Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 23
Filter
1.
Arch. argent. pediatr ; 119(1): e54-e57, feb. 2021. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1147264

ABSTRACT

La mioclonía palatina esencial es una entidad otoneurológi-ca rara. Se caracteriza por movimientos involuntarios de los músculos del paladar blando que causan un tinnitus objetivo.La mioclonía del paladar se clasifica en dos tipos: secundaria y primaria (mioclonía palatina esencial); esta última es más frecuente en pediatría. La tomografía computada y la reso-nancia magnética de cerebro normal orientan al diagnóstico. La mioclonía palatina esencial, generalmente, se resuelve en forma espontánea.Se presenta a una paciente de 8 años de edad con un "clic" rápido en forma rítmica en su boca que cedía en forma espontánea


Essential palatal myoclonus is a rare neurological disorder characterized by involuntary movements of the soft palate musculature causing objective-clicking tinnitus. The palatal myoclonus is classified in two forms, secondary and essential palatal myoclonus. Primary (essential) palatal myoclonus is the most common type in childhood. Normal computed tomography and magnetic resonance guide the diagnosis. Spontaneous resolution usually occurs in the essential type of palatal myoclonus.In this report, we present an 8-year-old child making rhythmic, rapid clicking noises from her throat with spontaneous resolution.


Subject(s)
Humans , Female , Child , Myoclonus/diagnosis , Pediatrics , Tinnitus , Myoclonus/therapy
2.
Arq. neuropsiquiatr ; 78(8): 512-522, Aug. 2020. tab, graf
Article in English | LILACS | ID: biblio-1131740

ABSTRACT

ABSTRACT Background: Neurophysiological studies are ancillary tools to better understand the features and nature of movement disorders. Electromyography (EMG), together with electroencephalography (EEG) and accelerometer, can be used to evaluate a hypo and hyperkinetic spectrum of movements. Specific techniques can be applied to better characterize the phenomenology, help distinguish functional from organic origin and assess the most probable site of the movement generator in the nervous system. Objective: We intend to provide an update for clinicians on helpful neurophysiological tools to assess movement disorders in clinical practice. Methods: Non-systematic review of the literature published up to June 2019. Results: A diversity of protocols was found and described. These include EMG analyses to define dystonia, myoclonus, myokymia, myorhythmia, and painful legs moving toes pattern; EMG in combination with accelerometer to study tremor; and EEG-EMG to study myoclonus. Also, indirect measures of cortical and brainstem excitability help to describe and diagnose abnormal physiology in Parkinson's disease, atypical parkinsonism, dystonia, and myoclonus. Conclusion: These studies can be helpful for the diagnosis and are usually underutilized in neurological practice.


RESUMO Introdução: Os estudos neurofisiológicos são métodos auxiliares para compreender melhor as características e a natureza dos distúrbios do movimento. A eletromiografia (EMG), em associação com o eletroencefalograma (EEG) e o acelerômetro, podem ser utilizados para avaliar um espectro de movimentos hipo e hipercinéticos. Técnicas específicas podem ser aplicadas para melhor caracterizar a fenomenologia, ajudar a distinguir a origem psicogênica da orgânica e avaliar o local mais provável de geração do movimento no sistema nervoso. Objetivo: Pretendemos fornecer ao clínico uma atualização sobre ferramentas neurofisiológicas úteis para avaliar distúrbios do movimento na prática clínica. Métodos: Revisão não sistemática da literatura publicada até junho de 2019. Resultados: Uma diversidade de protocolos foi encontrada e descrita. Dentre eles, inclui-se o uso de EMG para a definição do padrão de distonia, mioclonia, mioquimia, miorritmia e painfull legs moving toes, além do uso de EMG em associação ao acelerômetro para avaliar tremor e, em associação ao EEG para avaliar mioclonia. Ademais, técnicas para medida indireta de excitabilidade cortical e do tronco encefálico ajudam a descrever e diagnosticar a fisiologia anormal da doença de Parkinson, parkinsonismo atípico, distonia e mioclonia. Conclusão: Esses estudos podem ser úteis para o diagnóstico e geralmente são subutilizados na prática neurológica.


