Hypermanganesemia Induced Chorea and Cognitive Decline in a Tea Seller.

Background: Manganese associated neurotoxicity and neurodegeneration is quite rare yet established neurological disorder. This neurotoxic element has predilection for depositing in basal ganglia structures, manifesting mainly as parkinsonian and dystonic movement disorders with behavioral abnormalities. Case report: We report a 40-year-old man who presented with a subacute onset bilateral, asymmetric hyperkinetic movement disorder (predominantly left sided chorea) with multi-domain cognitive impairment, dysarthria, and generalized rigidity. Clinical history and examination yielded multiple differential diagnoses including deposition and metabolic disorders, autoimmune and paraneoplastic encephalitis involving basal ganglia, and neurodegenerative disorders with chorea and cognitive impairment. However, magnetic resonance imaging was suggestive of paramagnetic substance deposition, which came out to be manganese after laboratory investigations. History, clinical examinations, and investigation results pointed towards a diagnosis of acquired hypermanganesemia due to over-ingestion of manganese containing substance (i.e., black tea). He was treated symptomatically and with chelation therapy (calcium disodium edetate). At the sixth month of follow-up, complete resolution of chorea, dysarthria and partial amelioration of rigidity were observed. His cognitive decline and behavioral abnormalities improved. Discussion: This is probably the first reported case of acquired hypermanganesemia that presented as a combination of asymmetric chorea and cognitive dysfunction with atypical imaging characteristics. The clinical picture mimicked that of Huntington's disease. We highlight the potential deleterious effects of an apparently "benign" non-alcoholic beverage (i.e., black tea) on cerebral metabolism.

The neurobehavioural effects of kava.

OBJECTIVE: This review considers the context in which kava is used, together with its underlying psychopharmacological mechanisms, to investigate the neurobehavioural effects associated with kava use. METHOD: We conducted a systematic search using the computerized databases MEDLINE, OVID and PsychLIT for all articles containing any of the following words: kava, kavain, kawa and Piper methysticum. In the opinion of the authors, all articles from this collection containing data that could inform the neurological and cognitive sequelae of kava use were included for the purpose of this review. RESULTS: The use of kava occurs among indigenous populations in the South Pacific and in northern Australia, while also being used throughout the western world as a herbal medicine. Animal studies show that kava lactones alter neuronal excitation through direct interactions with voltage-dependent ion channels, giving rise to kava's muscle relaxant, anaesthetic, anxiolytic and anticonvulsive properties. Several isolated cases of psychotic and severe dystonic reactions following kava use suggest that kava also has psychoactive properties, yet there is no conclusive evidence that kava interferes with normal cognitive processes. CONCLUSIONS: Kava is effective in the treatment of tension and anxiety. There may be risk-factors for severe motor and psychiatric responses to kava use, although these are not well-understood. Given the increasingly widespread use of kava, further investigation is necessary to gain an understanding of its immediate neuropsychiatric effects and long-term cognitive effects.

[Manganese poisoning due to use of Chien Pu Wan tablets]. / Mangaanintoxicatie door het gebruik van Chien Pu Wan-tabletten.

A patient developed a severe chorea whilst taking Chien Pu Wan pills. At examination only a high blood level of manganese (3 times the normal value) was found. Chemical analysis of these Chinese herbal pills showed that each pill contained 14 micrograms of manganese. By taking 3 to 5 pills a day, our patient was receiving 42 to 70 micrograms of manganese over and above the normal absorbed quantity of 60-90 micrograms daily. Because the chorea developed during the use of these pills and resolved when the blood levels of manganese went down, and because the high manganese blood levels were the only abnormality we found, we assumed these Chien Pu Wan pills and the subsequent manganese intoxication to be the cause of the chorea. Manganese poisoning may cause extrapyramidal signs such as parkinsonism, dystonia and chorea. This form of alternative therapy is not yet subject to legislation. In order to be able to control the nature and (side) effects of this kind of therapy, legislation is required.

[The role of lipid peroxidation in the pathogenesis of neuropathological syndromes and in experimental therapy]. / Rol' perekisnogo okisleniia lipidov v patogeneze nekotorykh neiropatologicheskikh sindromov i pri éksperimental'noi terapii.

Activated lipid peroxidation observed in brain homogenates in experimental choreoid hyperkinesis and bemegride epilepsy can be arrested by antioxidant pretreatment. The same is true for parkinsonian and "malignant locomotion" syndromes provoked by intrastriatal pro-oxidant (oxidized oleinic acid) microinjection and systemic administration of haloperidol. Experimental therapy of choreoid hyperkinesis with GABA-positive and dopamine-blocking drugs alone proved ineffective. The effect was achieved at combined application of antioxidant and GABA-positive drugs.

Thalamotomy for the alleviation of levodopa-induced dyskinesia: experimental studies in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-treated parkinsonian monkey.

