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1.
Epilepsy Behav ; 160: 110039, 2024 Oct 11.
Artigo em Inglês | MEDLINE | ID: mdl-39395295

RESUMO

BACKGROUND: Utilizing interictal manifestations for the diagnosis of epilepsy is challenging. We investigated whether an interictal "head-turning sign," typically indicative of dependence on others observed in Alzheimer's disease, can act as a behavioral marker of idiopathic generalized epilepsy. METHODS: This multicenter study examined 579 consecutive patients, with a mean age of 36.8 ± 20.4 years, who did not have an intellectual disability and had their first outpatient visit for epilepsy evaluation between 2019 and 2023. Patients were categorized into IGE, non-IGE epilepsy, non-epileptic, and psychiatric conditions based on their ultimate diagnostic outcomes to identify difference of the occurrence of the head-turning sign among them. Additionally, we extracted data from patients under the age of 40, specifically adolescents and young adults (AYA). Then we used propensity score matching to confirm the reproducibility of observed differences and to identify associated factors within the AYA age group. RESULTS: The occurrence of the head-turning sign was significantly more prevalent in the IGE group compared to the non-IGE group (20.4 % vs. 2.2 %; P<0.0001) and non-epileptic group (20.4 % vs. 8.3 %; P=0.033). Following the matching, the head-turning sign was still evident in IGE relative to non-IGE patients (14.6 % vs. 4.5; P=0.004), yielding a 94 % specificity for IGE. IGE diagnosis (P<0.0001), myoclonic seizure (P<0.0001), being visited by a parent (P=0.017), and comorbidity with headache (P=0.021) were significantly associated with the head-turning sign. Multivariate analysis revealed that IGE (odds ratio: OR=2.80, P=0.028), attending with a parent (OR=2.92, P=0.029), and comorbidity with headache (OR=4.06, P=0.016) were independently associated with the head-turning sign. CONCLUSIONS: We confirmed a substantial association between the interictal "head-turning sign" and IGE. This unique sign may reflect a tendency towards dependence on others in IGE, and may serve as a promising diagnostic auxiliary marker for identifying IGE in the AYA age group.

2.
Sleep Breath ; 28(1): 393-399, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37422580

RESUMO

PURPOSE: Episodic nocturnal hypercapnia (eNH) in transcutaneous carbon dioxide pressure (PtcCO2) corresponding to rapid eye movement sleep hypoventilation is a useful biomarker for detecting nocturnal hypoventilation. However, the relationship between eNH and neurodegenerative diseases with sleep-related breathing disorders (SRBDs) is unknown. The aim of this study was to evaluate the relationship between eNH and nocturnal hypoventilation in neurodegenerative diseases. METHODS: Patients with neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), multiple system atrophy (MSA), Parkinson's disease, progressive supranuclear palsy, corticobasal syndrome, and idiopathic normal pressure hydrocephalus, were enrolled and received overnight PtcCO2 monitoring. The patients were divided into groups for eNH and sleep-associated hypoventilation (SH) prevalence analysis: A (ALS), B (MSA), and C (others). RESULTS: Among 110 patients, twenty-three (21%) and 10 (9%) of the patients met eNH and SH criteria, respectively. eNH and SH were significantly more frequent in groups A and B than in C. The prevalence of SH in the patients with eNH was 39% whereas most of patients with SH (90%) presented with eNH. Among patients with daytime carbon dioxide pressure in arterial blood ≤ 45 mmHg, eNH frequency was 13%, whereas none of the patients met SH criteria. The frequency of noninvasive positive pressure ventilation after PtcCO2 monitoring was significantly higher in those with than without eNH. CONCLUSIONS: eNH is common in patients with MSA and ALS who present with SRBD. eNH with overnight PtcCO2 monitoring is a useful biomarker to detect hypoventilation among neurodegenerative diseases with different SRBD mechanisms.


