Infantile tremor syndrome: Case report

Infantile tremor syndrome (ITS) is a very rare disease with an incidence of <0.2% in India. Affecting children are around 1 year of age and characterized by developmental delay, skin pigmentation, and coarse tremors. Studies since 1990 conclude that ITS is caused by nutritional deficiencies, most prominently of Vitamin B12. Since lactating mothers from a poor socioeconomic background in regions where vegetarianism is prevalent because of cultural reasons are most likely to be deficient in Vitamin B12, the syndrome is most likely to affect this segment. The case described herein is unique because it is from Mangalore in India which is a coastal town with very low levels of vegetarianism. A 14-month-old girl presented with fever, vomiting, and loose stools coupled with abnormal movements of eyes, tongue, and hands which were coarse, rhythmic, continuous of low amplitude, present throughout the day, and absent during sleep. Although initial suspicions were of seizures and the patient was administered anti-epileptics, there was no improvement in the condition of the patient. On suspicions of symptoms being of ITS, an MRI-brain was done which showed diffuse mild loss of white matter. Consequently, blood investigations showed macro-to-microcytic hypochromic anemia. Thereafter, the child was started on injectable B12 supplementation and propanolol at 0.5 mg/kg/day for tremors. The child responded well clinically.

Common invisible symptoms of multiple sclerosis

Multiple sclerosis (MS) with its protean manifestations of central and peripheral nervous system pose a challenge to its management in different clinical scenario. The issue becomes much more complicated with occurrence of strange and unusual symptoms intermingling with common symptoms in people living with MS. The frequency and severity of these unusual symptoms changes overtime and raise a suspicion of other neurological disease. The occurrence of any of these symptoms at times may be a sign of active disease. A reasonable understanding of these strange symptoms both to the patient and healthcare team could be of help in taking appropriate steps to manage MS more efficiently

Clinical study on diazepam combined with olanzapine in treatment of agitation in alcohol withdrawal delirium tremors / 中华行为医学与脑科学杂志

Objective To compare the efficacy and safety of diazepam combined with olanzapine in treatment of agitation symptoms in patients with alcohol withdrawal delirium tremors( DT) . Methods Total-ly 60 inpatients with DT according to CCMD-3 were enrolled in a 7-day,with 30 patients in the diazepam group ( control group) and 30 patients in diazepam combined with olanzapine group ( research group) . The delirium rating scale( DRS-R-98) was used to evaluate the severity of the delirium symptoms. The positive and negative syndrome scale excited component( PANSS-EC) was used to assess the agitation symptoms. The treatment emergent symptom scale ( TESS) was used to assess the safety and the vital signs. Results There was no significant difference in the delirium recovery time between the two groups(M(QR):24 h(24 h)vs 24 h(6 h))(Z=-0. 45,P=0. 65). The PANSS-EC score in research group(17. 00±2. 67) was significant low-er than that in control group(19. 80±2. 43) at the 6 hours after treatment(t=4. 26,P<0. 01),but there were no significant difference between the two groups at the baseline,24 h,48 h and 72 h(P>0. 05) . One case of dizziness,2 cases of lethargy in the control group;1 case of nasal congestion,5 cases of drowsiness,3 cases of constipation, 3 cases of dry mouth, 1 case of abnormal liver function in the research group. Conclusion The combination of olanzapine with diazepam can not reduce the recovery time of DT,but can quickly improve the agitation behavior.

Reversible Cerebral Atrophy in Infantile Tremor Syndrome.

Background: We report changes in MRI brain of children with Infantile Tremor Syndrome (ITS) at the onset of illness and following treatment. Case characteristics: Three children with infantile tremor syndrome were assessed for changes in brain neuroimaging at admission and at follow-up visit. On MRI, all children had mild to severe diffuse cerebral atrophy, which reverted back to normal on follow-up visits. Outcome: Children with infantile tremor syndrome have reversible diffuse cerebral atrophy on neuroimaging.

Síndrome de temblor y ataxia asociado a frágil X (FXTAS): revisión dela literatura / Fragile X associated tremor/ataxia syndrome: literature review