This work set out to test the hypothesis that thalamotomy in the area of the thalamus which receives the input from the medial segment of the globus pallidus would decrease or prevent levodopa-induced dyskinesia. Peak dose dyskinesia is a major problem in the treatment of parkinsonian patients with levodopa therapy but this remains the best pharmacological agent for treating the condition. The hypothesis was derived from previous work which has suggested that reduced pallidal inhibition of the thalamus results in dyskinesia [Crossman (1990) Movement Dis. 5, 100-108]. A neuroanatomical tracing study was carried out prior to the thalamotomy work, using the anterograde tracer wheatgerm-agglutinin conjugated to horseradish peroxidase. This delineated the anterior part of the ventrolateral thalamus in the primate in terms of its afferent inputs. Wheatgerm agglutinin-horseradish peroxidase was injected into the medial segment of the globus pallidus bilaterally in three Macaca fascicularis and traced to terminals in the ventral thalamus and other brain areas. The appropriate thalamic area involved was plotted on atlas sections in preparation for stereotactic thalamotomy. Previous studies of neuronal input to the ventral thalamus are confusing due to the different nomenclatures used by different workers. Early workers used cytoarchitectonic boundaries which do not correspond with function. There are also differences in nomenclature between man, monkey and other animals. The current study maps the pallidal terminal territory within the thalamus in terms of stereotactic co-ordinates related to a published macaque atlas [Shantha et al. (1968) A Stereotaxic Atlas of the Java Monkey Brain. S. Karger, Basel] and can thus be used by other workers in the field. A well-established primate model of Parkinsonism was used for the thalamotomy study. Eight monkeys (Macaca fascicularis) were rendered parkinsonian with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Regular dosing with levodopa or apomorphine reliably resulted in peak-dose dyskinesia which was scored in terms of its choreic and dystonic components. A radiofrequency electrode was used to create the ablative lesions. Chorea was always reduced and frequently abolished by a thalamotomy located in the pallidal terminal territory. This result was obtained after 10 thalamotomies in a total of six animals. Four animals received bilateral lesions, with an interval between operations and two animals underwent unilateral surgery.(ABSTRACT TRUNCATED AT 400 WORDS)

Subcortical changes in the regional uptake of [3H]-2-deoxyglucose in the brain of the monkey during experimental choreiform dyskinesia elicited by injection of a gamma-aminobutyric acid antagonist into the subthalamic nucleus.

Hemichorea/hemiballismus was induced in monkeys by localized injections of a gamma-aminobutyric acid antagonist into the contralateral subthalamic nucleus. During active dyskinesia, [3H]-2-deoxyglucose was administered and, subsequently, regional cerebral metabolic activity was examined by autoradiographic exposure of brain sections. The results indicate that during dyskinesia there was an overall decrease in local cerebral glucose utilization in a number of structures on the side of the brain contralateral to the abnormal movements (ipsilateral to the drug injection). These structures included the injected subthalamic nucleus, both medial and lateral segments of the globus pallidus, the substantia nigra, and the ventral anterior and ventral lateral nuclei of the thalamus. On the basis of evidence that changes in the energy requirement of neurons are due mainly to changes in synaptic activity, the autoradiographic findings are interpreted as indicating that during experimental hemichorea/hemiballismus there was an overall decrease in synaptic activity of subthalamopallidal, subthalamonigral and pallidothalamic pathways on the side of the brain contralateral to the dyskinesia. This interpretation is discussed in relation to current theories of the pathophysiology of choreiform dyskinesias.

[Morvan's fibrillary chorea and acrodynic syndrome following mercury treatment]. / Chorée fibrillaire de Morvan et syndrome acrodynique après un traitement mercuriel.

A 31 year-old inhabitant of French Guiana was prescribed mercuric iodide per os for two and a half months. Shortly before the end of the treatment he developed fasciculations in the trunk and particularly the lower limb muscles, distal painful paresthesias with vasomotor disorders, episodes of excessive perspiration and palmoplantar erythema, moderate fluctuating hypertension, progressive loss of weight and irritability with insomnia. Clinical and electrical signs of neuropathy were lacking. The clinical picture was that of Morvan's fibrillary chorea with acrodynia, the conditions of onset strongly suggesting a mercurial intoxication. Blood and particularly urine mercury levels were elevated. Administration of dimercaprol (BAL) considerably increased urinary excretion of mercury and there was progressive improvement and finally recovery after two months of BAL treatment. This case exemplifies the possible co-existence of fibrillary chorea and acrodynia. Whereas in many cases of fibrillary chorea a precise etiology cannot be determined, the affection can be induced by mercury as by gold administration. The fact that cases of fibrillary chorea due to mercury poisoning are rarely reported may be the result of individual patient hypersensitivity or particular metabolic absorption and excretion features of mercury. This case cannot be included within the continuous activity syndrome of muscle fibers described by Isaacs, since muscle contractures were absent and there was associated acrodynia. Moreover, there was no latent polyneuropathy, in spite of the intense fasciculations. It must be concluded, therefore, that in spite of its rarity fibrillary chorea should keep its semiologic autonomy.

Tetraethyl lead poisoning from gasoline sniffing.

In two cases of organic lead poisoning due to habitual gasoline sniffing, one patient had temporary hypomania and recovered with treatment, while the other patient (who died) had signs of severe CNS and peripheral nervous system, muscle, hepatic, and renal damage. In addition he had features of long-term inorganic lead poisoning. This and the response of both cases to chelating agents suggest that organic lead is degraded in vivo to inorganic lead.