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1.
Arch Neurol ; 37(6): 384-5, 1980 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-7387472

RESUMO

Two patients with Fisher's syndrome of ophthalmoplegia, ataxia, and areflexia experienced severe weakness and respiratory distress. Both patients required tracheostomy and assisted ventilation, but both made a complete recovery. Fisher's syndrome is generally considered to be a benign variant of acute infectious polyneuropathy (the Guillain-Barre syndrome). Our two patients demonstrate that the condition is not always benign.


Assuntos
Ataxia/diagnóstico , Oftalmoplegia/diagnóstico , Reflexo Anormal/diagnóstico , Adulto , Feminino , Humanos , Masculino , Transtornos Respiratórios/diagnóstico , Síndrome
2.
Arch Neurol ; 37(4): 249-51, 1980 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-7362494

RESUMO

A 26-year-old man had gelastic seizures ("laughing attacks") preceded by an aura of sexual pleasure. They were reflexly induced by volitional hyperextension of his neck and trunk. His EEGs showed a right temporal spike focus. We discuss the characteristics of this unique case.


Assuntos
Epilepsia do Lobo Temporal/diagnóstico , Riso , Postura , Reflexo Anormal/diagnóstico , Adulto , Eletroencefalografia , Potenciais Evocados , Humanos , Masculino
3.
Arch Neurol ; 44(2): 202-6, 1987 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-3028344

RESUMO

Four patients with botulism were studied on admission and at different times after intoxication, using a battery of cardiovascular autonomic tests. The results were compared with clinical status and single-fiber electromyographic findings. In the early stage of intoxication, the control of heart-rate and blood-pressure responsivity was markedly impaired, as was the neuromuscular transmission. At follow-up, results of sympathetic tests normalized earlier than those of parasympathetic tests. The recovery of autonomic function was slower than that of neuromuscular transmission in three patients. Monitoring autonomic derangement in botulism adds further information on the course of the disease and may identify patients at risk for cardiac or respiratory arrest. Further clinical investigation can help in determining more precisely the autonomic sites where the toxin acts.


Assuntos
Sistema Nervoso Autônomo/fisiopatologia , Botulismo/fisiopatologia , Reflexo Anormal/diagnóstico , Adolescente , Adulto , Pressão Sanguínea , Eletromiografia , Feminino , Frequência Cardíaca , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Músculos/inervação , Junção Neuromuscular/fisiologia , Reflexo Anormal/fisiopatologia , Transmissão Sináptica
4.
Arch Neurol ; 43(2): 150-2, 1986 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-3947255

RESUMO

The neurologic status of ten patients with osteogenesis imperfecta (OI) was evaluated. Four patients with mild OI (type I) had normal neurologic findings and normal computed tomographic scans. Three of these four patients had macrocephaly. All six patients with severe OI (type III) had cortical atrophy on computed tomographic scan and three were also macrocephalic.


Assuntos
Doenças do Sistema Nervoso/diagnóstico , Osteogênese Imperfeita/diagnóstico , Adolescente , Adulto , Encéfalo/anormalidades , Encéfalo/diagnóstico por imagem , Encéfalo/fisiopatologia , Criança , Eletroencefalografia , Feminino , Cabeça/anatomia & histologia , Humanos , Hidrocefalia/diagnóstico , Lactente , Recém-Nascido , Masculino , Destreza Motora , Músculos/fisiopatologia , Osteogênese Imperfeita/diagnóstico por imagem , Radiografia , Reflexo Anormal/diagnóstico
5.
Arch Neurol ; 35(1): 50-2, 1978 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-202235

RESUMO

A patient with a primary malignant lymphoma of the brain manifested a peripheral polyneuropathy during his illness that could not be accounted for by the usual causes. The polyneuropathy is considered to be a nonmetastatic complication of the primary brain tumor. A review of the literature did not uncover a similar case.


