Deficits in temporal processing correlate with clinical progression in Huntington's disease.

OBJECTIVES: Precise temporal performance is crucial for several complex tasks. Time estimation in the second-to-minutes range-known as interval timing-involves the interaction of the basal ganglia and the prefrontal cortex via dopaminergic-glutamatergic pathways. Patients with Huntington's disease (HD) present deficits in cognitive and motor functions that require fine control of temporal processing. The objective of the present work was to assess temporal cognition through a peak-interval time (PI) production task in patients with HD and its potential correlation with the Unified Huntington's Disease Rating Scale (UHDRS). MATERIALS AND METHODS: Patients with molecular diagnosis of HD and controls matched by age, sex and educational level (n=18/group) were tested for interval timing in short- (3 seconds), medium- (6 seconds) and long (12 seconds)-duration stimuli. RESULTS: Significant differences were observed in the PI task, with worse performance in HD compared to controls. Patients underestimated real time (left-shifted Peak location) for 6- and 12-second intervals (P<.05) and presented decreased temporal precision for all the intervals evaluated (P<.01). Importantly, a significant correlation was found between time performance and the UHDRS (P<.01). Patients' responses also deviated from the scalar property. CONCLUSIONS: Our results contribute to support that timing functions are impaired in HD in correlation with clinical deterioration. Recordings of cognitive performance related to timing could be a potential useful tool to measure the neurodegenerative progression of movement disorder-related pathologies.

Pseudomigraña con pleocitosis linfocitaria / Pseudomigraine with pleocytosis

El síndrome de cefalea y déficit neurológico transitorio con pleocitosis en el liquido cefalorraquídeoo pseudomigraña con síntomas neurológicos temporarios y pleocitosis linfocítica (HaNLD), es uncuadro de cefaleas recurrentes, déficit neurológico reversible y pleocitosis linfocitaria en el líquido cefalorraquídeo(LCR), de duración variable y resolución espontánea. Si bien se han propuesto múltiples mecanismosetiopatogénicos (vascular, infeccioso, inmunológico y alteración de los canales de calcio), su etiología es aúndesconocida. Describimos el caso de una mujer de 28 años de edad, con episodios recurrentes de migraña conpleocitosis, síndrome confusional y déficit neurológico transitorio, con remisión clínica dentro de los dos meses.Si bien la etiología sigue siendo desconocida. Presentamos los diagnósticos diferenciales a tener en cuenta ante este síndrome

The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis or pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (HaNDL) is a syndrome consisting of recurrent headaches, reversible neurological deficit, lymphocytic pleocytosis in cerebrospinal fluid (CSF), variable duration over time and spontaneous resolution. Although several etiopathogenic mechanisms have been suggested (vascular, infectous, immunological and calciumchannelopthy), its etiology remains unknown. We describe a 28 year old female, with recurrent migrainewith pleocytosis, confusional syndrome and transient neurological deficit. The clinical remission was achievedwithin two months. Although its etiology remains unknown the differential diagnosis is discussed in order to keepin mind this syndrome

Pseudomigraña con pleocitosis linfocitaria / Pseudomigraine with pleocytosis

El síndrome de cefalea y déficit neurológico transitorio con pleocitosis en el liquido cefalorraquídeoo pseudomigraña con síntomas neurológicos temporarios y pleocitosis linfocítica (HaNLD), es uncuadro de cefaleas recurrentes, déficit neurológico reversible y pleocitosis linfocitaria en el líquido cefalorraquídeo(LCR), de duración variable y resolución espontánea. Si bien se han propuesto múltiples mecanismosetiopatogénicos (vascular, infeccioso, inmunológico y alteración de los canales de calcio), su etiología es aúndesconocida. Describimos el caso de una mujer de 28 años de edad, con episodios recurrentes de migraña conpleocitosis, síndrome confusional y déficit neurológico transitorio, con remisión clínica dentro de los dos meses.Si bien la etiología sigue siendo desconocida. Presentamos los diagnósticos diferenciales a tener en cuenta ante este síndrome(AU)