Assuntos
Esclerose Lateral Amiotrófica , Doenças Neurodegenerativas , Humanos , Hipercapnia/diagnóstico , Hipercapnia/epidemiologia , Hipoventilação/diagnóstico , Dióxido de Carbono , Esclerose Lateral Amiotrófica/diagnóstico , Esclerose Lateral Amiotrófica/epidemiologia , Doenças Neurodegenerativas/diagnóstico , Doenças Neurodegenerativas/epidemiologia , Biomarcadores
3.
Cureus ; 16(3): e56696, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38646321

RESUMO

A 79-year-old woman visited our department for chronic visual field abnormalities with a floating sensation for two months. Neurological and ophthalmologic examinations yielded normal results, except for brain MRI indicating left hippocampal atrophy. Cognitive function tests were normal. EEG revealed frequent spikes and slow waves in the left frontotemporal region, corroborated by reduced accumulation in 123I-iomazenil single photon emission computed tomography. A diagnosis of temporal lobe epilepsy was established, and treatment with lacosamide resulted in a remarkable improvement in symptoms and EEG findings. Mild focal seizures from the temporal region might cause mild impaired awareness, resulting in the patient's report as a sensation of uncertainty between the self and the outside world, mimicking ophthalmologic abnormalities. The repeated nature of the seizures contributed to the absence of the term "transient" in symptom description. Diagnosing epilepsy in the elderly proves challenging due to nonspecific complaints.

4.
Cureus ; 16(2): e53811, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38465051

RESUMO

A man in his 70s with alcoholic dementia was admitted for acute, prolonged impaired consciousness. Blood and cerebrospinal fluid findings were unremarkable. Brain MRI revealed multiple high-signal cortical regions. Following diazepam and levetiracetam administration, electroencephalography (EEG) revealed <1 Hz lateralized periodic discharges, indicating that the seizures were ceasing. The periodic discharges had disappeared during the gradual recovery process by day 10; however, cortical arterial spin labeling findings persisted only in regions exhibiting cytotoxic edema. Without additional anti-seizure medication, no seizure recurred, but cognitive dysfunction remained. He was transferred to a rehabilitation hospital with the continued oral administration of levetiracetam at 1,000 mg/day. DWI-ADC (diffusion-weighted imaging-apparent diffusion coefficient) match may suggest an indication of a missed suitable treatment window for seizures.

5.
Cureus ; 15(11): e49552, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-38156189

RESUMO

Differentiating between syncope and epileptic seizures can be challenging when a specific medical history is not available. We herein report a 70s man who exhibited recurrent, brief unresponsiveness while at rest on five occasions over a year. While there were no convulsions, the patient consistently reported urinary incontinence. These events were preceded by an epigastric rising sensation without chest symptoms, suggesting a possible diagnosis of temporal lobe epilepsy, and subsequent EEG revealed temporal semi-rhythmic delta activity. In contrast, the ECG revealed a left bundle branch block, while the initial Holter ECG showed no abnormalities. However, subsequent follow-up examinations revealed a complete atrioventricular block necessitating permanent pacemaker implantation. It is important to exercise caution in the interpretation of EEG findings. Moreover, instances of 'urinary incontinence without convulsion' may indicate non-epileptic events.

6.
Rinsho Shinkeigaku ; 63(6): 375-378, 2023 Jun 28.
Artigo em Japonês | MEDLINE | ID: mdl-37197972

RESUMO

An 87-year-old woman was admitted with acute onset of disturbed consciousness. On neurological examination, both pupils were dilated and non-reactive to light. Decerebrate rigidity was present. Babinski testing was positive. CTA suggested an isolated left P1 segment occlusion. The P2 segment was supplied from the left internal carotid artery via the posterior communicating artery. MRI showed bilateral paramedian thalamic infarctions. Because occlusion of the artery of Percheron was suspected, intravenous thrombolysis was performed. Digital subtraction angiography (DSA) revealed occlusion of the left P1 segment and spontaneous recanalization before endovascular treatment. Her consciousness improved immediately. When acute bilateral thalamic infarction suggests top of the basilar artery syndrome but no basilar artery occlusion is found, occlusion of the artery of Percheron should be considered. Thrombectomy of the affected P1 segment may be needed.


