience is needed to determine the sensitivity of quantitative sensory threshold testing in this application, this simple, noninvasive technique appears to correlate both with clinical and SSEP findings.

Adult↗

Renal causes of elevated sedimentation rate in suspected temporal arteritis.

The erythrocyte sedimentation rate (ESR) is a frequently used but nonspecific indicator of inflammation or infection. Clinicians often check an ESR in patients with symptoms of headache, facial or jaw pain, and visual loss, as an aid in the diagnosis of temporal arteritis. We present two patients with these complaints, who did not have temporal arteritis, nor any other inflammatory condition or infection, but had ESRs near or above 100 mm/h, leading to diagnostic confusion. An occult nephrotic syndrome, with or without renal insufficiency, can cause such a highly elevated ESR, and was discovered in these patients.

Acute Disease↗

Systemic lupus erythematosus with central nervous system involvement.

Real or suspected brain involvement occurs in the majority of patients with systemic lupus erythematosus. The clinical manifestations are myriad and are accounted for by diverse pathogenic mechanisms. Purely psychological disturbances and psychiatric syndromes with organic components account for the majority of cases. Metabolic disturbances, drug effects, and infections may masquerade for immune-related brain dysfunction. In the absence of reliable and specific indicators of lupus brain activity, successful management requires the combined skills and clinical judgment of the rheumatologist, neurologist, and psychiatrist.

Brain Diseases↗

Cardiac transplantation in female Emery-Dreifuss muscular dystrophy.

A young woman with humeroperoneal muscular dystrophy and contractures received a heart transplant for a severe dilated cardiomyopathy. Cardiac histopathology consisted of myocyte hypertrophy, interstitial fibrosis, and nuclear hyperchromaticity without mitochondrial abnormalities. Myopathy and heart disease were not clinically evident in her family, although three relatives had unexplained shortened Achilles tendons without weakness. Tendon contractures may be a partial expression of this myopathic disorder, suggesting an autosomal dominant inheritance with variable penetrance. A muscular dystrophy clinically similar to that of the Emery-Dreifuss (EDMD) type can thus occur in women. Rather than the cardiac arrhythmias typical of EDMD, a dilated cardiomyopathy may occur and present with severe congestive heart failure. This is the first report of cardiac transplantation in such a case.

Adult↗

Olivopontocerebellar atrophy presenting with hemiparkinsonian ocular motor signs.

A patient with olivopontocerebellar atrophy presented with an asymmetric parkinsonian syndrome consisting of right limb rigidity, bradykinesia, masked facies, and a hypophonic, monotonous voice. Right limb clumsiness suggested corticospinal tract involvement. Gait was slightly wide based, but no other cerebellar or brainstem signs were present. Eye movements were characteristic of parkinsonism with low-gain pursuit and hypometric rightward saccades. Saccadic velocity and the vestibular ocular reflex were within normal limits. There was no pathologic nystagmus or saccadic dysmetria. Magnetic resonance imaging showed cerebellar and lower brainstem atrophy virtually diagnostic of olivopontocerebellar atrophy. Olivopontocerebellar atrophy may present uncommonly with asymmetric parkinsonian features, including parkinsonian eye movement abnormalities.

Humans↗

Brain metastases from undiagnosed systemic neoplasms.

Fifty-six patients presenting with symptomatic brain metastasis but undiagnosed primary neoplasm were retrospectively studied. Metastases were almost equally solitary (57%) as multiple (43%), and 30% were cerebellar. Cerebral metastases were most often parietal (67%). Underlying cancers were diagnosed in 84% of patients, usually before death, with the remainder having equivocal or unknown primary cancers. Lung cancer was most common (68%), especially adenocarcinoma or small ("oat")-cell types, followed by gastrointestinal primary cancers (9%), cancers of the bladder and thyroid (2% each), melanoma (2%), and lymphoma (2%). Breast cancer was remarkably absent, perhaps due to its greater systemic involvement prior to brain metastasis, or its earlier detection on physical examination. Overall group survival rates were 55% (6 months) and 13% (12 months), and cerebellar and noncerebellar metastases had the same survival rate at 12 months. The diagnostic evaluation of these patients, often extensive and costly, should be individually tailored, as 23% had complaints or findings indicative of their underlying primary cancer. Overall, chest roentgenograms and computed tomograms of the chest were the tests of greatest yield, followed by computed tomograms of the abdomen and pelvis. A rationale for evaluation is presented.

Brain Neoplasms↗

The relation of retinal artery occlusion and carotid artery stenosis.