Subject(s)
Humans , Dystonia , Movement Disorders/diagnosis , Myoclonus/diagnosis , Tremor/diagnosis , Electroencephalography , Electromyography , Neurophysiology
3.
Rev. cuba. med ; 52(1): 72-77, ene.-mar. 2013.
Article in Spanish | LILACS | ID: lil-671315

ABSTRACT

La mioclonía poshipoxia crónica o síndrome de Lance Adams es una complicación rara que se produce en pacientes que sobreviven a la hipoxia o la hipotensión prolongada, días o semanas después del daño cerebral. Se presentó un caso con este síndrome, secundario a shock hipovolémico por embarazo ectópico roto. El electroencefalograma con ausencia de paroxismos apoya el origen subcortical de las mioclonías, con respuesta favorable al alonazepán. Se detallaron estudios de neuroimagen y potenciales evocados auditivos de tallo cerebral


The chronic poshipoxia myoclonus or Lance Adams syndrome is a rare complication that occurs in patients who survive prolonged hypoxia or hypotension, days or weeks after the complication. A case with this syndrome, hypovolemic shock secondary to a ruptured ectopic pregnancy is presented. EEG with no paroxysms supports the origin of myoclonus subcortical with favorable response to alonazepán. Neuroimaging and auditory evoked potentials and brainstem studies were detailed


Subject(s)
Humans , Female , Young Adult , Cerebellar Ataxia/complications , Cerebellar Ataxia/rehabilitation , Myoclonus/diagnosis , Myoclonus/etiology
4.
Rev. bras. neurol ; 47(3): 7-15, jul.-set. 2011. tab
Article in Portuguese | LILACS | ID: lil-621072

ABSTRACT

Mioclonias são movimentos involuntários de instalação súbita e de duração breve, de rara ocorrência (2%), mesmo em serviços especializados. São classificadas de acordo com a sua distribuição, origem e etiologia. O diagnóstico se baseia, fortemente, na apresentação clínica associada às alterações eletrofisiológicas. O tratamento medicamentoso deve ser orientado conforme a sua origem anatômica, destacando-se as seguintes drogas: clonazepam, levetiracetam, piracetam e valproato de sódio. A politerapia é mais eficaz do que a monoterapia, particularmente nas mioclonias de origem cortical. O objetivo desta revisão é enfatizar o diagnóstico e o tratamento atual das condições mais expressivas observadas na prática neurológica, tais como: distonia-mioclônica, mioclonia proprioespinhal, epilepsia com ausência mioclônica, síndrome de West, epilepsia mioclônica juvenil, doença por corpos de Lewy, degeneração corticobasal, panencefalite esclerosante subaguda, doença de Creutzfeldt-Jakob, síndrome de Lance-Adams, mioclonia induzida por drogas, mioclonia medular e mioclonia periférica.


Myoclonus are sudden, brief and rare abnormal movements. They can be classified according to their distribution, origin and etiology. The diagnosis is based largely on the clinical features associated with electrophysiological data. The treatment must be guided according to anatomical origin, highlighting the following drugs: clonazepam, levetiracetam, piracetam and sodium valproate. Polytherapies are more effective than monotherapy, particularly in cortical myoclonus. The aim of this review is to emphasize the current diagnosis and treatment of the more expressive morbid conditions seen in neurological practice, such as: myoclonus-dystonia, propriospinal myoclonus, epilepsy with myoclonic absences, West syndrome, juvenile myoclonic epilepsy, Lewy body disease, corticobasal degeneration, subacute sclerosing panencephalitis, Creutzfeldt-Jakob disease, Lance-Adams syndrome, drug-induced myoclonus, spinal myoclonus and peripheral myoclonus.


