Tau protein concentrations in cerebrospinal fluid of patients with amyotrophic lateral sclerosis.

OBJECTIVE: To elucidate whether cerebrospinal fluid (CSF) concentrations of the microtubule-associated tau protein are related to the risk for sporadic amyotrophic lateral sclerosis (SALS). PATIENTS/METHODS: We measured tau concentrations in the CSF of 18 patients with SALS and 75 age- and sex-matched controls, using a specific ELISA method. RESULTS: The mean CSF concentrations of tau protein did not differ significantly between SALS patient and control groups, were not influenced by the clinical form (spinal vs bulbar) of ALS, and were not correlated with age, age at onset, and duration of the disease. CONCLUSIONS: CSF tau concentrations are not a biochemical marker of ALS.

[Bilateral hand dystonia secondary to a bilateral opercular syndrome or Foix-Chavany-Marie syndrome]. / Mano distónica bilateral secundaria a síndrome biopercular o síndrome de Foix-Chavany-Marie.

INTRODUCTION: The main clinical feature of the opercular syndrome (Foix Chavany Marie) is the automatic voluntary dissociation of the facio glosso pharyngeal movements (that is, the alteration of voluntary motility with preservation of authomatic movements). Less frequently, it is presented with movement disorders as dystonia. CASE REPORT: We report a male patient aged 40 years who developed a biopercular syndrome of vascular etiology (confirmed by neuroimaging), in the context or a clinical picture of global hipoxemia, for which the most outstanding clinical manifestation was the presence of dystonic posturing. CONCLUSION: Although dystonia is usually related with damage or dysfunction of the basal ganglia or thalamus, in some case it can be caused by lesions in other locations, such as in some patients with biopercular syndrome as in the present case

Mano distónica bilateral secundaria a síndrome biopercularo síndrome de Foix-Chavany-Marie / Bilateral hand dystonia secondary to a bilateral opercular syndrome or Foix-Chavany-Marie syndrome

Introducción. La principal característica clínica del síndrome opercular (Foix-Chavany-Marie) es la `disociación automática voluntaria' de los movimientos faciogloso-faríngeos (alteración de la motilidad voluntaria con preservación de los movimientos automáticos). Raramente cursa con trastornos del movimiento, como la distonía. Caso clínico. Presentamos un varón de 40 años que desarrolló un síndrome biopercular de etiología isquémica (que se confirmó con pruebas de neuroimagen), en el contexto de un cuadro de hipoxemia global, cuya manifestación clínica más llamativa era la presencia de posturas distónicas. Conclusión. Aunque la distonía suele relacionarse con la lesión o la disfunción de los ganglios basales o del tálamo, en algunos casos puede producirse por lesiones en otras localizaciones, como en algunos pacientes con síndrome biopercular, como el que presentamos (AU)

[Do antioxidants in the diet affect the risk of developing Parkinson disease?]. / Influyen los antioxidantes de la dieta en el riesgo para desarrollar enfermedad de Parkinson?

OBJECTIVE: A number of studies suggest the existence of 'oxidative stress' in the substantia nigra from parkinsonian patients. If 'oxidative stress' should be relevant in the pathogenesis of Parkinson's disease (PD), the consumption of antioxidant or prooxidant substances in the diet could theoretically influence the risk for this disease. DEVELOPMENT: A critical up to date review of the literature regarding premorbid consumption of antioxidants or prooxidants by PD patients and controls has been done. Most studies have been retrospective, they have been performed following different designs, and disclosed contradictory results. CONCLUSION: From the current literature, it is unlikely that dietetic consumption of prooxidants and antioxidant, specially vitamin E (the most frequently studied antioxidant) have any influence on the risk for PD.

[Brown-Séquard syndrome as the initial clinical manifestation ofmultiple sclerosis]. / Síndrome de Brown-Séquard como primera manifestación clínica de esclerosis múltiple.

Multiple sclerosis is a disorder with different forms of clinical presentation. The Brown-Séquard syndrome has occasionally been described in association with multiple sclerosis. We present the case of a patient whose initial clinical presentation of multiple sclerosis was the Brown-Séquard syndrome.

Cerebrospinal fluid carnitine levels in patients with Alzheimer's disease.

We assessed free carnitine (FC) and acylcarnitine esters (AC) in both cerebrospinal fluid (CSF) and plasma from 24 patients with diagnostic criteria for Alzheimer's disease (AD), and from 28 healthy matched-controls. We found no significant correlation between FC and AC levels in CSF. FC and AC levels in CSF did not differ significantly between AD patients and controls, but plasma FC levels were significantly lower in AD patients. CSF and plasma FC and AC levels did not correlate with age, age at onset of AD, duration of AD, and scores of the Minimental State Examination of Folstein. Although these results suggest that CSF carnitine levels are apparently unrelated with the risk for AD, the trend of the FC/AC ratio to be higher in AD patients might suggest the possibility of a lower carnitine acetyltransferase activity in AD, as previously reported in some brain areas.

Bilateral primary writing tremor.

Primary writing tremor is a task-specific tremor that is considered to be unilateral. We report a 59-year-old man with a 5-year history of a typical primary writing tremor in the right hand who developed similar symptoms in the left hand. Copyright 1998 Lippincott Williams & Wilkins

Premorbid dietetic habits and risk for Parkinson's disease.

