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1.
BMC Neurol ; 19(1): 301, 2019 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-31775676

RESUMO

BACKGROUND: McLeod syndrome is a rare X-linked recessive acanthocytosis associated with neurological manifestations including progressive chorea, cognitive impairment, psychiatric disturbances, seizures, and sensorimotor axonal polyneuropathy. However, no studies have investigated the functioning of central sensorimotor tracts in patients with McLeod syndrome. CASE PRESENTATION: A 66-year-old man had experienced slowly progressive chorea and gait disturbance due to lower limb muscle weakness since his early fifties. Blood examinations showed erythrocyte acanthocytosis and the reduction of Kell antigens in red blood cells. Brain magnetic resonance imaging showed atrophy of the bilateral caudate nuclei and putamen. The diagnosis of McLeod syndrome was confirmed by the presence of a mutation of the XK gene on the X chromosome. Somatosensory-evoked potential and transcranial magnetic stimulation studies demonstrated that the central sensory and motor conduction times were abnormally prolonged for the lower extremity but normal for the upper extremity. CONCLUSIONS: This is the first report of the involvement of the central sensorimotor tracts for the legs in a patient with McLeod syndrome. The clinical neurophysiological technique revealed the central sensorimotor tracts involvements clinically masked by neuropathy.


Assuntos
Neuroacantocitose/diagnóstico , Idoso , Atrofia , Humanos , Imageamento por Ressonância Magnética , Masculino , Transtornos dos Movimentos/etiologia , Debilidade Muscular/etiologia , Mutação
2.
Intern Med ; 61(13): 2061-2065, 2022 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-34924463

RESUMO

A 59-year-old man with advanced Parkinson's disease treated using levodopa-carbidopa intestinal gel (LCIG) presented with leg edema, hypoalbuminemia, and proteinuria at 1 year after the treatment. He subsequently developed a generalized tonic-clonic seizure, and brain magnetic resonance imaging indicated vasogenic edema in the white matter of the left frontal subcortex. He was diagnosed with nephrotic syndrome (NS) and atypical posterior reversible encephalopathy syndrome (PRES). LCIG cessation and corticosteroid treatment improved the NS. To our knowledge, this is the first case report of NS and atypical PRES in patients with Parkinson's disease. Patients being treated with LCIG should be closely monitored for NS.


Assuntos
Síndrome Nefrótica , Doença de Parkinson , Síndrome da Leucoencefalopatia Posterior , Antiparkinsonianos/efeitos adversos , Encéfalo/diagnóstico por imagem , Combinação de Medicamentos , Géis , Humanos , Levodopa , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/induzido quimicamente , Síndrome Nefrótica/complicações , Síndrome Nefrótica/tratamento farmacológico , Doença de Parkinson/complicações , Doença de Parkinson/tratamento farmacológico , Síndrome da Leucoencefalopatia Posterior/complicações , Síndrome da Leucoencefalopatia Posterior/diagnóstico por imagem
3.
Medicine (Baltimore) ; 100(24): e26315, 2021 Jun 18.
Artigo em Inglês | MEDLINE | ID: mdl-34128872

RESUMO

RATIONALE: Aseptic meningoencephalitis is a rare central nervous system complication of relapsing polychondritis (RP). PATIENT: We report a 61-year-old Japanese male patient with spiking fever and impaired consciousness. Neurological examination revealed meningealirritation, and cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with elevated protein (199 mg/dL) and interleukin-6 (3810 pg/mL). Serological analysis showed high levels of anti-type II collagen antibodies, and the result of auricular biopsy was consistent with the diagnosis of RP showing cartilage degeneration surrounded by inflammatory cell infiltrations. DIAGNOSIS: A clinical diagnosis of RP was made according to the diagnostic criteria established by MacAdams et al. INTERVENTION: Steroid pulse therapy (methylprednisolone 1000 mg, consecutive 3 days) followed by oral prednisolone (60 mg/day) resolved the patient's high fever and disturbance of consciousness. OUTCOMES: The patient rapidly improved after steroid treatments and has a normal quality of life under the maintenance dose of steroid plus methotrexate (4 mg/week). LESSONS: RP-associated meningoencephalitis is a rare complication with significant morbidity and mortality. It should be considered and differentiated in patients with RP with unexplained spiking fever and impaired consciousness. In addition, the assessment of cerebrospinal fluid interleukin-6 levels may be useful to investigate the disease activity of RP-related meningoencephalitis. Further prospective studies are required to confirm this result.


