[Neuropsychological study of 12 patients with pure degenerative cerebellar disease]. / Evaluación cognitiva en 12 pacientes con enfermedad cerebelosa degenerativa pura.

INTRODUCTION: The cerebellum has been traditionally associated with motor control learning and performance. However, since 1970 a growing body of clinical and experimental evidences has suggested that the cerebellum may be involved in nonmotor cognitive functions as well. OBJECTIVE: To explore the presence of eventual cognitive impairment in non-demented patients with isolated degenerative cerebellar diseases. PATIENTS AND METHODS: Twelve patients with the diagnosis of selective degenerative cerebellar disorders, either inherited or sporadic, were selected (mean age: 40.42 +/- 13.49 years; mean education level: 9.92 +/- 3.99 years; duration of illness: 12.13 +/- 11.27 years, MMSE: 26.75 +/- 1.5) and evaluated through a standardized neuropsychological tests battery. Normalized Z scores were estimated and compared against 0, employing the t test for one sample. RESULTS: Significant cognitive deficits were found in the following domains: executive, visuo-spatial, memory and attention functions. Performance on the Wisconsin test showed a significative number of perseverative errors. Memory deficits included verbal learning and free recall difficulties, with good recognition of the material presented. CONCLUSIONS: The findings of this study are consistent with the role of the cerebellum as modulator of mental functions. The cognitive deficits resulting from cerebellar pathology may be related with the disruption of cerebello-cortical connexions involving a complex network which includes the prefrontal region, suggesting that the cerebellum may process cortical information coming from different areas linked with the control of cognition.

Catalepsy after stroke.

Catalepsy denotes a tendency to maintain postures. We prospectively looked for catalepsy in 216 acute stroke patients. We found five patients with cataleptic postures. They were seen on the nonparetic side in all cases. Other movement abnormalities seen in the catatonic syndrome were absent. CT scans of the head showed ischemic infarctions in the middle cerebral artery territory in the five cases. Cataleptic postures associated with hemispheric lesions due to stroke may be a more common phenomenon than is believed, and their mechanism remains to be elucidated.

Spontaneous and reflex movements in brain death.

Spontaneous and reflex movements may be found in patients with brain death (BD). The authors prospectively evaluated their frequency using a standardized protocol. Among 38 patients who fulfilled criteria for BD, the authors found 15 (39%) with spontaneous or reflex movements. The most common movement was finger jerks. Undulating toe flexion sign, triple flexion response, Lazarus sign, pronation-extension reflex, and facial myokymia also were seen. These movements may be more common than reported and do not preclude the diagnosis of BD.

In vivo 31P NMR profiles of Alzheimer's disease and multiple subcortical infarct dementia.

We used in vivo phosphorus 31 nuclear magnetic resonance (31P NMR) spectroscopy to study regional high-energy phosphate and phospholipid metabolism in brains of patients with dementia associated with probable Alzheimer's disease (AD) and multiple subcortical cerebral infarctions (MSID). The MSID patients demonstrated elevations of the phosphocreatine (PCr)/inorganic orthophosphate (Pi) ratio in both the temporoparietal and frontal regions. Phosphomonoesters (PME) and the ratio of PME to phosphodiesters were elevated in the temporoparietal region of AD. Pi was also elevated in the frontal and temporoparietal regions of AD. Findings from 31P NMR were accurate in distinguishing MSID from AD. Values of PCr/Pi accurately classified 100% of the MSID patients and 92% of AD. Pi and PME, considered jointly, also accurately classified all MSID and all but 1 AD. Findings from in vivo 31P NMR spectroscopy appear to yield metabolic profiles useful in distinguishing AD from MSID.

Reliability of the NINDS Myotatic Reflex Scale.