To evaluate the possible role of antioxidant (vitamins A, E, C, beta-carotene, zinc, selenium) or pro-oxidant (iron, copper, manganese) factors present in the diet, in the risk for Parkinson's disease (PD), we conducted a dietetic study (pre- and postmarriage) which included 91 foodstuffs. We interviewed separately 119 PD patients (64 males, 55 females, age 65.3 +/- 8.5 years, age at marriage 27.8 +/- 5.9 years) and their spouses (age 65.0 +/- 9.2 years, age at marriage 27.2 +/- 5.9 years) as the control group (C). Premarriage consumption for each foodstuff was classified into: (1) rarely of annually, (2) monthly, or (3) weekly; and postmarriage consumption (which included the period between age at marruage and age 40 years; only concordant answers were considered to be valid) into: (1) PD > Control, (2) Control > PD, and (3) PD = Control. The premarriage interview showed a trend towards a lower consumption of peas in PD patients as compared to controls. The postmarriage interview in PD patients showed a higher tendency for the consumption of rice, bluefish, liver paté and eggs, and a less consumption of tea than controls. We were unable to find any consistent trend towards a lower consumption of vitamins E and C during adulthood in PD patients, but they seemed to have eaten selenium-rich foodstuffs more frequently than controls. These results differ from those obtained by previous studies, and question the possible role of premorbid dietary habits on the risk for PD.

Clinical features of essential tremor seen in neurology practice: a study of 357 patients.

To assess the clinical features of essential tremor (ET), we studied 357 patients with diagnostic criteria for this disease who were referred to the Neurology departments of three urban hospitals. This is not a true epidemiological study, and can be biased in favour of patients with functional disability. There was a predominance of affectation of females and a peak of age at onset in the sixth and seventh decades. Family history of tremor was positive in 46.8% of patients. Females showed a a significantly higher frequency of inheritance through maternal than through paternal line, and had a higher frequency of head and voice tremor, than males. When compared with patients without family history, those with family history of tremor had lower age at onset of tremor, and lower frequency of jaw and trunk tremor in non-familial cases.

Cerebrospinal fluid nitrate levels in patients with Alzheimer's disease.

It has been suggested that nitric oxide could be implicated in the pathogenesis of Alzheimer's disease (AD). Recently Kuiper et al. reported decreased CSF nitrate levels (oxidation product that provides an indirect estimation of nitric oxide) in AD patients, assessed with a colorimetric method. However other group, using a microplate version of the Griess reaction, did not confirm these findings. We studied the CSF and plasma levels of nitrate with kinetic cadmium-reduction method in 32 AD patients and 36 matched controls. The CSF and plasma nitrate levels did not differ significantly between the two study groups. CSF and plasma nitrate levels did not correlate with age at onset and duration in the patient group. These data suggest that CSF and plasma levels of nitrate are apparently unrelated with the risk for AD.

Drug-induced parkinsonism in a movement disorders unit: A four-year survey.

UNLABELLED: To establish the frequency of drug-induced parkinsonism (DIP) and the drugs responsible for this side-effect we reviewed the database of our Movement Disorders Unit during the first 4 years of its use. The diagnostic criteria for DIP included: (1) the presence of two or more cardinal symptoms of parkinsonism, (2) an absence of parkinsonian symptoms before the exposure to the offending drug, (3) a disappearance or significant improvement in parkinsonism after withdrawal of the offending drug, (4) no better explanation for the parkinsonism. One-hundred and five patients fulfilled the diagnostic criteria for DIP (16.3% of total patients referred and 33.8% of patients with parkinsonian syndromes). Drug-induced parkinsonism was related to 1, 2, 3, 4, 5 and 7 drugs in 62, 30, 9, 1, 2 and 1 patients, respectively. The most frequently offending drugs were: calcium-channel blockers (61 cases), antipsychotic drugs (29 cases), thiethylperazine (18 cases), clebopride (14 cases), and sulpiride (10 cases). When compared with idiopathic Parkinson's disease patients, DIP patients were predominantly female and showed an older age at the onset of parkinsonian signs. Parkinsonian signs only disappeared completely in 41 patients (39.0%). IN CONCLUSION: (1) DIP was a frequent cause of parkinsonism in our Movement Disorder Unit, (2) calcium-channel blockers, and/or orthopramides and substituted benzamides were a frequent cause of DIP in our series, (3) old age and the female gender were frequent among DIP patients, (4) DIP is not always reversible.

Plasma levels of inorganic sulfates in patients with Parkinson's disease.

Some investigators reported pharmacogenetic differences in the metabolism of sulfur-containing drugs and lower plasma sulfate levels in patients with Parkinson's disease (PD) compared with controls. However, other group did not confirm these findings. We studied the plasma levels of sulfate by indirect atomic absorption spectrophotometry in 55 PD patients and 57 age and sex-matched controls. The plasma sulfate levels did not differ significantly between PD patients and control groups (mean +/- SEM 73 +/- 8 and 75 +/- 7 mg/l, respectively). They were not influenced by antiparkinsonian drugs and they did not correlate with age at onset, duration, Hoehn & Yahr staging or activities of daily living subscale of the Unified Parkinson's disease rating scale. There was a low but significant correlation of plasma sulfate levels with motor examination (r = -0.39, p < 0.05) and total score (r = -0.33, p < 0.05) of this scale in the PD group. These data suggest that plasma sulfate levels are apparently unrelated with the risk for PD.