El síndrome de temblor y ataxia asociado al síndrome del cromosoma X frágil (FXTAS) es un desorden neurodegenerativoprogresivo (1), de inicio tardío, que ocurre entre los portadores de la premutación del gen FMR1(Fragile X Mental Retardation 1), el cual está estrechamente asociado con el síndrome del cromosoma X frágil(FXS). El FXTAS se caracteriza por déficits neurológicos que incluyen temblor de intención progresivo, ataxiacerebelosa, parkinsonismo, neuropatía periférica, déficits cognitivos y disfunción autonómica (2-4).El FXTAS surge como una importante opción diagnóstica en hombres con temblor, alteraciones en la marchay síntomas neurodegenerativos. En general existe subregistro de esta patología dado que es un síndrome recientementedescrito y falta conocimiento de los profesionales de salud al respecto, los cuales, debido a la similitudde su presentación clínica con otros desórdenes neurológicos, generalmente suelen confundir el diagnóstico (5).En Colombia no se ha documentado la prevalencia de SXF o de FXTAS. Sin embargo, se ha descrito un corregimientoen el Valle del Cauca que tiene una prevalencia de más de cien veces lo reportado en la literatura deSFX, lo que nos sugiere que en Colombia existe subregistro del SFX y de FXTAS.Esta revisión tiene por objeto difundir los avances del conocimiento de las manifestaciones clínicas, la neurofisiopatologíay las posibilidades de tratamiento de los pacientes con FXTAS, y así aumentar diagnóstico yaportar a mejorar la calidad de vida de los afectados y de sus familias.

Intoxicação por Ipomoea asarifolia em bovinos e ovinos no Rio Grande do Norte / Ipomoea asarifolia poisoning in cattle in Rio Grande do Norte

Ipomoea asarifolia é uma das principais plantas tóxicas para ruminantes nas regiões Norte e Nordeste. A intoxicação ocorre na época das secas quando há escassez de forragem e é mais frequente em ovinos do que em outras espécies, porem casos espontâneos também são relatados em caprinos, bovinos e búfalos. Este trabalho relata a ocorrência de 18 surtos de intoxicação em bovinos por I. asarifolia que ocorreram de agosto de 2012 a fevereiro de 2013 no estado do Rio Grande Norte. De um total de 1704 bovinos, 202 (11,8%) adoeceram e 34 (2%) morreram. Em seis propriedades onde havia ovinos estes também se intoxicaram. Foram observados também sinais clínicos em bezerros e cordeiros confinados, sem acesso às pastagens, que se intoxicaram pela ingestão do leite das mães que ingeriam I. asarifolia a pasto.(AU)

Ipomoea asarifolia is one of the main poisonous plants in the North and Northeast of Brazil. The poisoning occurs during the dry season when there is shortage of forage, and is more common in sheep; however spontaneous cases are also reported in goats, cattle and buffaloes. This paper reports 18 outbreaks of poisoning in cattle by I. asarifolia which occurred from August 2012 to February 2013 in the state of Rio Grande do Norte. From a total of 1,704 cattle, 202 (11.8%) were affected and 34 (2%) died. On six farms sheep were also affected. Poisoning outbreaks were also observed in lambs and calves that had no access to the pastures but suckled milk from cows and ewes that had ingested I. asarifolia.(AU)

Characteristics of Surface Electromyogram of Forearm Muscles of Parkinson's Disease Patients with or without Load / 中国康复理论与实践

Objective To explore the characteristics of surface electromyogram (sEMG) of the forearm muscles of Parkinson's disease patients with or without load. Methods 26 Parkinson's disease patients and 28 healthy individuals participated in this study. All the subjects performed isometric contraction for elbow flexion without load or with a load of 1.5 kg. The sEMG signals were collected by surface elec-trodes and processed by linear time and frequency domain method. Results The values of median frequency (MF) and mean power frequen-cy (MPF) were higher in the patients than in the healthy controls without load (P<0.05). The value of average EMG (AEMG) was lower in the patients than in the healthy controls with a load of 1.5 kg (P<0.001). For the patients, the values of MF and MPF were higher without load than with a load of 1.5 kg (P<0.001), and the value of AEMG was lower without load than with a load of 1.5 kg (P<0.001). For the healthy controls, only the value of AEMG was lower without load than with a load of 1.5 kg (P<0.001). Conclusion The values of MF and MPF are higher in Parkinson's disease patients than in the healthy controls without load while the value of AEMG is lower in Parkinson's dis-ease patients than in the healthy controls with load.

Niemann-Pick Disease Type A Presenting as Unilateral Tremors.

Niemann-Pick group of diseases are rare lysosomal storage disorders. The clinical phenotype is variable. We report a child who first time presented with tremors of tongue and tremors of one side of the body. On examination child had hemiparesis and hepatosplenomegaly. Bone marrow examination shows storage cells suggestive of Niemann-Pick cells and enzyme assay confirmed the diagnosis.