The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis or pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (HaNDL) is a syndrome consisting of recurrent headaches, reversible neurological deficit, lymphocytic pleocytosis in cerebrospinal fluid (CSF), variable duration over time and spontaneous resolution. Although several etiopathogenic mechanisms have been suggested (vascular, infectous, immunological and calciumchannelopthy), its etiology remains unknown. We describe a 28 year old female, with recurrent migrainewith pleocytosis, confusional syndrome and transient neurological deficit. The clinical remission was achievedwithin two months. Although its etiology remains unknown the differential diagnosis is discussed in order to keepin mind this syndrome(AU)

Pseudomigraña con pleocitosis linfocitaria / Pseudomigraine with pleocytosis

El síndrome de cefalea y déficit neurológico transitorio con pleocitosis en el liquido cefalorraquídeoo pseudomigraña con síntomas neurológicos temporarios y pleocitosis linfocítica (HaNLD), es uncuadro de cefaleas recurrentes, déficit neurológico reversible y pleocitosis linfocitaria en el líquido cefalorraquídeo(LCR), de duración variable y resolución espontánea. Si bien se han propuesto múltiples mecanismosetiopatogénicos (vascular, infeccioso, inmunológico y alteración de los canales de calcio), su etiología es aúndesconocida. Describimos el caso de una mujer de 28 años de edad, con episodios recurrentes de migraña conpleocitosis, síndrome confusional y déficit neurológico transitorio, con remisión clínica dentro de los dos meses.Si bien la etiología sigue siendo desconocida. Presentamos los diagnósticos diferenciales a tener en cuenta ante este síndrome(AU)

The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis or pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (HaNDL) is a syndrome consisting of recurrent headaches, reversible neurological deficit, lymphocytic pleocytosis in cerebrospinal fluid (CSF), variable duration over time and spontaneous resolution. Although several etiopathogenic mechanisms have been suggested (vascular, infectous, immunological and calciumchannelopthy), its etiology remains unknown. We describe a 28 year old female, with recurrent migrainewith pleocytosis, confusional syndrome and transient neurological deficit. The clinical remission was achievedwithin two months. Although its etiology remains unknown the differential diagnosis is discussed in order to keepin mind this syndrome(AU)

[Acute reversible paraparesis secondary to probable fibrocartilaginous embolism]. / Paraparesia aguda reversible secundaria a probable embolia fibrocartilaginosa.

Fibrocartilagenous embolism (FCE) of the intervertebral disc represents a very rare cause of spinal infarct. Up to now only 33 others cases in human beings have been reported in the literature, most of them diagnosed post mortem. We present a 14-year-old boy who developed acute dorsal back pain after lifting a heavy gate, followed by progressive paraparesis. An MRI of the spine showed a degenerative disc at D10-D11 without compromise of the spinal canal lumen associated with an acute Schmorl's nodule situated in the superior endplate of D11. A week later, a second MRI disclosed an intraxial spinal cord lesion at D7-D8 vertebral level involving the vascular territory of the anterior spinal artery. It also showed an abnormal signal located in the posterior third of the D8 vertebral body. These clinical and neuro-radiological findings are similar to those mentioned in the literature and support the diagnosis of an anterior spinal infarct secondary to a probable fibrocartilaginous embolism. This case highlights the importance of considering this etiology among the causes of spinal cord infarct, especially in young people, and underlines the utility of MRI in its diagnosis in vivo.

Playing harp, another unusual task-specific dystonia.

Herein we report a task-specific dystonia in a 48-year-old woman, with an unusual association between a familial harp-playing dystonia and essential tremor.

Overexpression of neutrophil neuronal nitric oxide synthase in Parkinson's disease.