We retrospectively studied 46 patients with symptomatic retinal artery occlusion and assessed the pattern and extent of carotid artery disease ipsilateral to the retinal artery occlusion. Ipsilateral internal carotid artery atherosclerotic lesions were virtually limited to the cervical arterial segment; 50% of such lesions were plaques or stenoses of less than or equal to 60%, whereas 15% of the angiograms were normal. No clinical features were significantly associated with a flow-limiting carotid stenosis of greater than 60%. Contrary to previous reports, the type of retinal artery occlusion, whether branch or central artery occlusion, was not predictive of severe underlying carotid stenosis or occlusion. Likely mechanisms of retinal artery occlusion include in situ thrombosis and emboli from carotid, and possibly cardiac, sources. Extension of thrombus from an occluded carotid artery into the ophthalmic artery did not appear to be a mechanism of retinal artery occlusion.

Adult↗

Adult intrasellar teratoid tumor.

Intracranial teratomas rarely occur in adults. The most common sites are the pineal followed by the suprasellar or hypothalamic areas. Infrequently, teratomas can arise within the sella turcica and mimic a pituitary adenoma or craniopharyngioma. Teratoid tumors contain tissue arising from only two of the three primitive germ layers, whereas teratomas have elements of all three. The following case illustrates the unusual occurrence of an intrasellar teratoid tumor in a 33-year-old man.

Adult↗

Transient Passovoy defect during a febrile illness.

The Passovoy defect is a recently characterized hemorrhagic diathesis. We describe a patient with a febrile illness, possibly from Epstein-Barr (EB) virus, who acquired this defect transiently. Prothrombin time; assays for factors VIII, IX, XI, XII; and Fletcher (prekallikrein) and Fitzgerald (high molecular weight kininogen) factors were normal. No definite circulating inhibitor could be demonstrated. The transient Passovoy defect could possibly be ascribed to the infectious process or sulfisoxazole, which the patient had received.

Adult↗

Long survival of primary cerebral lymphoma with progressive radiation necrosis.

A 56-year-old man lived 8 years after excision and irradiation of a primary cerebral lymphoma. Delayed radiation necrosis caused progressive neurologic deterioration and probably his steroid-responsive episodes of obtundation. Vasogenic edema induced by radiation may account for the latter. An incidental extraneural lymphoma was found postmortem without evidence of CNS lymphoma.

Brain Neoplasms↗

Isolated facial myokymia and facial contracture: computed tomography and magnetic resonance imaging correlation.

Isolated facial myokymia with contracture can be the earliest manifestation of intrinsic lesions of the brainstem. We report a case of facial myokymia with contracture occurring as the result of a pontine glioma, as depicted on cranial computed tomography and magnetic resonance imaging studies. The rostral location of the tumor supports the supranuclear disinhibition hypothesis of facial myokymia.

Adult↗

Painful tonic spasms caused by putaminal infarction.

Lacunar infarcts in the basal ganglia are known to cause various movement disorders, such as chorea, focal dystonia, and hemichorea-hemiballismus. We report here a case of putaminal lacunar infarction which presented with "painful tonic spasms" of the contralateral limbs. This consisted of paroxysmal brief, painful, flexor contractures of the upper, and occasionally the lower limb. These were not focal seizures but were controlled with carbamazepine, which has been used for the "painful tonic spasms" well-associated with multiple sclerosis. The putaminal infarct we describe is probably related to a lupus anticoagulant and systemic lupus erythematosus.

Adult↗

Quantitative sensory thresholds in carpal tunnel syndrome.

Thresholds for vibration and temperature sensation were quantitated at the second and fifth digits of 23 patients (28 hands) with symptomatic, electromyographically confirmed, carpal tunnel syndrome. Thresholds were determined in 23 age-matched controls. Abnormal sensory threshold data confined to the second digit, apparently related to carpal tunnel syndrome, was found in only 3 (11%) of 28 symptomatic hands. There was no apparent relationship between sensory thresholds and the degree of median nerve abnormality as determined by electromyographic nerve conduction studies. Focal demyelination with minimal axonal loss as is seen in mild or moderate carpal tunnel syndrome appears to be more readily detected by electromyographic nerve conduction studies than by quantitative sensory testing.

Adult↗

Aging and quantitative sensory thresholds.

Cutaneous thresholds for vibration and thermal sensitivity were quantitated at the index finger and great toe in 54 subjects without obvious neurological disease. Twelve patients over sixty years of age also consented to standard nerve conduction studies which proved normal. Vibration thresholds progressively increased with age to a significant degree at the finger (p less than 0.05), and especially at the toe (p less than 0.001). Thermal sensitivity thresholds showed no significant relationship with age. These findings confirm the clinical notion that vibration discrimination decreases with normal aging, especially in the toes.

Adult↗