The assessment of deep tendon reflexes is useful for localization and diagnosis of neurologic disorders, but only a few studies have evaluated their reliability. We assessed the reliability of four neurologists, instructed in two different countries, in using the National Institute of Neurological Disorders and Stroke (NINDS) Myotatic Reflex Scale. To evaluate the role of training in using the scale, the neurologists randomly and blindly evaluated a total of 80 patients, 40 before and 40 after a training session. Inter- and intraobserver reliability were measured with kappa statistics. Our results showed substantial to near-perfect intraobserver reliability, and moderate-to-substantial interobserver reliability of the NINDS Myotatic Reflex Scale. The reproducibility was better for reflexes in the lower than in the upper extremities. Neither educational background nor the training session influenced the reliability of our results. The NINDS Myotatic Reflex Scale has sufficient reliability to be adopted as a universal scale.

The effect of levodopa on the habituation of the acoustic-palpebral reflex in Parkinson's disease.

The aim of the present study was to assess the habituation of the blink responses evoked by repetitive auditory stimuli in patients with Parkinson's disease in different clinical states. We studied 28 parkinsonian patients. Eighteen patients without motor fluctuations were studied off and on levodopa, on two different sessions, one week apart. The remaining 10 parkinsonians had the wearing off phenomenon and were studied while being off their medication and while ON and OFF, as determined clinically, after a single dose of levodopa. Twelve age-matched controls were also studied in a single session. Unilateral auditory 1 kHz, 105 dB stimuli were delivered at a frequency of 0.2 Hz. The number of responses (R) from the orbicularis oculi was registered. R values were significantly smaller in controls and in treated parkinsonians than in untreated patients. R was also significantly smaller when ON than at baseline and when OFF in parkinsonians with the wearing off phenomenon. These results suggest a failure of the inhibition of the acoustic-palpebral reflex in Parkinson's disease. The improvement with levodopa and the similarities between the clinical and the electrophysiological changes suggest a dopaminergic role in the inhibition of this brainstem reflex.

[Hemichorea associated with cerebral toxoplasmosis and AIDS]. / Hemicorea asociada a toxoplasmosis cerebral y SIDA.

Only 12 AIDS cases with hemichorea were reported in the literature. We report the first case of hemichorea associated with AIDS and cerebral toxoplasmosis in our country. A 26-year-old man had 3 episodes of focal seizures on the left side with subsequent loss of consciousness. A few weeks later, he noticed progressive left-sided weakness. Examination revealed a left hemiparesis. MRI of the head showed a round mass in the right frontal lobe and a smaller lesion in the left temporo-occipital area. Laboratory showed positive serum ELISA and Western Blot analysis for HIV antibodies. Serum tests for Toxoplasma showed elevated titers. He was treated with pyrimethamine and sulfadiazine. His weakness improved and he had no further seizures. Two weeks later, choreic movements appeared in the left foot, finally involving the entire left hemibody. A second MRI showed a new small lesion in the right cerebral peduncle. The patient completed 6 weeks of treatment, with further reduction in the size of the lesions. Nevertheless, the left hemichorea persisted. We believe that the hemichorea our patient had was caused by the contralateral peduncular lesion. Lesions involving the subthalamic nucleus or its connections may cause contralateral hemiballismus or hemichorea. In spite of the favorable response to antitoxoplasmic therapy, the hemichorea persisted. The present report illustrates an uncommon neurological complication in AIDS. We believe that a combination of a focal cerebral lesion and the HIV infection caused the movement disorder presented by the patient.

Peripheral nerve involvement in Bell's palsy.

A group of patients with Bell's palsy were studied in order to disclose the presence of subclinical peripheral nerve involvement. 20 patients, 8 male and 12 female, with recent Bell's palsy as their unique disease were examined. In all cases other causes of polyneuropathy were ruled out. Patients were investigated with CSF examination, facial nerve latencies in the affected and in the sound sides, and maximal motor nerve conduction velocities, as well as motor terminal latencies from the right median and peroneal nerves. CSF laboratory examination was normal in all cases. Facial nerve latencies were abnormal in all patients in the affected side, and they differed significantly from those of control group in the clinically sound side. Half of the patients showed abnormal values in the maximal motor nerve conduction velocities and motor terminal latencies of the right median and peroneal nerves. These results agree with previous reports which have pointed out that other cranial nerves may be affected in Bell's palsy. However, we have found a higher frequency of peripheral nerve involvement in this entity. These findings, support the hypothesis that in some patients Bell's palsy is the component of a more widespread disease, affecting other cranial and peripheral nerves.

