Tick paralysis in Australian birds caused by Ixodes holocyclus.

Tick paralysis is an uncommon cause of neuromuscular paralysis affecting 0.12% of wild birds presented to Currumbin Wildlife Sanctuary, Queensland, with a strong seasonal predilection towards spring and summer. Clinical signs and progression of paralysis showed similarities to companion animals and were consistent across 20 species. Tick location, number of engorged ticks and number of clinical signs did not affect the outcome; however, all mortalities occurred within 4 days of admission. Treatment with canine-derived tick antiserum resulted in clinical improvement within 24 h and a recovery rate of 73%. Average time to resolution of clinical signs was 4.3 days, with juvenile birds recovering more quickly than adults. The treatment and release of wild birds affected by tick paralysis are both achievable and rewarding, further research is required to establish treatment guidelines in birds.

[Two cases of urinary retention secondary to aseptic meningitis].

We report two rare cases of urinary retention secondary to aseptic meningitis. Case I was in a 13-year-old boy admitted to the pediatric department due to aseptic meningitis. Eight days after his admission, urinary retention developed and cystometry showed atonic bladder. Case 2 was in a 18-year-old woman consulted the urological department with a chief complaint of urinary retention accompanied with high fever, headache and vomiting. The spinal fluid examination and cystometry revealed aseptic meningitis and atonic bladder, respectively. In both cases, the patients were treated with conservative therapy and bladder dysfunction was resolved after a few weeks. Eleven cases of urinary retention secondary to aseptic meningitis have been reported in the previous literature.

Cerebral oxygen and glucose metabolism in glycogen storage disease with normal acid maltase: case report.

A 26-year-old male with cardiomyopathy, cervical muscle weakness and mental retardation was diagnosed as having glycogen storage disease with normal acid maltase on the basis of his clinical, pathological and biochemical findings. Positron emission tomography showed that cerebral oxygen metabolism was normal, while cerebral glucose metabolism was decreased in the cerebral cortexes. The decrease of the glucose metabolic rate may reflect an abnormality of cerebral glucose metabolism in this disorder and may be related to mental retardation, which is one of the characteristic symptoms.

Assessment of autonomic function in myotonic dystrophy by spectral analysis of heart-rate variability.

We analyzed by means of autoregressive spectral analysis the spontaneous beat-to-beat heart-rate variability (HRV) of 10 myotonic dystrophy (MD) patients (4 men and 6 women, aged 37-53 years) and 10 age- and sex-matched healthy, sedentary humans (control) at rest in the supine position. All MD patients had no cardiac conduction disturbances (i.e., atrioventricular or intraventricular conduction defects) on 12-lead electrocardiogram and were able to walk and perform daily activities. In the MD group, the total power, the power of the low-frequency component (a marker of sympathetic and vagal modulation of heart rate) and that of the high-frequency component (a marker of vagal modulation of heart rate) were smaller than those in the control group (P < 0.01, P < 0.05 and P < 0.05, respectively). The results of this study suggest that the cardiovascular autonomic nervous system contributing to the HRV may be disturbed even in the MD patients who can walk and perform daily activities. Therefore, one must give careful consideration to the cardiovascular autonomic dysfunction, as well as the cardiac conduction disturbance in the MD patients.

[A case of glycogen storage disease with normal acid maltase accompanied with the abnormal platelet function].

