[The usefulness of timed motor tests in assessing Parkinson's disease]. / Utilidad de los test motores cronometrados en la evaluación de la enfermedad de Parkinson.

INTRODUCTION: At present, the evaluation of Parkinson's disease (PD) relies on clinical scales, mainly Unified Parkinson's Disease Rating Scale (UPDRS); however, other objective methods have been considered including timed tests. PATIENTS AND METHODS: We studied the motor performance of 53 patients with PD (34 male, 19 female; age 61.9 +/- 8.9 years; age at onset 51.9 +/- 11 years, clinical stage: 2.6 +/- 0.73). Motor evaluation comprised UPDRS and CAPIT timed tests including pronation-supination, finger dexterity, movement between two points or tapping, and walking test. Clinical evaluation was performed in baseline conditions (twelve hours off their medication) and in their best on state, after a standard dose of 200 mg of levodopa. RESULTS AND CONCLUSIONS: All CAPIT timed tests, especially tapping, maintained an excellent correlation with UPDRS in both off and on state. Tapping seems to be the best CAPIT timed test for objective motor evaluation of PD.

Utilidad de los test motores cronometrados en la evaluación de la enfermedad de Parkinson / The usefulness of time motor test in assessing parkingon´s disease

Introducción. En el momento actual, la evaluación de la enfermedad de Parkinson (EP) se basa en escalas clínicas, especialmente la Unified Parkinson’s Disease Rating Scale (UPDRS); sin embargo se han sugerido otros métodos de valoración que incluyen los test cronometrados. Pacientes y métodos. Hemos valorado la actividad motora de 53 pacientes con EP (34 hombres y 19 mujeres; edad media: 61,9 ± 8,9 años; edad inicial: 51,9 ± 11 años; estadio clínico: 2,6 ± 0,73). La evaluación consistió en la aplicación de la UPDRS, además de los test cronometrados del protocolo CAPIT, incluidos la pronosupinación, la habilidad digital, el movimiento entre dos puntos o tapping y el test de la marcha. La evaluación se desarrolló en cada caso en condiciones basales, a primera hora de la mañana, tras 12 horas sin medicación y, posteriormente, en situación on confirmada, tras una dosis estándar de 200 mg de levodopa. Resultados y conclusiones. Todos los test cronometrados CAPIT, especialmente el tapping mantuvieron una correlación significativa con la escala UPDRS, tanto en off como en on. El tapping, por su sencillez, parece ser el mejor test cronometrado CAPIT para la valoración objetiva de la EP (AU)

Introduction. At present, the evaluation of Parkinson’s disease (PD) relies on clinical scales, mainly Unified Parkinson’s Disease Rating Scale (UPDRS); however, other objective methods have been considered including timed tests. Patients and methods. We studied the motor performance of 53 patients with PD (34 male, 19 female; age 61.9 ± 8.9 years; age at onset 51.9 ± 11 years, clinical stage: 2.6 ± 0.73). Motor evaluation comprised UPDRS and CAPIT timed tests including pronation-supination, finger dexterity, movement between two points or tapping, and walking test. Clinical evaluation was performed in baseline conditions (twelve hours off their medication) and in their best on state, after a standard dose of 200 mg of levodopa. Results and conclusions. All CAPIT timed tests, especially tapping, maintained an excellent correlation with UPDRS in both off and on state. Tapping seems to be the best CAPIT timed test for objective motor evaluation of PD (AU)

[Freezing of gait or freezing of quadruped gait]. / Bloqueo freezing del gateo o bloqueo de la marcha cuadrúpeda.

Parkinson's disease (PD) is frequently associated with freezing of gait. Patients with advanced PD learn to use tricks to relieve freezing of gait. Recently one of our patients informed us that he could overcome freezing of gait by crawling. Since crawling is a sort of quadruped gait that humans develop transiently, we wondered whether quadruped gait is preserved in PD. We recruited 16 patients with PD who had frequent and disabling freezing of gait. Under experimental conditions, after 12 hours without medication, seven patients developed biped freezing of gait. Of these seven patients, only two patients (the patients with the most severe freezing) also developed quadruped freezing of gait. This experiment suggests that bipedal gait is affected early in PD, while quadruped gait is preserved until late in this disease.

Progressive response to botulinum A toxin in cerebral palsy.

Botulinum A toxin (BT) has been successfully used for the management of spasticity in cerebral palsy (CP). However, the long-term results of BT have not yet been determined. We have studied the evolution of a homogeneous group of patients with CP treated with BT. All these patients had an equinus gait resulting from calf muscle spasticity without other muscle group involvement. All of these patients were treated with the same total dose (4 microg/kg) at the same time interval (three months). The mean follow-up time was 33 months. Gait evaluation was made blind on videotape recordings by two independent physicians according to five point scale. All our patients exhibited a progressive improvement in their gait pattern. None of our patients developed fixed contractures nor did any of them need surgical correction. No significant side-effects were seen. The response observed in our study could be due to a progressive symptomatic effect of BT, but it might be also explained by a change in the natural history of the spasticity related to CP, at least in this selected group of patients.

