RESUMO
Background: The wing-beating tremor, characteristic of Wilson's disease (WD), is a disabling symptom that can be resistant to anti-copper and anti-tremor medications. Phenomenology Shown: This video illustrates severe bilateral wing-beating tremor, moderate head and lower limb tremors, mild cervical dystonia, and subtle cerebellar ataxia, with nearly resolution after penicillamine treatment. Educational Value: This case highlights a typical aspect of WD, emphasizing the importance of early detection and treatment, and its correlation with MRI findings. Highlights: This case highlights the typical wing-beating tremor in Wilson's disease and its correlation with the involvement of the dentato-rubro-thalamic pathway. The early diagnosis and initiation of treatment with penicillamine resulted in an excellent clinical and radiological response.
Assuntos
Degeneração Hepatolenticular , Penicilamina , Humanos , Cobre/farmacologia , Degeneração Hepatolenticular/diagnóstico por imagem , Degeneração Hepatolenticular/tratamento farmacológico , Imageamento por Ressonância Magnética , Penicilamina/uso terapêutico , Tremor/diagnóstico por imagem , Tremor/tratamento farmacológico , Tremor/etiologiaRESUMO
BACKGROUND: Prelemniscal radiations (Raprl) are composed of different fiber tracts, connecting the brain stem and cerebellum with basal ganglia and cerebral cortex. In Parkinson disease (PD), lesions in Raprl induce improvement of tremor, rigidity, and bradykinesia in some patients, while others show improvement of only 1 or 2 symptoms, suggesting different fiber tracts mediate different symptoms. OBJECTIVE: To search for correlations between improvements of specific symptoms with surgical lesions of specific fiber tract components of Raprl in patients with PD. METHODS: A total of 10 patients were treated with unilateral radiofrequency lesions directed to Raprl. The improvement for tremor, rigidity, bradykinesia, posture, and gait was evaluated at 24 to 33 mo after operation through the Unified Parkinson's Disease Rating Scale (UPDRS) score, and the precise location and extension of lesions through structural magnetic resonance imaging and probabilistic tractography at 6 to 8 mo postsurgery. Correlation between percentage of fiber tract involvement and percentage of UPDRS-III score improvement was evaluated through Spearman's correlation coefficient. RESULTS: Group average improvement was 86% for tremor, 62% for rigidity, 56% for bradykinesia, and 45% for gait and posture. Improvement in global UPDRS score correlated with extent of lesions in fibers connecting with contralateral cerebellar cortex and improvement of posture and gait with fibers connecting with contralateral deep cerebellar nuclei. Lesion of fibers connecting the globus pallidum with pedunculopontine nucleus induced improvement of gait and posture over other symptoms. CONCLUSION: Partial lesion of Raprl fibers resulted in symptom improvement at 2-yr follow-up. Lesions of selective fiber components may result in selective improvement of specific symptoms.
Assuntos
Doença de Parkinson , Humanos , Imageamento por Ressonância Magnética , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico por imagem , Tremor/diagnóstico por imagem , Tremor/etiologiaAssuntos
Ataxia/diagnóstico por imagem , Síndrome do Cromossomo X Frágil/diagnóstico por imagem , Transtornos de Início Tardio/diagnóstico por imagem , Paralisia Supranuclear Progressiva/diagnóstico por imagem , Tremor/diagnóstico por imagem , Idoso de 80 Anos ou mais , Ataxia/complicações , Ataxia/genética , Feminino , Síndrome do Cromossomo X Frágil/complicações , Síndrome do Cromossomo X Frágil/genética , Humanos , Transtornos de Início Tardio/complicações , Transtornos de Início Tardio/genética , Masculino , Pessoa de Meia-Idade , Paralisia Supranuclear Progressiva/complicações , Paralisia Supranuclear Progressiva/genética , Tremor/complicações , Tremor/genéticaRESUMO
Background: Holmes tremor is a rare symptomatic movement disorder, characterized by a combination of resting, postural, and intention tremor. It is usually caused by lesions in the brainstem, thalamus, and cerebellum. Despite pharmacological advances, its treatment remains a challenge; many medications have been used with various degrees of effectiveness. Stereotactic thalamotomy and deep brain stimulation in the ventralis intermedius nucleus have been effective surgical procedures in cases refractory to medical treatment. Case Report: Here we report a young woman with topiramate-responsive Holmes tremor secondary to a brainstem cavernoma. Discussion: Herein we report a Holmes tremor responsive to Topiramate.
