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Biomedical subjects

N Charles

Publications and source records attributed to N Charles.

31 records · Page 2Linked to original sources

Korsakoff's syndrome as the initial presentation of multiple sclerosis.

A 37-year-old man presented with an acute amnestic syndrome of Korsakoff's type and an upper brain-stem oculomotor syndrome. After a moderate improvement with steroid therapy, he developed progressive behavioural changes due to a frontal lobe syndrome, in addition to motor and visual impairment. Memory performance was investigated on several occasions during an 11-year follow-up. Diagnosis of laboratory-supported definite multiple sclerosis was established and magnetic resonance imaging showed disseminated white matter lesions, especially in both medial temporal lobes. No other cause than multiple sclerosis was found for the amnestic syndrome. Among the rare cases of Korsakoff's syndrome in the course of multiple sclerosis, this is to our knowledge the first case in which a memory deficit was the initial manifestation of the disease.

Adult↗

Clinical validation of antidromic stimulation of the ring finger in early electrodiagnosis of mild carpal tunnel syndrome.

Median and ulnar sensory distal latencies were measured antidromically on the fourth finger in 158 patients (224 hands) with suspected carpal tunnel syndrome (CTS), in 60 normal subjects (100 hands), and in 30 patients (30 hands) who suffered from paresthesiae due to a cervical spondylotic radiculopathy (CSR). The difference between these 2 latencies was less than 0.4 msec in all normals and patients with CSR, while in all of the patients with CTS it was more than 0.5 msec. Median sensory nerve conduction was significantly slower for the fourth than for the second finger in the CTS group, but not in controls. The difference between median and ulnar sensory distal latencies on the fourth finger proved to be the most sensitive of the tested parameters and was the only abnormal one in 20% of the clinically affected hands. The ring finger technique is a quick and easy procedure, which should be recommended in the early diagnosis of mild CTS.

Adult↗

Fulminant myasthenia gravis soon after initiation of acebutolol therapy.

We report the case of myasthenia gravis in a 74-year-old woman, 2 weeks after acebutolol had been introduced as the sole treatment for systemic hypertension. The disease developed at a fulminant and fatal course despite drug withdrawal. The possibilities of coincidental association between the two events, triggering of a preexisting latent myasthenia gravis or full induction of the disease by acebutolol, are discussed.

Acebutolol↗

[Dementia and psychiatric disorders in Kufs disease].

Three patients with adult-onset neuronal-ceroid lipofuscinosis (Kufs' disease) are presented. Two cases were familial (autosomal recessive inheritance) and 1 case was possibly sporadic. The main clinical feature was progressive deterioration of cognitive functions. In 2 siblings, aged 37 and 41, dementia was associated with personality and behaviour changes, suggesting a psychotic disorder and with dysarthria and tic-like dyskinetic movements. In a third patient, dementia was only associated with an asymptomatic pigmentary retinal degeneration. CT scan revealed diffuse cerebral atrophy in all cases. Diagnosis was established by brain biopsy in 2 cases. Autofluorescence emission and absorption spectra from the abnormal pigment was studied and was not found contributive.

Adult↗

A rare case of 68,XX triploidy diagnosed by amniocentesis.

68,XX triploidy was found in the amniotic fluid cell culture of a 40-year-old patient. Elective termination of the pregnancy revealed a fetus with multiple congenital anomalies. While this case does show some common features with monosomy X, a greater similarity to the triploidy syndrome is observed.

Abnormalities, Multiple↗

Bombesin and nutrients stimulate release of CCK through distinct pathways in the rat.

An isolated, vascularly perfused duodenojejunum model was developed in the rat to investigate the mechanisms involved in the release of cholecystokinin (CCK) by vascular bombesin (BBS) and luminal nutrients (LN). Immunoreactive peptides released into the portal vein effluent were measured with three antisera that were relatively CCK specific, gastrin specific, and cross-reactive to both CCK and gastrin, respectively. Bombesin (10(-7) M) provoked a biphasic release of CCK, consisting of a transient rise (approximately 700% above basal) followed by a sustained response (approximately 250% above basal). Gastrin accounted for less than 25% of the total immunoreactivity as measured with the nonspecific antiserum. The two phases of the CCK response were related to the dose of BBS in the range 10(-10)-10(-6) M, with a half-maximal effect at 10(-8) M BBS. Tetrodotoxin (TTX, 10(-6) M) reduced the second phase only (by 80%), whereas hexamethonium (10(-4) M) and atropine (10(-5) M) left the two phases unaltered. LN induced a prompt and well-sustained release of CCK (approximately 250% above basal) that was not affected by arterial TTX or atropine. These results demonstrate that BBS stimulated the release of CCK through both a direct pathway and an indirect, noncholinergic mechanism, while the effect of LN did not appear to involve intramural nerves.

Animals↗

Retinal and optic nerve findings in Goldenhar-Gorlin syndrome.

Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more common than is generally appreciated. We examined seven patients with this syndrome. Abnormalities included diminished visual acuity, tilted optic disc, optic nerve hypoplasia, tortuous retinal vessels, macular hypoplasia and heterotopia, microphthalmia and anophthalmia. In one case, pathologic study showed agenesis of the optic nerve. It is proposed that retinal, optic nerve and craniofacial abnormalities in this condition may reflect an asynchrony in the migration of the neural crest cells in the early stages of embryonal development.