Assuntos
Arteriopatias Oclusivas , Insuficiência Vertebrobasilar , Humanos , Feminino , Idoso de 80 Anos ou mais , Infarto Cerebral/diagnóstico por imagem , Infarto Cerebral/etiologia , Imageamento por Ressonância Magnética , Artéria Basilar , Trombectomia
7.
Rinsho Shinkeigaku ; 62(12): 915-921, 2022 Dec 17.
Artigo em Japonês | MEDLINE | ID: mdl-36450489

RESUMO

Associated factors of the Myasthenia Gravis Activities of Daily Living (MG-ADL) score were investigated in 55 patients who had had generalized MG for more than 5 years. In multivariate analysis, correlates of the MG-ADL score at the last follow-up were the total number of fast-acting treatments (FTs) (standardized regression coefficient 0.617,P < 0.001) and Myasthenia Gravis Foundation of America (MGFA) classification (standardized regression coefficient 0.227,P = 0.032) (F = 32.7,P < 0.001). In patients with a score of 5 or more on MG-ADL at the last follow-up, tendency as follows were seen: 1) early-onset (P = 0.002), 2) longer duration (P = 0.014), 3) high frequency of MGFA classification V (P = 0.017), 4) high frequency of the total number of FTs (P < 0.001), and 5) higher dose of prednisolone at the last follow-up (P = 0.003). MGFA V, early-onset without depending on E-L-T classification, or difficulty of reduction for high doses of prednisolone can be the target of novel treatment for MG, and future prospective study will be expected.


Assuntos
Atividades Cotidianas , Miastenia Gravis , Humanos , Estudos Prospectivos , Miastenia Gravis/diagnóstico , Miastenia Gravis/tratamento farmacológico , Prednisolona , Pacientes
8.
Intern Med ; 61(24): 3733-3738, 2022 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-35598995

RESUMO

Central nervous system methotrexate-associated lymphoproliferative disorder (CNS-MTX-LPD) is rare, but its spontaneous regression has been observed in some patients after withdrawal of agents. We herein report three cases of primary CNS-MTX-LPD that received oral MTX for rheumatoid arthritis. Epstein-Barr virus and oligoclonal bands (OCBs) were positive, while proton magnetic resonance spectroscopy (1H-MRS) showed an elevated lipid peak and slightly elevated choline/N-acetylaspartate ratio in common. After MTX withdrawal, brain lesions showed spontaneous regression in all cases. Our patient's 1H-MRS findings and OCBs may reflect a non-monoclonal lymphoproliferative histology as benign-type lesions in CNS-MTX-LPD.


Assuntos
Antirreumáticos , Infecções por Vírus Epstein-Barr , Transtornos Linfoproliferativos , Humanos , Metotrexato/efeitos adversos , Bandas Oligoclonais/uso terapêutico , Antirreumáticos/uso terapêutico , Infecções por Vírus Epstein-Barr/patologia , Herpesvirus Humano 4 , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/diagnóstico por imagem , Transtornos Linfoproliferativos/tratamento farmacológico , Prognóstico , Sistema Nervoso Central/patologia , Espectroscopia de Ressonância Magnética
9.
Intern Med ; 61(9): 1387-1392, 2022 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-34670888

RESUMO

A 36-year-old man experienced severely impaired consciousness twice after drinking because of hyperammonemia. No abnormal blood tests were found other than ammonia levels. However, magnetic resonance imaging (MRI) showed atrophy of the brain parenchyma. One the second occasion, the patient suffered severe impairment of consciousness, and because of seizures and glossoptosis, mechanical ventilation was started. Urea cycle disorders (UCDs) were assumed to be involved. Genetic testing revealed a monoallelic mutation of the carbamoyl phosphate synthase 1 (CPS1) gene. When transient hyperammonemia of unknown cause occurs repeatedly in adults, an active investigation for UCDs should be conducted.