Assuntos
Neoplasias Encefálicas/diagnóstico , Corpo Caloso , Linfoma Difuso de Grandes Células B/diagnóstico , Doenças do Sistema Nervoso Periférico/diagnóstico , Neoplasias Encefálicas/patologia , Corpo Caloso/patologia , Doenças Desmielinizantes/patologia , Eletromiografia , Paralisia Facial/diagnóstico , Nervo Femoral/patologia , Humanos , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Condução Nervosa , Quadriplegia/diagnóstico , Reflexo Anormal/diagnóstico
6.
Neurology ; 34(7): 953-6, 1984 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-6539872

RESUMO

Clinical and electrophysiologic data recorded in patients with Fisher syndrome characterized by ophthalmoplegia, ataxia, and areflexia are presented. Cases with limb weakness or pleocytosis in the CSF were excluded, according to Fisher. Ten patients were selected. All had hand and foot numbness. A large amount of protein without cell reaction was found during the third week of illness in serial CSF examinations. EMGs showed very slight limb involvement without spontaneous activity, and in most cases, facial muscles exhibited a denervation pattern. Distal motor nerve conduction velocity on limbs and F wave latencies were normal, whereas the sensory nerve action potentials were altered in all but one case when tested (seven out of eight cases). By means of blink reflex study performed in four patients, no significant pattern of brainstem dysfunction was discovered. The authors discuss the preeminent role of peripheral nerve lesions with regard to the ataxia and ophthalmoplegia.


Assuntos
Ataxia/diagnóstico , Oftalmoplegia/diagnóstico , Reflexo Anormal/diagnóstico , Adolescente , Adulto , Idoso , Eletroencefalografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome
7.
Neurology ; 29(6): 797-801, 1979 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-221856

RESUMO

In three patients, intractable hiccups occurred as part of the symptomatology of multiple sclerosis. In one patient intractable hiccups were the presenting complaint, and in another patient exacerbations of symptoms were almost always heralded by intractable hiccups. Intractable hiccups occur in a variety of diseases, including many that affect the brainstem and cervical cord, but have not been reported in multiple sclerosis. The hiccup may be a "primitive" gastrointestinal reflex that is disinhibited by lesions such as multiple sclerosis plaques. Carbamazepine was successful in arresting the hiccups in one of the cases presented.


Assuntos
Soluço/etiologia , Esclerose Múltipla/diagnóstico , Adolescente , Hormônio Adrenocorticotrópico/uso terapêutico , Adulto , Carbamazepina/uso terapêutico , Feminino , Soluço/tratamento farmacológico , Humanos , Masculino , Esclerose Múltipla/complicações , Esclerose Múltipla/tratamento farmacológico , Prednisona/uso terapêutico , Reflexo Anormal/diagnóstico
8.
Neurology ; 31(5): 555-60, 1981 May.
Artigo em Inglês | MEDLINE | ID: mdl-7194971

RESUMO

Three children, age 2, 7, and 12, developed Fisher syndrome: inability to walk because of ataxia, complete areflexia, and ophthalmoplegia. Ptosis was prominent in all the children, but the pupillary response to light was affected only in one child. Limb weakness was never present, and sensation normal. One patient was obtunded for several days. Two had prodromal upper respiratory tract illnesses, and the third patient was bitten by an insect 2 days before her symptoms began. Cerebrospinal fluid (CSF) protein content was moderately increased in all patients, but only one child's CSF had a pleocytosis. CSF gamma globulin levels were normal. In the acute phase, F waves and H responses were absent in two cases but returned to normal with clinical recovery. All three patients had marked electroencephalographic (EEG) abnormalities that later improved. The EEG pattern suggested a brainstem disorder. These findings plus the obtundation, gaze paralysis, and ataxia indicate that in Fisher syndrome there is parenchymal involvement of the central nervous system with or without nerve root involvement.


Assuntos
Ataxia/diagnóstico , Oftalmoplegia/diagnóstico , Reflexo Anormal/diagnóstico , Ataxia/líquido cefalorraquidiano , Criança , Pré-Escolar , Eletrocardiografia , Feminino , Humanos , Masculino , Contração Muscular , Oftalmoplegia/líquido cefalorraquidiano , Reflexo Anormal/líquido cefalorraquidiano , Síndrome
9.
Neurology ; 38(10): 1630-1, 1988 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-3419608

RESUMO

Three patients with Fisher's syndrome had normal MRI scans of the brainstem 6 days, 7 days, and 5 weeks, respectively, after the onset of illness. This further supports a peripheral origin for the ataxia and ophthalmoplegia in this illness and distinguishes it from brainstem encephalitis.