Adolescent↗

First-aid for snake-bite: efficacy of a constrictive bandage with limb immobilization in the management of human envenomation.

A herpetologist was bitten on the thumb by a common brown snake (Pseudonaja textilis). A constrictive bandage to impede lymphatic and capillary flow was applied, and the upper limb was immobilized. Two hours after the bite, there were no signs of symptoms of envenomation and venom (to a sensitivity of 0.5 ng/mL) was undetectable in serum and urine. Within five minutes of removal of the constrictive bandage, significant signs of envenomation developed, and serum and urine levels of venom rose significantly. The patient received two ampoules of brown-snake antivenom, and had recovered within six hours. No anaphylaxis of other allergic phenomena occurred, despite the fact that three other doses of antivenom had been administered in the preceding 36 months for prior elapid envenomation. By means of an experimental whole-mouse technique, and an enzyme-linked immunospecific assay (ELISA) system, the snake involved was shown to deliver 4.91 mg of venom in an average bite. A constrictive bandage properly applied to impede lymphatic and capillary flow, together with limb immobilization, is effective in the field management of human elapid envenomation.

Animals↗

Attitudes of patients after "genetic" termination of pregnancy.

Twelve of 15 women who had had abnormal fetuses agreed to be interviewed after termination of the pregnancy. The survey showed that persistent adverse psychological and social reactions may be much commoner in patients undergoing termination of pregnancy for genetic rather than "social" indications. Adequate information and counselling before, and support after, termination of an abnormal pregnancy are essential if these women are to cope with the experience.

Abortion, Eugenic↗

[Hemiballismus with logorrhea and thymo-affective disinhibition caused by hematoma of the left subthalamic nucleus].

A 71 year-old hypertensive right-handed man presented a small intracerebral haematoma limited to the left subthalamic nucleus that resulted in right hemiballismus and a neurobehavioral disinhibition syndrome. The latter associated logorrhoea and euphoria, but there was no frontal signs nor mental confusion. The patient kept full insight into his mental change, that contrasted with his prior personality. Hemiballismus and the manic-like state resolved within 2 months. This case and some others from the literature illustrated that a focal lesion in the basal ganglia could result in manic-like affective change, and that a relation did exist between hyperkinetic syndrome and euphoria. Our case contrasted with most of the published cases of neurobevioral changes following basal ganglia discrete lesions, expressing as hypokinesia, apathy and loss of motivation. With reference to the functional circuits linking the frontal cortex and the basal ganglia, as described by Alexander, this syndrome of combined disinhibition of motricity, speech and affect could involve either the lateral orbito-frontal or the anterior cingulate loops, more especially the indirect pathways travelling through the subthalamic nucleus, lesion of which is known to disinhibit thalamo-cortical activity.

Aged↗

Snakebite in herpetologists.

OBJECTIVES: To define the risk of snake envenomation in herpetologists, assess factors which lead to their being bitten, assess their occupational morbidity and propose preventive stratagems. SUBJECTS AND DESIGN: Interview of 14 of the most experienced professional herpetologists in Queensland; and questionnaire to 14 members of the Cape York Peninsula Herpetological Society, Cairns, Queensland. RESULTS: The 28 herpetologists had sustained 119 bites by potentially dangerous species, and hundreds of clinically insignificant bites. Seventeen had been admitted to hospital. Only five had never been bitten by a medically dangerous snake and 14 had been bitten on two or more occasions. Correct first-aid techniques were applied in less than half the bites. Two had permanent morbidity--renal damage, and permanent tissue damage to the hands. Anaphylactic reactions from the repeated use of antivenom did not occur. The experienced herpetologists sustained a life-threatening bite every 10 years (median). Four herpetologists had been bitten by the western taipan (Oxyuranus microlepidotus), the world's most venomous snake. CONCLUSIONS: Herpetologists working with highly venomous snakes are at high risk. Prevention involves recognising the risk; possessing a first-aid certificate; applying standard first-aid if a bite is sustained; carrying a mobile telephone; and not working alone.

Animals↗

Assessment of motor conduction times using magnetic stimulation of brain, spinal cord and peripheral nerves.

Central conduction times (CCTs) in the motor pathways were assessed in normal subjects using a magnetic stimulus for the recording of cortical and spinal (Cv7 and L4-L5) Motor Evoked Potentials (MEPs) as well as F-waves in upper and lower limbs. M-responses of maximal amplitude could be reliably obtained by using a 4.5 Tesla coil and a stimulus intensity of 85% of the maximal output. It was found that the CCTs derived from the latency difference between cortical and spinal MEPs were, on average, 0.90 ms and 3.15 ms longer, respectively for upper and lower limbs, than those obtained by combining cortical MEPs and F-wave latency measurements. This difference is similar to that reported when electric stimulation is used to elicit F-waves and/or spinal MEPs. It suggests that motor root fibers are depolarized at a distance of 7.8 and 17 cm from the cord by a spinal magnetic stimulus applied respectively at Cv7 and L4-L5 levels. This study shows that magnetic stimulation alone permits to assess CCT in motor pathways with the same reliability as any of the other stimulation modalities hitherto proposed in literature.

Adult↗