Craniocerebral involvement in lymphoma.

Nine-hundred-eighty-nine patients with lymphoma were studied. Fifty-three cases (5.3%) had lymphomatous craniocerebral infiltration. The principal factors of risk for this complication were: advanced stage of the lymphoma (III or IV), diffuse histiocytic, diffuse poorly differentiated lymphocytic, or mixed cellularity lymphoma histological type, bone marrow involvement, and previous systemic chemotherapy. Thirty-two per cent of the cases of meningeal lymphomatous infiltration were asymptomatic and represented autopsy findings. CT-scan was an useful test to detect brain focal parenchymatous infiltration, as opposed to meningeal infiltration. Mean survival time in patients with lymphomatous meningeal infiltration was 4.3 months, following the combined use of systemic chemotherapy, radiation therapy and intrathecal methotrexate. Two cases had primary cerebral lymphoma, although without associated immunodeficiency. Twenty patients (2%) had intracranial hemorrhage, in clear relationship with platelet alterations. Fifteen patients (1.5%) had CNS infection, caused by common bacteria or opportunistic agents. In 7 cases, the diagnosis was made at autopsy. Thirty-six autopsies were performed. In 8 cases (22%), pathologic findings such as, demyelination, microcalcifications, coagulative necrosis, or gliosis, suggested complications from treatment.

Motor activation time in Parkinson's disease.

Motor activation time (MAT), considered in the present work as the actual intracerebral processing delay during a reaction time (RT) task, was assessed in 17 patients with Parkinson's disease (PD) and in 7 age-matched healthy volunteers. MAT was calculated by subtracting from the premotor RT the afferent and efferent times obtained by sensory and motor evoked potentials. When compared with healthy volunteers, patients exhibited significantly (p < 0.02) prolonged MATs. In a smaller group of 9 PD patients MAT and the auditory P3 event related potential were assessed while on antiparkinsonian treatment and after a 12-hour withdrawal period. During the off medication condition patients showed a significant slowing (p < 0.01) of MAT values without any remarkable change in P3 latency or amplitude. These results suggest that MAT slowing indicates an abnormal function of the dopaminergic mechanism involved in the initiation of movement which is not related to changes in the arousal or cognitive state.

Undulating toe movements in brain death.

For many years, death implied immobility. Nevertheless, there are anecdotal reports of spontaneous or reflex movements (SRMs) in patients with Brain death (BD). The presence of some movements can preclude the diagnosis of BD, and consequently, the possibility of organ donation for transplantation. McNair and Meador [(1992), Mov Dord7: 345-347] described the presence of undulating toe flexion movements (UTF) in BD patients. UTF consists in a sequential brief plantar flexion of the toes. Our aim was to determine the frequency, characteristics and predisposing factors of UTF movements in a prospective multicenter cohort study of patients with BD. Patients with confirmed diagnosis of BD were assessed to evaluate the presence of UTF using a standardized protocol. All patients had a routine laboratory evaluation, CT scan of the head, and EEG. Demographic, clinical, hemodynamic and blood gas concentration factors were analyzed. amongst 107 BD patients who fulfilled the AAN requirements, 47 patients (44%) had abnormal movements. UTF was observed in 25 (23%) being the most common movement (53%). Early evaluation (OR 4.3, CI95% 1.5-11.9) was a predictor of UTF in a multivariate regression model. The somato-sensory evoked potential (SSEPs) as well as brainstem auditory evoked potentials (BAEPs) did not elicit a cortical response in studied patients with UTF. This spinal reflex is probably integrated in the L5 and S1 segments of the spinal cord. Abnormal movements are common in BD, being present in more than 40% of individuals. UTF was the most common spinal reflex. In our sample, early evaluation was a predictor of UTF. Health care professionals, especially those involved in organ procurement for transplantation, must be aware of this sign. The presence of this motor phenomenon does not preclude the diagnosis of BD.

Peripheral nervous system and spinal cord involvement in lymphoma.