Assuntos
Ataxia/diagnóstico , Tronco Encefálico/patologia , Oftalmoplegia/diagnóstico , Reflexo Anormal/diagnóstico , Idoso , Ataxia/diagnóstico por imagem , Tronco Encefálico/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Oftalmoplegia/diagnóstico por imagem , Reflexo Anormal/diagnóstico por imagem , Síndrome , Tomografia Computadorizada por Raios X
10.
Neurology ; 29(6): 893-6, 1979 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-572016

RESUMO

Four episodes of acute left hemiparesis occurred in three pediatric-age, insulin-dependent diabetic patients. Each had a concomitant respiratory tract infection and headache, but no obvious signs of systemic hypoglycemia. No cerebral abnormalities were demonstrated by angiography or computerized tomography. The clinical course was benign, with slow but complete resolution of neurologic abnormalities within 8 to 24 hours of onset, and no further neurologic complaints in a 7- to 16-month follow-up.


Assuntos
Diabetes Mellitus Tipo 1/complicações , Neuropatias Diabéticas/diagnóstico , Hemiplegia/diagnóstico , Doença Aguda , Adolescente , Glicemia/metabolismo , Angiografia Cerebral , Criança , Pré-Escolar , Diabetes Mellitus Tipo 1/tratamento farmacológico , Eletroencefalografia , Paralisia Facial/diagnóstico , Feminino , Humanos , Insulina/uso terapêutico , Masculino , Reflexo Anormal/diagnóstico , Infecções Respiratórias/complicações , Tomografia Computadorizada por Raios X
11.
Pediatrics ; 63(2): 238-41, 1979 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-108661

RESUMO

The serious prognostic implications of familial dysautonomia (FD) for the affected individual and his family make early definitive diagnosis mandatory. Familial dysautonomia has rarely been diagnosed in the neonatal period in hitherto unaffected families. We describe here three such newborn patients to reinforce the limited data available on this subject. In spite of the variability of expression and the incompleteness of the manifestations of FD in the neonatal period, as well as the presence of a number of "dysautonomic" features in normal newborns, we believe that it is possible to establish a diagnosis of FD neonatally. We pay particular note to the altered state of consciousness and behavior in neonatal FD, the unusual posture and limb movements, and the swallowing disorder with tendency to neonatal aspiration. In addition, the incidental finding of bile pigment in the amniotic fluid of an affected fetus without hemolytic disease may hint at a possible approach to fetal diagnosis of this condition.


Assuntos
Disautonomia Familiar/diagnóstico , Diagnóstico Diferencial , Nutrição Enteral , Feminino , Humanos , Recém-Nascido , Masculino , Reflexo Anormal/diagnóstico
12.
Pediatrics ; 62(2): 205-8, 1978 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-693157

RESUMO

Behcet's disease is a chronic, recurrent, inflammatory disease of unknown cause that involves mucous surfaces, skin, eyes, joints, and the CNS. Although not reported in the pediatric literature, some cases start in childhood. This report describes a child whose chronic oral and genital ulcers preceded recognition of her gastrointestinal tract, eye, and CNS disease for nine years. The current knowledge of Behcet's disease in children is discussed.


Assuntos
Síndrome de Behçet/diagnóstico , Estomatite Aftosa/diagnóstico , Adolescente , Criança , Pré-Escolar , Doença Crônica , Doença de Crohn/diagnóstico , Diagnóstico Diferencial , Erros de Diagnóstico , Feminino , Herpes Simples/diagnóstico , Humanos , Irite/diagnóstico , Períneo , Pioderma/diagnóstico , Reflexo Anormal/diagnóstico , Úlcera Cutânea/diagnóstico
13.
Urology ; 15(4): 432-3, 1980 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-7394974

RESUMO

The results of combined cystometry and perineal electromyography were reviewed retrospectively in 50 consecutive, traumatic spinal cord-injured patients. Early and frequent urodynamic evaluation was found necessary in order to tailor treatment to the changing cystometric and electromyographic states in these patients.