Much evidence supports a role of nitric oxide (.NO) and peroxynitrite (ONOO(-)) in experimental and idiopathic Parkinson's disease (PD); moreover, an overexpression of neuronal nitric oxide synthase (nNOS) was recently reported in the basal ganglia of PD patients. In accord, we previously found a 50% increased.NO production rate during the respiratory burst of circulating neutrophils (PMN) from PD patients. As PMN express the nNOS isoform, the objective of the present study was to ascertain whether this increased.NO production is representative of nNOS gene upregulation. PMN were isolated from blood samples obtained from seven PD patients and seven age- and sex-matched healthy donors; nNOS mRNA was amplified by reverse transcriptase-polymerase chain reaction and the products were hybridized with a probe for nNOS. Nitrotyrosine-containing proteins and nNOS were detected by Western blot and NO production rate was measured spectrophotometrically by the conversion of oxymyoglobin to metmyoglobin. The results showed that both.NO production and protein tyrosine nitration were significantly increased in PMN isolated from PD patients (PD 0.09 +/- 0.01 vs 0.06 +/- 0.008 nmol min(-1) 10(6) cells(-1); P < 0.05). In addition, five of the seven PD patients showed about 10-fold nNOS mRNA overexpression; while two of the seven PD patients showed an expression level similar to that of the controls; detection of nNOS protein was more evident in the former group. In summary, it is likely that overexpression of nNOS and formation of ONOO(-) in PMN cells from PD patients emphasizes a potential causal role of.NO in the physiopathology of the illness.

Circulating plasma factors in Parkinson's disease enhance nitric oxide release of normal human neutrophils.

Nitric oxide (*NO)-mediated toxicity has been involved in neurodegenerative diseases, including Parkinson's disease (PD). We have recently reported an increase of about 50% in *NO production rate in PMA-activated polymorphonuclear leukocytes (PMN) from either newly diagnosed or chronically treated PD patients. As humoral factors in sera from PD patients could inhibit cell dopaminergic activity, the aim of this study was to determine whether a plasma circulating factor from PD patients could modify *NO metabolism in PMN from healthy control subjects. To this purpose, we determined simultaneously the maximal production rate of *NO and hydrogen peroxide (H2O2) of PMA-activated PMN isolated from healthy control subjects in the presence of aliquots of plasma of PD patients. The results showed that, after 30 min incubation, plasma from newly diagnosed (n=4) or from L-Dopa chronically treated (n=7) PD patients enhanced *NO release in neutrophils isolated from healthy controls by about 50% and 47% respectively, with respect to non-parkinsonian control plasma (n = 10); in the same condition, H2O2 production did not differ among the groups. These data suggest that an overproduction of *NO related to plasma circulating factors, already detected at initial stages of the disease, participates in the pathophysiology of Parkinson's disease.

[Intracranial pressure monitoring and eclampsia]. / Monitorización de la presión intracraneal y eclampsia.

Intracranial pressure (ICP) monitoring has been shown to improve clinical-pharmacological treatment of intracranial hypertension (ICH) in a rising number of situations, assuring effective cerebral perfusion pressure (CPP) and, concomitantly, reducing the risk of brain ischemia. Although its use in entities such as eclampsia have been reported, the continuous use of ICP monitoring is restricted. We report the case of an eclampsic woman in whom ICP was monitored. Recordings allowed CPP to be correctly stabilized, with strict correlation between ICP and tomographic measurements of density. We also review the pathophysiologic mechanisms that have been proposed to cause ICH in eclampsia and emphasize the usefulness of ICP monitoring to manage this complication.

Neutrophil function, nitric oxide, and blood oxidative stress in Parkinson's disease.

We studied nitrogen radical nitric oxide (.NO) release and reactive oxygen species (ROS) production by isolated neutrophils after phorbol myristate acetate (PMA) stimulation in 12 newly diagnosed and nine treated Parkinson's disease (PD) patients and 10 age-matched healthy controls. Neutrophils of both groups of PD patients had an elevated PMA-activated release of .NO [61 and 57%, respectively, higher than that of controls (p < 0.05)]. In contrast, H2O2 release was only significantly increased by 56% in chronically treated patients. In agreement, the maximum rate of luminol-dependent chemiluminescence, which partly represents O2- H2O2- .NO interactions, was increased only in the treated group. When other blood markers of oxidative stress were compared, only erythrocyte catalase activity was decreased in both PD patient series by 33 and 39%, respectively (p < 0.05), whereas plasma antioxidant capacity and erythrocyte superoxide dismutase activity levels were decreased only in treated PD patients. This study suggests that neutrophils express a primary alteration of .NO release in PD patients, whereas H2O2 and oxidative-stress parameters are more probably related to the evolution of PD or to effects of treatment with L-dopa.