Nine-hundred-eighty-nine patients with diagnosis of lymphoma were studied. Forty-six cases (4.6%) had compressions of the spinal cord or roots. Forty-two patients (4.2%) had Herpes zoster virus infections, which in 6 cases were of disseminated type. The major predisposing factors for infection were: advanced stage of lymphoma, previous systemic chemotherapy and splenectomy. Toxic polyneuropathy secondary to chemotherapy was found in 39 patients (3.9%). In 14 cases, the polyneuropathic symptoms were the main complaint (Group 1), while in the remaining 25 cases the diagnosis was made during neurological consultations because of unrelated symptoms (Group 2). Both groups did not have significant differences in the total dose of chemotherapy received. The electrophysiological studies showed an axonal neuropathy in both groups. The discontinuation of chemotherapy was found to be a limiting factor in the appearance of neuropathic symptoms. Other less frequent forms of involvement were: compression of peripheral nerves or nerve plexi from lymphadenopathies (3 cases), radiation myelopathy (1 case), and Guillain-Barré Syndrome associated with Hodgkin's Lymphoma (1 case).

Hemicorea asociada a toxoplasmosis cerebral y SIDA / Hemichorea associated with cerebral toxoplasmosis and AIDS

Se observan complicaciones neurologicas en 40 por ciento de enfermos con SIDA. De estos, en 10 por ciento puede ser la manifestación inicial de la enfermedad. En otro 11 por ciento pueden aparecer trastornos del movimimiento. Comunicamos el primer caso de hemicorea asociada a toxoplasmose cerebral y SIDA en nuestro pais. Hombre de 26 anos, con diagnóstico de SIDA y toxoplasmosis cerebral. Habia comenzado con crisis motoras simples de hemicuerpo izquierdo, con generalización secundaria y luego perdida de fuerza progresiva en dicho hemicuerpo. La RMN de cerebro mostro una lesión frontal derecha y otra temporo-occiptal izquierda, con gran edema perilesional y efecto de masa. Las serologias para HIV y toxoplasmosis fueron positivas. Comenzo tratamiento con sulfadiazina y pirimetamina. Al duodecimo dia aparecieron movimientos involuntários del pie izquierdo, coreicos, que se extendieron mas tarde a todo ese miembro inferior y luego al hemicuerpo. Nueva RMN de cerebro mostro disminución del edema y efecto de masa de las lesiones. Sin embargo, se observó una nueva lesion a nivel peduncular derecho. Movimientos involuntarios en pacientes con toxoplasmosis cerebral la literatura mundial y el primero en nuestro pais de hemicorea asoicada a toxplasmosis y SIDA

Correlación entre el diagnóstico clínico y la tomografía computada cerebral / Correlation between clinical diagnosis and cerebral computed tomography

Presentamos un estudio realizado sobre 208 pacientes del Servicio de Neurología del Hospital Ramos Mejía, en quienes efectuamos una tomografía computada de cerebro como examen complementario. En cada uno de los casos correlacionamos la impresión diagnóstica clínica al ingreso del paciente, tanto desde el punto de vista etiológico como topográfico, con los resultados de las tomografías efectuadas en esos pacientes. Observamos que los cuadros de infartos isquémicos fueron sobrediagnosticados clínicamente a expensas de los accidentes cerebrovasculares hemorrágicos y de los tumores intracerebrales. Los hematomas intracerebrales espontáneos tuvieron una mayor coincidencia clínico-tomográfica tanto etiológica como topográficamente. En la patología tumoral hubo una menor coincidencia entre la clínica y los resultados de las TC. Observamos también que la TC no siempre coincide, en lo que hace a la presunción histopatológica para el caso de los tumores, con los resultados del examen anatomopatológico. Además destacamos que a través de la TC fue posible realizar el diagnóstico de egreso en el 71% de nuestros pacientes, evitando la realización de otros métodos más cruentos

Estado clínico al despertar en la enfermedad de Parkinson y su relación con el sueño / Motor function in the morning in Parkinson's disease and its relationship with sleep