Assuntos
Períneo/fisiopatologia , Traumatismos da Medula Espinal/fisiopatologia , Bexiga Urinária/fisiopatologia , Eletromiografia , Humanos , Reflexo Anormal/diagnóstico , Traumatismos da Medula Espinal/complicações , Doenças da Bexiga Urinária/diagnóstico , Doenças da Bexiga Urinária/etiologia , Bexiga Urinaria Neurogênica/diagnóstico
14.
Urology ; 13(1): 109-10, 1979 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-442313

RESUMO

Rapid-fill gas cystometry has been criticized for giving a falsely low value of bladder volume and for inducing an "artefact", the hyperreflexic bladder. This study in 72 patients revealed that the average capacity with slow-fill air cystometry was less than with rapid fill. The diagnosis of hyperreflexic bladder was made more often with slow fill than with rapid fill in these 72 patients.


Assuntos
Reflexo Anormal/diagnóstico , Doenças da Bexiga Urinária/diagnóstico , Bexiga Urinária/fisiopatologia , Adulto , Pré-Escolar , Humanos , Reflexo Anormal/fisiopatologia , Doenças da Bexiga Urinária/fisiopatologia , Bexiga Urinaria Neurogênica/diagnóstico , Bexiga Urinaria Neurogênica/fisiopatologia
15.
Urology ; 26(4): 423-5, 1985 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-2931887

RESUMO

Twenty-three patients who previously demonstrated no detrusor contraction during cystometry were evaluated using a combined uroflowmetry and abdominal wall electromyography. Ten patients voided without abdominal straining (normal detrusor reflexia), 11 could not void without abdominal straining (detrusor areflexia, detrusor weakness, or bladder outlet obstruction), and 2 had equivocal findings with the method.


Assuntos
Músculos Abdominais/fisiopatologia , Contração Muscular , Reflexo Anormal/diagnóstico , Bexiga Urinária/fisiopatologia , Micção , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Eletromiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reto/fisiopatologia , Reflexo Anormal/fisiopatologia , Reologia , Doenças da Bexiga Urinária/diagnóstico , Doenças da Bexiga Urinária/fisiopatologia , Urodinâmica
16.
Urology ; 15(4): 379-88, 1980 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-7190336

RESUMO

Overinhibition or underfacilitation of the pudendal nucleus may cause profound involuntary relaxation of the striated muscles of the pelvic floor and perineum, including the "external" striated urethral sphincter. Pudendal nucleus instability may adversely affect urethral closure pressure and preclude a successful voluntary "recovery" effort to limit stress incontinence. It may also result in the involuntary loss of an important source of inhibition to the sacral micturition reflex center, which can lead to detrusor instability. The bilateral reciprocal relationship which exists between the detrusor reflex and the pelvic floor muscle tonus is fundamental to micturition instability syndromes. In this article, the pathophysiology of interaction of pudendal nucleus instability, detrusor reflex instability, and stress is described, and the importance of careful diagnostic evaluation of each case of incontinence is emphasized.


Assuntos
Reflexo Anormal/complicações , Uretra/inervação , Incontinência Urinária/fisiopatologia , Eletromiografia , Feminino , Humanos , Masculino , Neurônios Motores/fisiologia , Pelve/inervação , Períneo/inervação , Reflexo Anormal/diagnóstico , Uretra/fisiopatologia , Incontinência Urinária/diagnóstico , Incontinência Urinária/etiologia , Incontinência Urinária/terapia , Incontinência Urinária por Estresse/fisiopatologia , Micção
17.
Urology ; 33(1): 80-2, 1989 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-2911934

RESUMO

Tethered cord syndrome, a form of spinal dysrhaphism, may involve vesical neurologic dysfunction. We present herein 60 cases of tethered cord syndrome, including 24 patients who underwent preoperative urodynamics studies. Preoperative cystometrography revealed areflexic bladders in 71 percent of cases and hyperreflexic bladders in 29 percent. Eight patients had serial pre- and postoperative urodynamics testing. Slight improvement was noted in postoperative urodynamics studies performed on 4 of 6 areflexic bladders and in the only hyperreflexic bladder studied. In addition, of the 3 patients in the serial urodynamics groups who had urinary symptoms preoperatively, 2 noted clinical improvement postoperatively. The urologist's role is important in the early detection, evaluation, and treatment of tethered cord syndrome and the neuropathic bladders that may result.


