Síndrome de encefalopatía progresiva de comienzo temprano / Early-onset progressive encephalopathy syndrome

El síndrome PEHO (encefalopatía progresiva, edema, hipsarritmia y atrofia óptica), descrito en 1991, es un trastorno infrecuente con inicio en los primeros meses, que cursa con hipotonía, edemas, crisis epilépticas, retraso psicomotor y evolución tórpida con fallecimiento en edades tempranas. En 1993, se establecen los criterios clínicos para el síndrome PEHO, y se identifica el síndrome similar al PEHO, sobre la base de hallazgos neurorradiológicos, al compartir la semiología clínica y, con matices, los hallazgos neurofisiológicos. Los primeros y gran parte de los casos descritos son finlandeses. A partir de 1995, han aparecido pacientes en otros países. Ambos síndromes tienen la misma gravedad en ambos sexos. Se estima una herencia autosómica recesiva. No se ha encontrado alteración analítica que justifique los hallazgos clínicos ni radiológicos. Se sugiere que el incremento de los niveles de óxido nítrico en ambos síndromes y la disminución de los niveles del factor de crecimiento similar a la insulina en el síndrome PEHO en el líquido cefalorraquídeo están comprometidos en su patología. El síndrome PEHO y el síndrome similar al PEHO difieren en los hallazgos neurorradiológicos, infratentoriales en el primero y supratentoriales en el segundo. Al cursar ambos con encefalopatía, neuropatía, disfunción autonómica y epilepsia progresiva se sugiere que constituyen variantes de un mismo continuum neurobiológico...

Frequency characteristics of the semicircular canals lesion in Hunt syndrome with vertigo / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To research the frequency characteristics of the semicircular canals lesion in Hunt syndrome with vertigo and the clinical value of the video head impulse test (vHIT) for vestibular function evaluated in this disease.</p><p><b>METHODS</b>Thirty normal persons (control group) accepted the vHIT, 26 patients with Hunt syndrome with vertigo (study group) accepted low, mid and high frequency vestibular function tests, including caloric test (CT), head shaking test (HST) and vHIT. The parameters of the unilateral weakness (UW), head shaking nystagmus (HSN) and video head impulse test gain (vHIT-G) were observed. The correlations and characteristics of the results of the three tests in Hunt syndrome with vertigo deal were analyzed with SPSS 16.0 software.</p><p><b>RESULTS</b>The values of vHIT-G of the six groups semicircular canal in the control group were normal distribution without statistical significance (F = 0.005, P < 0.01), two sides anterior, horizontal and posterior semicircular canals vHIT-G average (x ± s) were (16.80 ± 9.80)%, (16.57 ± 10.30)%, (16.52 ± 11.12)% respectively; in the study group the separately vHIT-G of the three canals of the affected side were (46.96 ± 34.54)%, (75.35 ± 35.29)% and (41.65 ± 32.87)%, in which statistical significance comparing with the control group was detected (all P < 0.01); the positive one of the three tests vHIT, HSN and CT were 23 cases (88.46%), 22 cases (84.61%) and 24 cases (92.31%), bilateral exact probability χ² test all the P > 0.05;there were Positive correlation between UW and the vHIT-G of lateral semicircular canal (r = 0.692, P < 0.01).</p><p><b>CONCLUSIONS</b>The vestibular lesion of Hunt syndrome with vertigo is almost complete or multiple-frequency, which is characterized by the reduced or even aborted nerve conduction. Therefore, vHIT can be ultimately used for the screening test evaluating due to the vestibular function in Hunt syndrome with vertigo.</p>

Effectiveness of neuromuscular rehabilitation in multiple sclerosis patients on balance and walking ability