Subject(s)
Humans , Male , Child, Preschool , Child , Dyskinesias , Myoclonus/classification , Myoclonus/diagnosis , Myoclonus/drug therapy , Spasms, Infantile , Review Literature as Topic , Creutzfeldt-Jakob Syndrome , Myoclonic Epilepsy, Juvenile , Anticonvulsants/therapeutic use
5.
Rev. chil. neuro-psiquiatr ; 46(4): 288-292, dic. 2008.
Article in Spanish | LILACS | ID: lil-547791

ABSTRACT

Lance-Adams syndrome was described in 1963 is a rare complication due to recovered hypoxic episodes or prolonged hypotension events. Is characterized by action myoclonus and cerebellar ataxia. We report two patients studied with this syndrome. A 51 year-old men and a 72 years-old men fully recovered after a brief cardiorespiratory arrest they developed intention myoclonus, triggered by voluntary movements, posture, also by sounds, touches and emotional stimuli. It also was accompanied by cerebellar syndrome, ataxia and posture control alterations. They had a Magnetic Resonance (MR), EEG and normal metabolic parameters. Myoclonus was treated with sodium valproate and clonazepam. The neurophysiologic interpretation of this motor imbalance is an abnormal functioning of the Central Pattern Generator Netwoks (CPGN) located in the mesencephalic region. Hypoxic lesions in vermian purkinje and paravermal cerebellum neurons have an inhibitory effect in this system, producing motor control attenuation, generating an imbalance in the motoneurons of the spinal cord contraction sequence, which starts shooting in an uncoordinated way. As in almost all cerebellar lesions with time they tend to compensate and to diminish myoclonus.


El Síndrome de Lance-Adams descrito en 1963, es una rara complicación que sigue tardíamente a episodios hipóxicos o de hipotensión prolongada, ya recuperados. Se caracteriza por mioclonías de acción y ataxia cerebelosa. Se describen dos pacientes estudiados con este síndrome. Son dos hombres de 51 y 72 años que después de un paro cardiorrespiratorio breve, de recuperación completa, iniciaron mioclonías de intención, activadas por movimientos voluntarios, posturas, estímulos sonoros, táctiles y afectivos. Acompañado además de un síndrome cerebeloso, ataxia de la marcha y alteraciones del control postural. Cursaron con RM (Resonancia Magnética), EEG (Electroencefalograma) y parámetros metabólicos sin relevancia patológica. Las mioclonías fueron controladas con ácido valproico y clonazepam. La interpretación neurofisiológica de este desajuste motor es la alteración en el funcionamiento del patrón central de circuitos generadores (PCCG) ubicado en la región mesencefálica. Las lesiones hipóxicas de las neuronas de Purkinje del vermis y paravermianas del cerebelo, que tienen un efecto inhibitorio para este sistema, producen una atenuación del control motor del PCCG, generando desajuste en la secuencia de la contracción de las motoneuronas de la médula espinal, que comienzan a dispararse de manera independientemente. Como ocurre con la mayoría de las lesiones cerebelosas, con el tiempo tienden a compensarse y por consiguiente a disminuir las mioclonías.


Subject(s)
Humans , Male , Middle Aged , Hypoxia, Brain/complications , Myoclonus/diagnosis , Myoclonus/etiology , Myoclonus/drug therapy , Valproic Acid/therapeutic use , Anticonvulsants/therapeutic use , Clonazepam/therapeutic use , Cardiopulmonary Resuscitation/adverse effects , Syndrome
6.
Pediatria (Säo Paulo) ; 29(2): 150-153, 2007.
Article in Portuguese | LILACS | ID: lil-471435

ABSTRACT

Objetivo: descrever um caso da síndrome de opsoclonia-mioclonia-ataxia. Relato de caso: uma menina de 2 anos de idade passou a apresentar, há 1 ano, distúrbio da marcha, cefaléia e dificuldade visual. Ao exame físico tinha movimentos...


Objective: to report a case of opsoclonus-myoclonus-ataxia syndrome. Case report: a two years old girl was taken to hospital with a complaint of gait difficulty, headache and visual impairment lasting one year. At physical exam fast eyes movement...