Botulinum toxin treatment for spasmodic dysphonia: percutaneous versus transoral approach.

Spasmodic dysphonia (SD) is at present defined as focal dystonia. Botulinum toxin (BT) injection is the treatment of choice for SD. BT is usually injected by a percutaneous route, but a direct, visually guided transoral approach has also been successful. It is not known whether percutaneous injection is as effective as the transoral approach. This article reviews our experience with both techniques of injection on 29 patients with adductor type SD. Since 1992, we have carried out 48 treatment sessions with the transoral technique and 76 treatment sessions with the percutaneous technique. Two patients did not respond to the percutaneous technique despite several attempts, but they did respond to the transoral approach. Globally, transoral technique was superior to percutaneous technique in terms of effectiveness (48 of 48 responses with transoral technique versus 61 of 76 responses with percutaneous approach, p < 0.01). Dosage of BT, duration, and side effects were similar with both techniques. This article also describes a simple, inexpensive device, composed of materials on hand at every hospital, that facilitates the transoral approach.

[Adduction spastic dysphonia: clinical and treatment]. / Disfonía espástica de aducción: clínica y tratamiento.

Adduction spastic dystonia (SD) is currently considered a focal dystonia involving laryngeal muscles. SD is one of the most poorly known focal dystonias. We reviewed our experience with twentynine patients with adduction SD and compared the clinical and epidemiologic variables with the other focal dystonias studied at our institution in the last five years (132 patients). Mean age of patients (47.2 +/- 13 years), sex, clinical course in years (5.7 +/- 5) and presence of circadian fluctuations did not differ significantly from those observed in patients with other focal dystonias. Likewise, there were no significant differences regarding the presence of a family history of dystonia, essential tremor, or stuttering. Our results confirm the similarity of the clinical and epidemiologic data of SD with the other focal dystonias. All patients with SD were treated with a local injection of botulinum toxin. A total of 108 treatments were performed, 41 with a visually guided transoral technique and 67 with a percutaneous technique. The transoral technique was effective in all cases (41/41) but not all treatments with the percutaneous technique were effective (53/67). Three patients required the shift to the transoral procedure to achieve enough symptomatic alleviation.

[Laryngeal dystonia. Comparison of transcutaneous and transoral injection of botulinum toxin]. / Distonía laríngea. Tratamiento con toxina botulínica mediante técnica transcutánea y transoral. Estudio comparativo.

Spasmodic dysphonia is a focal dystonia that effects the proximal muscles (adduction dystonia) or dilatory (abduction dystonia) of the larynx. Botulinum toxin (BTX), generally delivered by percutaneous injection, is the treatment of choice. Recently, use has been made of a transoral route of delivery, with BTX injected through a curved device with visual control. It remains to be determined which route is better. Percutaneous injection is simple and well-tolerated, but the transoral route is theoretically more effective. We assessed the efficacy of both techniques in 19 patients with adduction dystonia, conducting 55 treatment sessions by percutaneous injection and 20 by transoral injection. All the transoral treatments were effective (20/20, 100%), but only 81% (45-155) of the percutaneous treatments were (p < 0.05). Two patients who had doubtful responses after percutaneous delivery improved considerably when the transoral approach was used. In spite of its greater complexity, the transoral approach is probably more effective than the percutaneous route. We describe a curved device for transoral injection that is composed of simple elements available at any health center.

[Botulinum A toxins in the treatment of spasticity in cerebral palsy during childhood]. / Tratamiento de la espasticidad asociada a parálisis infantil con toxina botulínica.

We evaluated the safety and efficacy of Botulinum- A toxin (BTX) in patients with equinus deformity associated with cerebral palsy (CP). We prescribed BTX to six patients (all of whom had previously participated in a multicenter, randomized, double blind study of the same drug); treatment continued for at least 15 months. Four children showed striking improvement, being converted from toe-toe to consistent or occasional heel-toe gait. No side effects were observed. BTX appears to be safe and effective in patients with CP.

Cerebrospinal fluid homovanillic acid is reduced in untreated Huntington's disease.

We measured homovanillic acid (HVA), 5-hydroxy indole acetic acid (5-HIAA), and tryptophan (TP) in cerebrospinal fluid (CSF) of 20 neuroleptic-free patients with Huntington's disease (HD), and compared mean values with those from four control groups including 15 normal individuals, 38 patients with dystonia, 23 untreated patients with Parkinson's disease, and 61 patients with other neurological diseases (ONDs). The mean levels of HVA in the CSF of patients with HD were reduced compared with those from normal controls (p < 0.001), dystonic patients (p < 0.005), individuals with ONDs (p < 0.0001), and even from untreated parkinsonian patients (p < 0.05). 5-HIAA and TP levels in the CSF of patients with HD were not significantly different from those in the CSF of control patients. Our data suggest a reduced dopamine neurotransmission in HD and may account for the bradykinesia observed in our patients.