Assuntos
Anticonvulsivantes/uso terapêutico , Frutose/análogos & derivados , Tremor/terapia , Adulto , Encéfalo/diagnóstico por imagem , Estimulação Encefálica Profunda , Feminino , Frutose/uso terapêutico , Humanos , Imageamento por Ressonância Magnética , Tálamo/fisiologia , Topiramato , Tremor/diagnóstico por imagemRESUMO
BACKGROUND: Selective improvement of symptoms may be required when treating Parkinson disease (PD) patients with a predominantly monosymptomatic clinical picture. OBJECTIVE: To define a target in prelemniscal radiation fibers (Raprl) related to the physiopathology of tremor evidenced by tractography. CASE REPORT: We report a patient with predominant unilateral rest and postural tremor, diagnosed as PD based on 80% improvement induced by the administration of L-DOPA/carbidopa, subsequently complicated by motor fluctuations, L-DOPA dyskinesia, and a reduced ON period. A stereotactic radiofrequency lesion was made for tremor control, and postoperative diffusion-weighted imaging (DWI) demonstrated the precise location and extension of the lesioned tract. RESULTS: Perfect control of the tremor was achieved with the patient OFF medication; this has lasted for 5 years, without hypotonia in the treated extremities. DWI revealed a 3.0-mm lesion at the base of the nucleus ventralis intermedius (Vim) interrupting cerebellar-Vim fibers sparing the cerebellar ventralis oralis posterior nucleus component. CONCLUSION: Selective improvement of symptoms is feasible in patients with a predominantly monosymptomatic PD clinical presentation.
Assuntos
Doença de Parkinson/diagnóstico por imagem , Doença de Parkinson/cirurgia , Tremor/diagnóstico por imagem , Tremor/cirurgia , Núcleos Ventrais do Tálamo/diagnóstico por imagem , Núcleos Ventrais do Tálamo/cirurgia , Idoso , Carbidopa/uso terapêutico , Combinação de Medicamentos , Humanos , Levodopa/uso terapêutico , Masculino , Doença de Parkinson/tratamento farmacológico , Resultado do Tratamento , Tremor/tratamento farmacológicoRESUMO
Background: The development of Holmes tremor (HT) after a direct lesion of the midbrain has rarely been reported in the literature, although several etiologies have been linked with HT, such as stroke, brainstem tumors, multiple sclerosis, head trauma, or infections. Phenomenology Shown: A 31-year-old male, having been stabbed in the right eye, presented with a rest and action tremor in the left upper limb associated with left hemiparesis with corresponding post-contrast volumetric magnetic resonance imaging T1 with sagittal oblique reformation showing the knife trajectory reaching the right midbrain. Educational Value: Despite the rarity of the etiology of HT in the present case, clinicians working with persons with brain injuries should be aware of this type of situation.
Assuntos
Mesencéfalo/lesões , Tremor/etiologia , Ferimentos Perfurantes/complicações , Adulto , Traumatismos Oculares/complicações , Traumatismos Oculares/diagnóstico por imagem , Traumatismos Oculares/fisiopatologia , Humanos , Masculino , Mesencéfalo/diagnóstico por imagem , Mesencéfalo/fisiopatologia , Paresia/diagnóstico por imagem , Paresia/etiologia , Paresia/fisiopatologia , Tremor/diagnóstico por imagem , Tremor/tratamento farmacológico , Tremor/fisiopatologia , Ferimentos Perfurantes/diagnóstico por imagem , Ferimentos Perfurantes/fisiopatologiaRESUMO
OBJECTIVE: The aim of this work was to study mechanisms of action of electrical stimulation of prelemniscal radiations (Raprl) in the treatment of Parkinson disease, using 2-deoxy-2-fluoro-D-glucose (18F-FDG) Positron Emission Tomography (PET/CT). Materialand Methods: Five patients with PD and predominant unilateral tremor, rigidity and bradykinesia underwent deep brain stimulation (DBS) in contralateral Raprl that improved symptoms from 82.4 to 94.5%. 18F-FDG PET studies were performed before electrode implantation and after DBS therapy. Changes in metabolic activity in PET were evaluated by the maximal standardized uptake value (MSUV) and statistical parametric mapping (SPM) for regions of interest (ROIs) ipsilateral and contralateral to the stimulation site. ROIs were derived from a preoperative probabilistic tractography and included primary motor, supplementary motor and orbitofrontal cortices: Raprl, ventrolateral thalamus, putamen and cerebellum. RESULTS: No significant MSUV changes occurred in ROIs contralateral to Raprl-DBS. In contrast, MSUV decreased ipsilateral to DBS in Raprl, the thalamus, and the primary and supplementary motor cortices. SPM analysis showed metabolic changes which were significantly different after DBS therapy in all ROIs ipsilateral to DBS compared to those in the contralateral side. CONCLUSION: Raprl-DBS decreases the metabolic activity of areas anatomically related to its fiber composition. Improvement of symptoms may result from a decrease in pathological overactivity of circuits related to the ROIs.