Controle de temperatura em intervenção cirúrgica abdominal convencional: comparação entre os métodos de aquecimento por condução e condução associada à convecção / Control de temperatura en intervención quirúrgica abdominal convencional: comparación entre los métodos de calentamiento por conducción y conducción asociada a la convección / Temperature control in conventional abdominal surgery: comparison between conductive and the association of conductive and convective warming

JUSTIFICATIVA E OBJETIVOS: Hipotermia intra-operatória é complicação frequente, favorecida por operação abdominal. A eficácia da associação dos métodos de aquecimento por condução e convecção na prevenção de hipotermia e seus efeitos no período de recuperação pós-operatória foram os objetivos deste estudo. MÉTODO: Quarenta e três pacientes de ambos os sexos de 18 a 88 anos de idade, submetidos à laparotomia xifopúbica sob anestesia geral e monitorização da temperatura esofágica, foram distribuídos de modo aleatório em dois grupos de aquecimento: COND (n = 24), com colchão de circulação de água a 37ºC no dorso e COND + CONV (n = 19), com a mesma condição associada à manta de ar aquecido a 42ºC sobre o tórax e membros superiores. Analisados peso, sexo, idade, duração da operação e anestesia, temperaturas na indução anestésica (Mi), horas consecutiva (M1, M2), final da operação (Mfo) e anestesia (Mfa), entrada (Me-REC) e saída (Ms-REC) da recuperação pós-anestésica (SRPA), além das incidências de tremores e queixas de frio no pós-operatório. RESULTADOS: Os grupos foram semelhantes em todas as variáveis analisadas, exceto nas temperaturas em M2, M3, M4, Mfo e Mfa. O grupo COND reduziu a temperatura a partir da segunda hora da indução anestésica, mas o grupo COND + CONV só na quarta hora. Em COND, observou-se hipotermia na entrada e saída da SRPA. CONCLUSÕES: Associar métodos de aquecimento retardou a instalação e diminui a intensidade da hipotermia intra-operatória, mas não reduziu a incidência das queixas de frio e tremores.

JUSTIFICATIVA Y OBJETIVOS: La Hipotermia intraoperatoria es una complicación frecuente, favorecida por la operación abdominal. La eficacia de la asociación de los métodos de calentamiento por conducción y convección en la prevención de hipotermia y sus efectos en el período de recuperación postoperatoria, fueron los objetivos de este estudio. MÉTODO: Cuarenta y tres pacientes de los dos sexos, entre 18 y 88 años de edad, sometidos a la laparotomía xifopúbica bajo anestesia general y monitorización de la temperatura esofágica, aleatoriamente distribuidos en dos grupos de calentamiento: COND (n = 24) colchón de circulación de agua a 37,0ºC en el dorso y COND + CONV (n = 19) la misma condición asociada a la manta de aire calentado a 42ºC sobre el tórax y los miembros superiores. Se analizó el peso, sexo, edad, duración de la operación y anestesia, temperaturas en la inducción anestésica (Mi), horas consecutiva (M1, M2), final de la operación (Mfo) y anestesia (Mfa), entrada (Me-REC) y salida (Ms-REC) de la recuperación postanestésica (SRPA), además de las incidencias de temblores y quejidos de frío en el postoperatorio. RESULTADOS: Los grupos fueron similares en todas las variables analizadas, excepto en las temperaturas en M2, M3, M4, Mfo y Mfa. El Grupo COND redujo la temperatura a partir de la segunda hora de la inducción anestésica, pero el grupo COND + CONV sólo en la cuarta hora. En COND se observó una hipotermia en la entrada y en la salida de la SRPA. CONCLUSIONES: El asociar métodos de calentamiento, retardó la instalación y redujo la intensidad de la hipotermia intraoperatoria, pero no redujo la incidencia de los quejidos de frío y los temblores.

BACKGROUND AND OBJECTIVES: Intraoperative hypothermia is a common complication, and its development is favored by abdominal surgeries. The efficacy of the association of conductive and convective warming methods in the prevention of hypothermia, and its effects during postoperative recovery were the objectives of this study. METHODS: Forty-three patients of both genders, ages 18 to 88 years, undergoing xyphopubic laparotomy under general anesthesia and monitoring of the esophageal temperature were randomly divided in two groups, according to the warming method: COND (n = 24), circulating-water mattress at 37º C on the back, and COND + CONV (n = 19), circulating-water mattress associated with warm air blanket at 42º C over the thorax and upper limbs. Weight, gender, age, duration of surgery and anesthesia, temperature on anesthetic induction (Mi), consecutive hours (M1, M2), end of surgery (Mes) and anesthesia (Mea), and admission (Ma-REC) and discharge (Md-REC) from the post-anesthetic recovery room (PARR), besides the postoperative incidence of tremors and complaints of cold, were analyzed. RESULTS: Both groups were similar regarding all parameters analyzed, except temperatures on M2, M3, M4, Mes, and Mea. The temperature of patients in the COND group decreased from the second hour of anesthetic induction on, but in the COND + CONV group it only happened in the fourth hour. Patients in the COND group presented hypothermia upon admission and discharge from the PARR. CONCLUSIONS: The association of different warming methods delayed the beginning and reduced the severity of intraoperative hypothermia, but it did not reduce the complaints of feeling cold and tremors.