Gene/Protein Disease Symptom Drug Enzyme Compound
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Partial ophthalmoplegia due to third nerve palsy with an intact pupil is a frequent cause of diploplia observed in diabetic patients. Pupillary muscle involvement, such as anisocoria and loss of light reflex, is usually uncommon in this diabetic cranial mononeuropathy. A 65-year-old woman with non-insulin dependent diabetes mellitus (NIDDM) suddenly developed a severe headache and diplopla. Right oculomotor nerve palsy was observed in association with anisocoria, ptosis of the right lid, and a defective light reflex. No exophthalmos or vascular bruit was observed in the right orbital region. Computed tomography and magnetic resonance images of the head were negative. Cerebral angiography revealed a carotid cavernous sinus fistula (CCF). The patient was successfully treated with external carotid artery embolization combined with radiation. It is well known that pupil sparing in oculomotor nerve palsy predicts an extraaxial ischemic lesion, while pupil involvement predicts an extraaxial compression lesion. Therefore, pupillary involvement in oculomotor nerve palsy in diabetic patients necessitates cerebrovascular investigation to rule out ICPC aneurysm or tumor. In this circumstance, a variant type of CCF without characteristic ocular signs should be included in the differential diagnosis.
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PMID:[A case of NIDDM associated with oculomotor palsy due to atypical carotid cavernous sinus fistula]. 827 44

We studied 6 patients and 2 dogs that have been bitten by South American rattlesnake Crotalus durissus terrificus and one rabbit inoculated with crotalid venom. We analyzed sensory and motor peripheral nerve conduction, repetitive stimulation for studying neuromuscular transmission and electromyographies. Muscle biopsies were processed by histochemistry. All patients had peripheral mononeuropathy of the closest sensitive nerve to the area of snakebite. The neuromuscular transmission alterations were minimal. Muscle histochemistry of 4 patients, 2 dogs and 1 rabbit showed findings of mitochondrial myopathy. The majority of authors admit that crotalid venom causes myastenic syndrome. Our findings suggest that palpebral ptosis, myastenic facies and muscular weakness observed after crotalid poisoning are, probably, due to transient and reversible mitochondrial myopathy. As far as we know, this is the first report on the ability of the venom of this rattlesnake to cause local sensitive mononeuropathy and the first muscle histochemistry showing mitochondrial myopathy in humans poisoned by crotalid venom.
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PMID:[Neuromuscular action of crotalid venoms: preliminary data]. 873 37

Twenty-three diabetic patients -- 16 men and seven women (mean age: 50.7 +/- 17.4 years; mean duration of diabetes: 13.6 +/- 6.9 years) -- with diabetic mononeuropathy of the cranial nerves participated in the study. Four of them were with mononeuropathia multiplex and total ophthalmoplegia, affecting the oculomotor, trochlear and abducent nerves; 12 with paresis of the oculomotor nerve, one -- of the trochlear nerve and six -- of the abducent nerve. They were treated with alpha-lipoic acid (600 mg) for 10 days daily intravenously, thereafter one film tablet of 600 mg daily for 60 days. On the 10th day, we found significant improvement in the clinical signs of diabetic mononeuropathy - double vision, motility and position of the eyeball, ptosis of the upper eyelid and mydriasis. The mean period of oral treatment was 69.1 +/- 23.8 days, following the 10-day intravenous application of alpha-lipoic acid, and full recovery of the diabetic mononeuropathy was achieved with this therapeutic approach. Peripheral neuropathy was present in 17 patients (74%). On the 10th day, we established a decrease in total symptom score by an average of 2.7 +/- 1.4 points and by the end of the treatment period it was improved by 5.9 +/- 1.9 points (p = 0.04). On the 10th day, we found a decrease of 33% in foot pain and by the end of the second month, it fell by 65.5% (p < 0.0001). Vibration perception threshold was reduced in these patients at entry -- mean: 2.42 +/- 1.8 at the great toe, 2.89 +/- 1.8 at the first metatarsal and 3.65 +/- 1.7 at the medial malleolus. By the end of the second month, it reached mean 4.7 +/- 1.8 (p < 0.002) at the great toe, 4.92 +/- 2.1 (p = 0.004) at the first metatarsal and 5.3 +/- 1.4 (p < 0.01) at the medial malleolus. Cardiovascular autonomic neuropathy was present in two of the patients and there was improvement after treatment in the Ewing's tests -- Valsalva manoeuvre, deep-breathing test and lying-to-standing test. The results of our study demonstrate that alpha-lipoic acid appears to be an effective drug in the treatment for not only peripheral and autonomic diabetic neuropathy, but also diabetic mononeuropathy of the cranial nerves leading to full recovery of the patients.
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PMID:Treatment for diabetic mononeuropathy with alpha-lipoic acid. 1592 91