Assuntos
Doença da Deficiência da Carbamoil-Fosfato Sintase I , Hiperamonemia , Distúrbios Congênitos do Ciclo da Ureia , Adulto , Carbamoil-Fosfato Sintase (Amônia)/genética , Doença da Deficiência da Carbamoil-Fosfato Sintase I/complicações , Doença da Deficiência da Carbamoil-Fosfato Sintase I/genética , Carbamoil-Fosfato , Estado de Consciência , Humanos , Hiperamonemia/complicações , Hiperamonemia/diagnóstico , Hiperamonemia/genética , Masculino , Mutação/genética , Distúrbios Congênitos do Ciclo da Ureia/complicações
10.
Rinsho Shinkeigaku ; 61(10): 658-662, 2021 Oct 28.
Artigo em Japonês | MEDLINE | ID: mdl-34565749

RESUMO

A 76-year-old man with renal cell carcinoma exhibited consciousness disturbance and high fever after two cycles of combination therapy with ipilimumab and nivolumab. His cerebrospinal fluid (CSF) showed a protein concentration of 385 mg/dl, a cell count of 147/mm3, an interleukin-6 concentration of 1,280 pg/ml, and an adenosine deaminase concentration of 24.8 U/l. Contrast-enhanced FLAIR images were notable for diffuse meningeal enhancement. He was diagnosed with meningoencephalitis caused by an immune-related adverse event from immune checkpoint inhibitors (ICIs). His symptoms improved after repeated intravenous methylprednisolone pulse therapy and oral prednisolone. The meningeal enhancement disappeared, and the CSF findings became almost normal. As consciousness levels improved, we observed quadriplegia and peripheral neuropathy with antiganglioside antibodies, which led to a diagnosis of polyradiculoneuropathy. This is a rare case of a patient with overlapping meningoencephalitis and polyradiculo-neuropathy induced by ICIs.


Assuntos
Ipilimumab/efeitos adversos , Neoplasias Renais , Meningoencefalite , Nivolumabe/efeitos adversos , Polirradiculoneuropatia , Idoso , Humanos , Masculino , Meningoencefalite/induzido quimicamente , Meningoencefalite/tratamento farmacológico , Polirradiculoneuropatia/induzido quimicamente
11.
J Neurol Sci ; 430: 120024, 2021 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-34627053

RESUMO

AIMS: The mainstay of treatment for idiopathic normal-pressure hydrocephalus (iNPH) is spinal fluid shunting. A tap test (TT) is recommended as an indication of shunting. Patients with iNPH are often elderly and have multiple comorbidities affecting the shunting outcome. We investigated the factors affecting TT in patients with iNPH. METHODS: Seventy-five patients with iNPH were admitted to our department for a TT from April 2010 to May 2021. The patients were divided into a responsive group and an unresponsive group according to the clinical outcomes after TT on the Timed Up and Go Test (TUG), Mini-Mental State Examination (MMSE), or iNPH grading scale. Factors affecting the TT were compared between the responders and nonresponders. RESULTS: There were 38 patients (50.7%) in the TT responder group, and the prevalence of improvement was 82.9% in the TUG, 27.6% in the MMSE, and 76.3% in the iNPH grading scale. There were no significant differences in the vascular risk factors between the two groups. The prevalence of lumbar spondylosis, compression fracture, severe periventricular hyperintensity, deep and subcortical white matter hyperintensity (DSWMH), and old cerebral infarcts was significantly higher among the TT nonresponders. The logistic regression analysis showed that severe DSWMH and lumbar spondylosis were associated with a TT nonresponse (p < 0.001 and p = 0.003, respectively). Shunting was performed in 22 patients, 19 of whom were TT responders. CONCLUSION: Severe DSWMH and lumbar spondylosis were associated with a poor response to the TT in iNPH patients. We should consider risk factors when selecting candidates for shunt surgery.


Assuntos
Hidrocefalia de Pressão Normal , Equilíbrio Postural , Idoso , Derivações do Líquido Cefalorraquidiano , Humanos , Hidrocefalia de Pressão Normal/epidemiologia , Hidrocefalia de Pressão Normal/cirurgia , Estudos de Tempo e Movimento
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