Multiple sclerosis [MS] is a chronic, progressive neurological disease with a clinical course that may result in severe disability. It is characterized by movement, balance and walking impairment. In the past, physical therapy management of MS focused on passive treatment to avoid exacerbating fatigue or the disease process. Current research is demonstrating overwhelming benefits when MS patients perform exercise. The aim of this study was to investigate the effectiveness of neuromuscular rehabilitation program on MS patients with cerebello-pyramidal manifestations. Twenty five patients diagnosed as clinically definite MS were recruited from the out patient Department of Neurology, Kasr El-Aini hospital, Cairo University. They were 14 females and 11 males; their mean age was 41.6 +/- 7.9; mean duration of illness was 4.5 +/- 2.7 years and the mean Expanded Disability Status Scale [EDSS] was 4.9 +/- .912. Assessment was done in the morning before beginning the treatment program and after seven weeks of treatment. Six Spot Step Test is a quantitative assessment of lower extremity function, which is measured in the time domain by digital stop watch. Biodex stability system is an objective assessment of balance. The overall stability index was used as a measure of postural stability. Patients were assessed with bilateral stance at level 8 [most stable] over a period of 20 seconds. The neuromuscular rehabilitation program consisted of: rhythmic stabilization of proprioceptive neuromuscular facilitation [PNF], slow reversal of PNF, Frenkel's coordination exercises in supine, sitting and standing positions, static and dynamic balance training in crawling, kneeling and half kneeling positions, static and dynamic balance training by means of external postural perturbation, weight shift to both extremities and to the posterior part of feet while standing with feet together, in tandem stance, single limb support and Cawthorne-Cooksey exercises. Patients received the program three times per week for seven weeks. The results of this study showed the effectiveness of neuromuscular rehabilitation program in improving both the walking ability [p=.001] and balance [p=.001] in MS patients. Moreover weak non significant correlation was found between the tested variables and EDSS

Ramsay hunt syndrome complicated by contralateral cerebral infarction

Varicella-zoster virus has been associated with a variety of neurological manifestations. We describe a patient with the Ramsay Hunt Syndrome who developed a contralateral cerebral infarction.

Pattern-visual evoked potentials in thinner abusers

Organic solvents cause injury to lipids of neuronal and glial membranes. A well known characteristic of workers exposed to thinner is optic neuropathy. We decided to look for neurophysiologic signs of visual damage in patients identified as thinner abusers. Pattern reversal visual evoked potentials was performed on 34 thinner abuser patients and 30 controls. P-100 wave latency was found to be longer on abuser than control subjects. Results show the possibility of central alterations on thinner abusers despite absence of clinical symptoms

Dissinergia cerebelar mioclônica de Ramsay-Hunt. Estudo clínico, eletroencefalográfico e por tomografia axial computadorizada - Diagnóstico diferencial / Ramsay-Hunt cerebellar myoclonic dyssynergia

Os autores descrevem um caso de dissinergia cerebelar mioclônica de Ramsay-Hunt, associada a epilepsia em uma menina de 14 anos. O diagnóstico foi baseado em um quadro cerebelar associado a mioclonias, epilepsia grande mal e história familiar. O ECG mostrou uma atividade de fundo normal, associado a ondas agudas, polipontas e polipontas ondas, que näo sofrem alteraçäo pela alcalose cerebral, mas que é ativado pela fotoestimulaçäo intermitente. Os autores concluem que o diagnóstico pode ser dado pelo quadro clínico, traçado eletroencefalográfico característico, e a inclui entre as epilepsias mioclônicas progressivas e degenertivas

Herpes-zoster ótico: apresentaçäo de dois casos / Herpes zoster oticus: report of 2 cases

O quadro de Herpes-zoster ótico apresenta, obrigatoriamente, o envolvimento do VII par craniano. O acometimento do VII par, assim como o de outros pares, têm incidência variável segundo a maioria dos autores. O tratamento clínico é, a priori, o indicado, sendo a evoluçäo satisfatória na maioria dos casos, principalmente em pacientes jovens

Discinergia cerebelar mioclônica com epilepsia: síndrome de Ramsay-Hunt / Myoclonic cerebellar dysynergia with epilepsy: Ramsay-Hunt syndrome

Descrevem-se dois casos de Discinergia cerebelar mioclônica com Epilepsia (Síndrome de Ramsay-Hunt), fazendo-se uma análise de sua sintomatologia clínica e eletroencefalográfica