Assuntos
Meningoencefalite/etiologia , Policondrite Recidivante/complicações , Glucocorticoides/administração & dosagem , Humanos , Interleucina-6/líquido cefalorraquidiano , Leucocitose/líquido cefalorraquidiano , Leucocitose/complicações , Masculino , Meningoencefalite/líquido cefalorraquidiano , Meningoencefalite/terapia , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Policondrite Recidivante/líquido cefalorraquidiano , Policondrite Recidivante/terapia
4.
Clin Neurophysiol ; 131(8): 1896-1901, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32593964

RESUMO

OBJECTIVE: We retrospectively investigated the utility of the central motor conduction time (CMCT) in detecting upper motor neuron (UMN) involvements in patients with amyotrophic lateral sclerosis (ALS). METHODS: Fifty-two ALS patients and 12 disease control patients participated in this study. Surface electromyograms were recorded from the first dorsal interosseous (FDI) and tibialis anterior (TA) muscles. We stimulated the motor cortex, brainstem, and spinal nerve using transcranial magnetic stimulation (TMS) in order to measure the cortical, brainstem, and spinal latencies. We divided the ALS patients into 2 subgroups (with UMN impairment vs. without UMN impairment) and calculated the rates of abnormal CMCT prolongation judged by their comparison with the normal ranges obtained by the measurement in the control patients. RESULTS: The CMCTs in the FDI and TA were abnormally prolonged in over 40% of the ALS patients with UMN impairment and in nearly 30% of those without UMN impairment. CONCLUSIONS: CMCT shows UMN dysfunction in ALS patients without clinical UMN impairment. SIGNIFICANCE: TMS still has diagnostic utility in a significant portion of ALS patients.


Assuntos
Esclerose Lateral Amiotrófica/fisiopatologia , Neurônios Motores/fisiologia , Condução Nervosa , Idoso , Tronco Encefálico/fisiopatologia , Potencial Evocado Motor , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Córtex Motor/fisiopatologia , Músculo Esquelético/fisiopatologia , Tempo de Reação , Nervos Espinhais/fisiopatologia , Estimulação Magnética Transcraniana
5.
eNeurologicalSci ; 18: 100229, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32090177

RESUMO

Autoimmune GFAP astrocytopathy is a new clinical entity and only a limited number of cases have been reported. Here we report the results of multimodal central conduction studies performed in a case of this disorder. A 72-year-old woman developed gradual cognitive decline and gait disturbance. A neurological examination revealed moderate amnesia, papilloedema, and pyramidal tract impairment of the bilateral lower limbs. The diagnosis of autoimmune GFAP astrocytopathy was made based on the typical MRI findings of periventricular radial linear gadolinium enhancement in the brain and longitudinally extensive lesions in the spinal cord, and anti-GFAP antibody detected in the cerebrospinal fluid. Somatosensory evoked potentials and transcranial magnetic stimulation studies revealed prolongation of conduction times. Visual evoked potentials showed an unusual W-shaped pattern. To our knowledge, this is the first neurophysiological demonstration of prolonged central conduction times in the autoimmune GFAP astrocytopathy. Further investigations are needed to establish the clinical value the neurophysiological examinations in this disorder.

6.
Rinsho Shinkeigaku ; 59(3): 133-138, 2019 Mar 28.
Artigo em Japonês | MEDLINE | ID: mdl-30814444

RESUMO

A 68-year-old man visited our hospital emergency department with consciousness disturbance. He was diagnosed as bacterial meningitis with septic shock, and initial empirical antibacterial therapy was initiated immediately. Streptococcus pneumoniae. was cultured from the cerebrospinal fluid (CSF), and brain MRIs showed pyogenic ventriculitis. Even though CSF findings improved, he was still in coma and finally died with pneumonia. It is unknown how pyogenic ventriculitis affects the course of bacterial meningitis. We analyzed total 11 inpatients with bacterial meningitis associated with or without the pyogenic ventriculitis, including the present patient, in our hospital. Severity of clinical symptoms and CSF findings might determine the duration of antimicrobial administration, regardless of whether pyogenic ventriculitis existed or not.


Assuntos
Antibacterianos/administração & dosagem , Ventriculite Cerebral/complicações , Meningite Pneumocócica/complicações , Meningite Pneumocócica/tratamento farmacológico , Idoso , Encéfalo/diagnóstico por imagem , Ventriculite Cerebral/diagnóstico por imagem , Líquido Cefalorraquidiano/microbiologia , Quimioterapia Combinada , Evolução Fatal , Humanos , Imageamento por Ressonância Magnética , Masculino , Meningite Pneumocócica/diagnóstico , Meningite Pneumocócica/microbiologia , Índice de Gravidade de Doença , Streptococcus pneumoniae/isolamento & purificação , Supuração
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