Se estudiaron 36 pacientes con enfermedad de Parkinson (EP), con el fin de evaluar el probable efecto beneficioso del sueño sobre el estado motor al despertar, referido por algunos enfermos. Todos los pacientes respondieron a un cuestionario con información acerca de características de la enfermedad, presencia de fluctuaciones, medicación y trastornos del sueño. También se evaluó a los pacientes con las escalas URSPD, H&Y y de depresión geriátrica. Trece pacientes (36,1 por ciento) se sintieron mejor por la mañana, 13 peor y 10 (28 por ciento) igual. Los pacientes que se hallaban igual por la mañana tenía estadios más temprano de la enfermedad. Las fluctuaciones motoras fueron más frecuentes en el grupo que estaba peor por la mañana, los cuales tenían además menor edad. Los pacientes que estaban mejor por la mañana tendieron a tener más horas de sueño. Los trastornos del sueño en la EP no fueron distintos a los de una población control de la misma edad. Creemos que el estado motor matinal en la EP es una manifestación de las fluctuaciones motoras, aunque el número de horas de sueño también tiene una influencia positiva

Peripheral nerve involvement in Bell's palsy.

Um grupo de pacientes com paralisia de Bell foi estudado com o objetivo de descobrir a presenca de comprometimento nervoso periferico subclinico. 20 pacientes, 8 homens e 12 mulheres com paralisia de Bell recente como unica enfermidade, foram examinados. Em todos os casos outras causas de polineuropatia foram excluidas. Os pacientes foram estudados com exames de liquido cefalorraqueano; latencia distal nos nervos faciais do lado afetado e do lado sadio e velocidade de conducao motora maxima; latencia distal dos nervos mediano e peroneiro direitos.O exame de LCR foi normal em todos os casos. A latencia do nervo facial foi anormal em todos os pacientes no lado afetado e, no lado clinicamente sadio, diferia significativamente do grupo controle. A metade dos pacientes mostrou valores anormais na velocidade motora maxima e/ou na lactancia distal de ambos os nervos, como sejam: mediano e peroneiro.Esses resultados estao de acordo com publicacoes anteriores nas quais se afirma que outros nervos craneanos podem estar afetados na paralisia de Bell. Temos encontrado uma elevada frequencia de polineuropatia nesta enfermidade. Estes achados nos levam a crer que, em alguns enfermos a paralisia de Bell e um componente de uma doenca mais disseminada afetando outros nervos craneanos e perifericos

Complicaciones craneoencefalicas de los linfomas / Craniocerebral complications of lymphomas

Fueron estudiados 41 pacientes portadores de linfomas, que desarrollaron, en algún momento de su enfermedad complicaciones del Sistema Nervioso Central (SNC). La incidencia de este grupo con respecto a la población linfomatosa en general fue del 6,6%. Consideramos en nuestra serie un 57% de linfomas No Hodgkin (LNH) y un 43% de linfomas Hodgkin (LH), predominando ambos grupos en el sexo masculino. Fueron realizadas un total de 27 necropsias. El mecanismo lesional más frecuentemente observado fue la infiltración (68%), ya sea meníngea, parenquimatosa u ósea. Los tipos histológicos que predominaron fueron el difuso (en el caso de los LNH) y el de celularidad mixta (tratándose de los LH). La incidencia de infecciones fue mayoritaria en el grupo de los LH. La mayor frecuencia de complicaciones en el SNC se observó en estadios avanzados de la enfermedad (IV A y B), mostrando una tendencia decreciente en las formas más benignas. Los tiempos de latencia entre el diagnóstico del linfoma y la aparición de la complicación neurológica, fueron de 60 meses para los LH, en tanto que disminuyó a 21 meses en el grupo de los LNH. Los signos y síntomas más frecuentemente ballados fueron el compromiso de los pares craneales (especialmente oculomotores y el facial) y las alteraciones del estado de conciencia, sumadas a variables déficits focales. El compromiso de la Médula Osea se relacionó en forma directa con los casos en que se confirmó compromiso del SNC por mecanismo infiltrativo. Los métodos complementarios de diagnóstico por imágenes fueron eficaces en un 68%, aportando escasa utilidad en la identificación de las leptomeningitis linfomatosas (LML). El pronóstico más severo se verificó en el grupo de las LML (3,8 meses de sobrevida)...