Assuntos
Defeitos do Tubo Neural/complicações , Medula Espinal/anormalidades , Bexiga Urinaria Neurogênica/etiologia , Adolescente , Criança , Pré-Escolar , Eletromiografia , Humanos , Defeitos do Tubo Neural/cirurgia , Reflexo Anormal/diagnóstico , Reflexo Anormal/etiologia , Estudos Retrospectivos , Medula Espinal/cirurgia , Síndrome , Bexiga Urinaria Neurogênica/diagnóstico , Urodinâmica
18.
J Neurol ; 229(1): 45-54, 1983.
Artigo em Inglês | MEDLINE | ID: mdl-6189976

RESUMO

An 18-year-old boy showed childhood onset of mental retardation, neurogenic muscle atrophy with hyperreflexia, Marfan-like features, multiple epiphyseal dysplasia, increased urinary excretion of dermatan sulfate, and decreased lysosomal enzyme activities in beta-galactosidase, beta-glucuronidase, and N-acetyl-beta-D-glucosaminidase. This case may be a new syndrome, the combination of neurogenic muscle atrophy with lysosomal enzyme deficiencies.


Assuntos
Mucopolissacaridoses/diagnóstico , Atrofia Muscular/diagnóstico , Adolescente , Diagnóstico Diferencial , Humanos , Deficiência Intelectual/diagnóstico , Leucócitos/enzimologia , Masculino , Erros Inatos do Metabolismo/diagnóstico , Muramidase/metabolismo , Reflexo Anormal/diagnóstico , Síndrome
19.
J Neurol ; 229(1): 33-44, 1983.
Artigo em Inglês | MEDLINE | ID: mdl-6189975

RESUMO

Fisher's syndrome was studied in six patients, one of whom was an 11-year-old girl, and the nosological position of this neuro-ophthalmological disorder was reviewed. The clinical features of the ophthalmoplegia in these cases were reported as secondary to a transient inflammatory lesion in the mid-brain; this was demonstrated in one of the cases by an enhancing area on CT scan. This mid-brain localization could explain not only the classical Fisher's triad but also many variations of the syndrome. The overlap of our cases of Fisher's syndrome with so-called brain-stem encephalitis of Bickerstaff has been emphasized. Many cases of Fisher's syndrome could therefore correspond to an inflammatory process at brain-stem level with or without nerve-root involvement. Clinical and neurophysiological findings show, however, that there is peripheral damage in some cases, so that a neutral attitude needs to be maintained on the nosological position of Fisher's syndrome.


Assuntos
Ataxia/complicações , Oftalmoplegia/complicações , Polirradiculoneuropatia/diagnóstico , Reflexo Anormal/complicações , Doença Aguda , Adulto , Ataxia/diagnóstico , Criança , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-Idade , Oftalmoplegia/diagnóstico , Reflexo Anormal/diagnóstico , Reflexo de Estiramento , Síndrome
20.
Schizophr Bull ; 7(1): 1-11, 1981.
Artigo em Inglês | MEDLINE | ID: mdl-7233095

RESUMO

The physical and psychological characteristics of hypotonic schizophrenic children are described. It is hypothesized that hypotonic schizophrenia constitutes a homogeneous subgroup within the schizophrenic spectrum. The criteria for diagnosing hypotonic schizophrenia are stated. By selecting a homogeneous subgroup of schizophrenic patients, investigators may improve the probability of identifying a common biochemical etiology within this subgroup.


Assuntos
Hipotonia Muscular/diagnóstico , Esquizofrenia/diagnóstico , Transtorno Autístico/diagnóstico , Criança , Transtornos Globais do Desenvolvimento Infantil/diagnóstico , Pré-Escolar , Diagnóstico Diferencial , Humanos , Masculino , Reflexo Anormal/diagnóstico , Síndrome
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