Glycogen storage disease with normal acid maltase first reported by Danon et al. was characterized clinically by mental retardation, cardiomyopathy, and proximal myopathy. Since the first report, 17 patients have been reported including 5 patients from Japan. In this paper we described a 26-year-old man who had dilatated cardiomyopathy with a pacemaker implanted at age 22 years. He was admitted to our hospital complaining of easy fatigability in February 1992. Neurological findings showed that he had mental retardation. Serum CK, GOT, GPT and aldolase levels were elevated. Histopathological study of biopsied skeletal muscle showed intracytoplasmic vacuoles with increased acid phosphatase and slightly increased PAS positive material. Electron microscopic study revealed numerous glycogenosomes (autophagic vacuoles containing glycogen). These pathological findings were similar to acid maltase deficiency, but activities of carbohydrate metabolic enzyme including acid maltase activity were normal in the biopsied muscle. From these results, he was diagnosed as having glycogen storage disease with normal acid maltase. We also found abnormal platelet function and glycogen accumulation in the platelets, which have not been previously described. The disease is probably a systemic disorder affecting not only skeletal and cardiac muscles, but platelets.

[A case of Listeria rhombencephalitis with a secondary vasculitis suggested by MRI].

We reported a rare case of Listeria rhombencephalitis with meningitis. A 48-year-old healthy man suddenly experienced high fever and headache, then he had lower cranial nerve's palsies and mental dysfunction developed during one week period. On admission, his temperature was 38 degrees C. He was slightly delirious and euphoric. He had nuchal rigidity, mild paresthesia over his left cheek to left upper lip, a right sixth nerve palsy, dysphagia, hiccup, nasal voice and left cerebellar ataxia. His tongue deviated toward the right side on protrusion. A CSF culture grew Listeria monocytogenes. Intravenous antibiotic therapy (PIPC, minocycline hydrochloride) produced improvement in one month except for mild paresthesia and dysphagia. He almost recovered after 7 months of illness. Brain MRI on T2 weighted image demonstrated multiple small ischemic lesions in the left lateral medulla, upper pontine tegmentum in the right side, and pontine tegmentum in the left side. These lesions enhanced by Gd. were assumed to be due to the secondary vasculitis. Listeria rhombencephalitis is extremely rare in human beings. To our knowledge only thirteen cases have been reported. In seven cases, post-mortem pathological findings confirmed necrotizing angitis in brainstem. Clinical aspects of Listeria rhombencephalitis were discussed, and the entity of this disease should be considered as a treatable cause of acute progressive brainstem meningoencephalitis.

[A case of recurrent multiple cerebral infarctions with protein C abnormality].

A 38-year-old man of recurrent episodes of multiple cerebral infarctions with protein C abnormality was reported. He had six episodes of visual disturbance within a year. The neurological examination revealed bilateral visual loss and impaired visual field except for small central area. However, there were no abnormalities in the deep tendon reflexes and muscle strength of all extremities and cerebellar examinations. CT scan revealed multiple low density areas in the bilateral posterior lobes, and cerebral angiography demonstrated the severe stenosis of the ganglionic portion and the supraclinoid portion of the right internal carotid artery. Plasma levels of antithrombin-III activity, plasminogen activity, protein C activity, protein C antigen and protein S antigen were 105, 106, 28, 166 and 120%, respectively. Because only protein C activity decreased markedly, a diagnosis of protein C abnormality was established. We started warfarin therapy under the administration of concentrates of factor IX complex, since then there has been no cerebral accident.

Variations of activities in the segments of respiratory chain among tissues in a patient with mitochondrial encephalomyopathy.

Enzymatic activities of NADH cytochrome c reductase and cytochrome c oxidase were determined in the mitochondria from various tissues of a patient with mitochondrial encephalomyopathy and compared with those of controls. NADH cytochrome c reductase in the present patient decreased significantly in the liver and spleen and to a less extent in the kidneys. On the other hand, cytochrome c oxidase of the patient decreased severely in the skeletal muscle and kidneys and partially in the heart. Difference spectrum of reduced-minus oxidized form of mitochondria from patient's skeletal muscle and heart showed a decrease of cytochrome aa3 peak in the alpha region at 605 nm. These results indicate that there are cryptic deficiencies in the segments of the respiratory chain in the mitochondria from several tissues of the present patient, such as liver, kidney, spleen without any clinical manifestation. The weakness and atrophy of skeletal muscle was, however, well correlated to the biochemical analysis.