Subject(s)
Humans , Female , Child, Preschool , Gait Ataxia , Neuroblastoma/diagnosis , Paraneoplastic Syndromes, Nervous System/diagnosis , Headache/etiology , Myoclonus/diagnosis , Neuroblastoma , Neuroblastoma/etiology
7.
Article in English | IMSEAR | ID: sea-89083

ABSTRACT

A 55 years old, hypertensive, diabetic lady presented with sudden onset jerky movement of lower trunk and legs. It was present both in awake and sleep and got aggravated by mental stress as well as sensory stimulation. Examination revealed rhythmic jerks affecting muscles of lower abdomen and legs. The lower limbs had normal muscle bulk and power, increased tone, exaggerated deep tendon reflexes, bilateral flexor plantar response with normal sensory autonomic and cerebellar function. Investigations including CSF study, MRI of dorsal spine and NCV were normal. A combination therapy with tizanidine, baclofen and clonazepam induced gradual improvement within 6 weeks.


Subject(s)
Baclofen/therapeutic use , Clonazepam/therapeutic use , Clonidine/analogs & derivatives , Diagnosis, Differential , Humans , Lower Extremity/physiopathology , Middle Aged , Myoclonus/diagnosis , Spinal Cord/physiopathology , Spinal Cord Diseases/diagnosis
9.
J Indian Med Assoc ; 2004 Nov; 102(11): 647-8
Article in English | IMSEAR | ID: sea-105246

ABSTRACT

A 2 year old boy presented with features of opsoclonus, myoclonus and ataxia. Routine investigations of blood, urine, x-ray chest, bone scan, EEG and MRI of brain, were normal. Urine for VMA was negative. A right suprarenal mass was detected at MRI of abdomen. The mass was resected completely and was found histologically to be of differentiating type of neuroblastoma. The child was treated initially with prednisolone for 6 weeks along with sodium valproate. He is still on sodium valproate for his neurological symptoms. His symptoms still persist though they have decreased in intensity.


Subject(s)
Cerebellar Ataxia/diagnosis , Child, Preschool , Diagnosis, Differential , Humans , Magnetic Resonance Imaging , Male , Myoclonus/diagnosis , Neuroblastoma/diagnosis , Ocular Motility Disorders/complications , Paraneoplastic Syndromes, Nervous System/diagnosis , Prednisolone/therapeutic use , Treatment Outcome
10.
Arq. neuropsiquiatr ; 58(3B): 901-4, Sept. 2000. ilus
Article in English | LILACS | ID: lil-273116

ABSTRACT

We describe two cases of palatal myoclonus (PM), one essential and another secondary to a stroke. Case 1: a 64 years old female who developed clicking sounds in both ears after a stroke and three years later on noticed a progressive involuntary movement of the throat associated with rhythmic contractions of the soft palate, muscles of tongue and throat. MRI showed an ischemic area in brainstem. The patient had a partial response to the use of sumatriptan 6 mg subcutaneously. Case 2: a 66 years old female who began with ear clicking at left ear that worsed slowly associated with tinnitus and arrhythmic movements of soft palate and an audible click at left ear. Brain MRI was normal; audiometry showed bilateral neurosensory loss. She was prescribed clonazepan 1 mg daily with complete recovery. Primary and secondary palatal myoclonus share the same clinical features but probably have different pathophysiological underlying mechanisms


Subject(s)
Humans , Female , Middle Aged , Anticonvulsants/therapeutic use , Clonazepam/pharmacology , Myoclonus/drug therapy , Serotonin Receptor Agonists/pharmacology , Sumatriptan/pharmacology , Electromyography , Magnetic Resonance Imaging , Myoclonus/diagnosis , Palate, Soft/drug effects
11.
Bahrain Medical Bulletin. 1998; 20 (2): 53-55
in English | IMEMR | ID: emr-47626

ABSTRACT

Opsoclonus-myoclonus [OPMS] is a rare neurological syndrome of children and adults. Association with neuroblastoma occurs in 20-50% of all cases. We have recently encountered an infant with this syndrome and detailed work up revealed her to have an underlying neuroblastoma. Etiology and pathogenesis of this syndrome have been reviewed, it is important for general paediatricians to recognise this entity in order to initiate investigations and possible treatment


Subject(s)
Humans , Female , Myoclonus/diagnosis , Child
12.
Indian J Pediatr ; 1997 Sep-Oct; 64(5): 583-602
Article in English | IMSEAR | ID: sea-84314

ABSTRACT

The possible associations of myoclonic phenomenae, progressive or non-progressive encephalopathies and epileptic features are reviewed, with special emphasis on pediatric age. This leads to recognize the following five groups of conditions: (1) Myoclonus without encephalopathy and without epilepsy; (2) Encephalopathies with non-epileptic myoclonus; (3) Progressive encephalopathies presenting myoclonus seizures of epileptic syndromes (Progressive myoclonus epilepsies); (4) Epileptic encephalopathies with myoclonic seizures; (5) Myoclonic epilepsies. Within the first group, which also includes physiologic myoclonus, a more thorough description of "Benign sleep myoclonus of newborn" and "Benign myoclonus of early infancy" is given. Characteristics of group 2 are "Kinsbourne Syndrome" and certain types of "Hyperekplexia" which pose interesting differential diagnosis with stimulus-sensitive epilepsies. In group 3, the concept of progressive encephalopathies is stressed. The fourth group refers to severe epilepsies, mainly on infancy and childhood, which lead to mental retardation irrespective of their aetiology. Group 5 comprises the true myoclonic epilepsies, differentiating syndromes recognized as idiopathic--such as "Benign myoclonic epilepsy of infancy" and "Juvenile myoclonic epilepsy"--from those which are cryptogenic and carry a more cautious prognosis--as "Cryptogenic myoclonic and myoclonoastatic epilepsies" and "Severe myoclonic epilepsy of infancy". Other epileptic syndromes not usually considered as myoclonic epilepsies, but presenting sometimes as myoclonic seizures, are finally referred.


Subject(s)
Adolescent , Child , Child, Preschool , Diagnosis, Differential , Electroencephalography , Female , Humans , Infant , Infant, Newborn , Male , Myoclonic Epilepsies, Progressive/diagnosis , Myoclonic Epilepsy, Juvenile/diagnosis , Myoclonus/diagnosis
13.
Arq. neuropsiquiatr ; 52(3): 406-9, set. 1994. ilus
Article in Portuguese | LILACS | ID: lil-141247

ABSTRACT

Tem sido descrita na literatura ocorrência de movimentos mioclônicos e distônicos como expressäo isolada de uma condiçäo neurológica de caráter hereditário. Para essa entidade alguns autores propöem a denominaçäo "distonia mioclônica hereditária", enquanto outros preconizam o uso da expressäo "mioclonia essencial hereditária". O presente relato refere-se a uma família em que essa peculiar associaçäo de movimentos anormais acometia diversos membros em três geraçöes. O propósito é uma paciente de 14 anos com movimentos distônicos instalados aos 7 anos e miocloniais aos 13 anos, com evoluçäo lenta e tendendo à estabilizaçäo. Näo havia referência a melhora sob açäo do álcool (paciente abstêmia). A história familiar mostrava outros casos semelhantes. A investigaçäo complementar (dosagem sericas de cobre, ceruloplasmina, T3, T4, TSH; pesquisa de acantócitos; exame do LCR; tomografia e ressonância do segmento cefálico) näo revelou anormalidades. O quadro foi satisfatoriamente controlado com clonazepam na dose de 3 mg/dia


Subject(s)
Adolescent , Middle Aged , Humans , Female , Dystonia/genetics , Myoclonus/genetics , Clonazepam/therapeutic use , Dystonia/diagnosis , Myoclonus/diagnosis
14.
Rev. bras. neurol ; 29(2): 45-9, mar.-abr. 1993. ilus
Article in Portuguese | LILACS | ID: lil-197999

ABSTRACT

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


Subject(s)
Humans , Female , Adolescent , Electroencephalography , Myoclonic Cerebellar Dyssynergia/diagnosis , Myoclonus/diagnosis , Seizures , Tomography
15.
Neuroeje ; 9(2): 38-43, 1991. ilus
Article in Spanish | LILACS | ID: lil-110116

ABSTRACT

El diagnóstico diferencial de fenómenos paroxísticos convulsivos versus con convulsicos y sobre todo aquellos que ocurren durante el sueño, requiere no solamente de elementos clínicos, sino también de estudios complementarios por medio de variadas técnicas electroencefalográficas. Dentro de los fenómenos que fácilmente podrían ser confundidos con convulsiones están las mioclonías del sueño en el período neonatal. Reportamos dos casos y hacemos una revisión de la literatura, enfatizando en el diagnóstico diferencial y en técnicas de estudio para su soporte


Subject(s)
Humans , Infant, Newborn , Infant , Myoclonus/diagnosis , Spasms, Infantile/diagnosis , Electroencephalography , France
16.
Arq. neuropsiquiatr ; 47(3): 346-51, set. 1989. ilus
Article in English | LILACS | ID: lil-77679

ABSTRACT

Foram examinados dois irmäos portadores de uma síndrome miclónica progressiva comn discretos sintomas cerebelares. O exame neurológico mostrava sinais cerebelares moderados e papilas pálidas; mioclonais assíncronas, arrítmicas e assimétricas, um déficit artrestésico e ausência de fraqueza muscular. A atividade de base do EEG era moderadamente lenta e sem atividade irritativa. A TC era normal em ambos os casos. A estimulaçäo fótica intemitente aumentava a freqüência dos abalos mioclônicos que se tornavam bilaterais e sincronos, progredindo para uma crise tônico-clônica generalizada. Potenciais evocados e RMN em um caso foram normais . Drogas anticonvulsivantes foram ineficazes no controle das mioclonias. O diagníostico de encefalomiopatia mitocondrial foi realizado através do achado em espécimes musculares de membranas basais espessadas, com degeneraçäo miofibrilar e um número elevado de mitocondrias distribuídos perifericamente e com uma matriz densa, granular e com alguns vacúolos. Os achados clínicos e eletrográficos sugerem uma origem subcortical para esta síndrome mioclônica


Subject(s)
Adult , Middle Aged , Humans , Male , Brain Diseases/physiopathology , Mitochondria, Muscle/ultrastructure , Myoclonus/diagnosis , Neuromuscular Diseases/pathology , Electroencephalography , Evoked Potentials , Myoclonus/etiology
18.
Rev. Hosp. Clin. Fac. Med. Univ. Säo Paulo ; 41(5): 210-2, set.-out. 1986. tab
Article in Portuguese | LILACS | ID: lil-36027

ABSTRACT

Foram avaliados 30 pacientes consecutivos com movimentos periódicos do sono, ou miclonias noturnas, diagnosticados por traçado polissonográfico de noite inteira. A média etária foi 50 anos, variando de 30 a 73. Queixa primária de insônia foi vista em 11 casos; sonolência excessiva diurna, em 16 casos; parassonias, em três casos. A média de eficiência do sono foi 83%, sendo que 90% dos pacientes tinham eficiência abaixo de 95%. Estágio 1, durava em média 8,2% do tempo total de sono; estágio 2, 59,8%; estágio 3, 12,3%; estágio 4, 7,1% mas 16 pacientes näo tinham estágio 4, e estágio REM, 16,7%. A latência do estágio Rapid Eye Movement (REM) média, a partir do estágio 2, foi 110,3 m, sendo que 43,3% dos casos tinham este índice abaixo do normal e 23,3% o tinham acima do normal. A densidade REM média foi 11,2%. O número médio de despertares prolongados foi 8,5 e de mudança de estágios foi 49,9. O índice de despertar parcial estava diretamente relacionado à latência REM ao passo que o índice de complexos K estava inversamente relacionado


Subject(s)
Adult , Middle Aged , Humans , Male , Female , Arousal , Myoclonus/diagnosis , Sleep Wake Disorders/diagnosis , Electrodiagnosis , Monitoring, Physiologic , Sleep Stages , Sleep, REM
20.
SELECTION OF CITATIONS
SEARCH DETAIL