Revisiting Clinical Utility of Chest Radiography and Electrocardiogram to Determine Ischemic Stroke Subtypes: Special Reference on Vascular Pedicle Width and Maximal P-Wave Duration.

BACKGROUNDS: It is often difficult to diagnose stroke subtypes at admission, particularly in sinus rhythm cases. Vascular pedicle width (VPW) on chest X-ray (CXR) and maximal P-wave duration (P-max) on electrocardiogram (ECG) are again realized as useful parameters reflecting intravascular volume and atrial conduction status, respectively. We investigated the utility of VPW and P-max as a tool for differentiating ischemic stroke subtypes. METHODS: We studied 343 acute stroke patients showing sinus rhythm on admission. Dividing the patients into cardioembolic (CE) stroke (n = 57) and non-CE (n = 286) groups, we compared clinical backgrounds including VPW on CXR, and P-max in lead II and premature atrial contraction (PAC) on 12-leads ECG. Then, we investigated the independent factors for CE. RESULTS: Independent factors associated with CE were VPW (≥59.3 mm) (p < 0.001; odds ratio (OR), 10.12; 95% confidence interval (CI), 4.13-24.8), P-max in lead II (≥120 ms) (p < 0.001; OR, 8.61; 95% CI, 3.96-18.7), PAC (p = 0.002; OR, 7.35; 95% CI, 2.14-25.3) and D-dimer level (≥1.11 µg/ml) (p = 0.016; OR, 2.57; 95% CI, 1.20-5.51). CONCLUSIONS: VPW, P-max, PAC and D-dimer are useful parameters for diagnosing CE stroke in patients with sinus rhythm at admission.

Self-rated anosognosia score may be a sensitive and predictive indicator for progressive brain atrophy in amyotrophic lateral sclerosis: an X-ray computed tomographic study.

We investigated whether a self-rated anosognosia score can be an indicator for progression of brain atrophy in patients with amyotrophic lateral sclerosis (ALS). Scores for 16 patients were compared with the ventricular areas of the bilateral anterior and inferior horns measured on x-ray computed tomography. Longitudinal enlargement was expressed as a monthly increase in size: (ventricular size at the initial scan - ventricular size at the follow-up scan)/scan interval (months). The anosognosia scores ranged from -4 to 3 and 3-18 in patients with and without frontotemporal lobar degeneration (FTLD), respectively (p = 0.0011). Anosognosia scores were significantly correlated with sizes of anterior (r = 0.704, p = 0.0016) and inferior (r = 0.898, p < 0.0001) horns. In non-demented patients for whom follow-up CT scans were available (n = 7), the scores were significantly correlated with the longitudinal increase in inferior horn size (r = 0.754, p = 0.0496), but not with that of anterior horn size (r = -0.166, p = 0.7111). In conclusion, anosognosia in ALS is associated with greater anterior and inferior horn sizes, reflecting frontotemporal lobar atrophy. Moreover, mild anosognosia in ALS patients without FTLD may predict impending inferior horn enlargement, reflecting medial temporal atrophy.

Effects of beta-hydroxy beta-methyl butyrate calcium combined with exercise therapy in patients with cardiac disease: a study protocol for clinical trial.

INTRODUCTION: The current treatment for heart disease consists of exercise therapy in addition to pharmacotherapy, nutritional support and lifestyle guidance. In general, nutritional support focuses on protein, salt and energy restrictions, with no active protein or amino acid intake in cases involving moderate or higher renal failure. From this perspective, patients with cardiac disease are at high risk of frailty.Beta-hydroxy beta-methyl butyrate (HMB) is a metabolite of leucine. HMB is widely used for muscle strengthening and can be safely ingested even by patients with renal failure. The proposed study protocol will investigate the effects of HMB-calcium (HMB-Ca) administered in combination with comprehensive cardiac rehabilitation for muscle strength, muscle mass and cardiac function in patients with cardiac disease during the convalescent period. The primary outcome will be knee extensor strength. Secondary outcomes will be gross isometric limb strength and skeletal muscle mass. METHODS AND ANALYSIS: This study will be a single-blinded, randomised, controlled trial with parallel comparisons between two groups. The study period will be 60 days from the start of outpatient cardiac rehabilitation. Participants will be randomly divided into two groups: an HMB group consuming HMB-Ca one time per day for 60 days; and a Placebo group consuming reduced maltose once one time per day for 60 days. Exercise therapy will be performed by both groups. ETHICS AND DISSEMINATION: The study protocol will be published in a peer-reviewed journal. Ethics approval was provided by the Showa University Clinical Research Review Board. TRIAL REGISTRATION NUMBER: jRCTs031220139; Japan Registry of Clinical Trails.

The brainstem is at high risk for recurrent noncardioembolic cerebral infarction in association with diabetes mellitus: a hospital-based study.

The goals of the study were to investigate the importance of brainstem infarction (BSI) in recurrent noncardioembolic ischemic stroke and to examine the relevant clinical background. Data were retrospectively reviewed for 655 consecutive patients with acute noncardioembolic infarction who were admitted to our hospital from January 2004 to August 2010. The patients were divided into first-stroke (n = 592) and recurrent-stroke (n = 63) groups. Acute infarcted lesions were explored on MRI, and clinical background factors including age, sex, smoking, atrial fibrillation, coronary heart disease, hypertension, hyperlipidemia and diabetes mellitus (DM) were assessed. The frequency of BSI in the recurrent-stroke group was significantly higher than that in first-stroke patients (30.2 vs. 14.9%, p = 0.0033). No other clinical background factors differed between the two groups. Only the frequency of DM differed significantly among four subgroups formed based on stroke recurrence and BSI (p < 0.0001): DM was present in 63.2% of recurrent-stroke patients with BSI, 54.5% of first-stroke patients with BSI, 27.4% of first-stroke patients without BSI, and 20.5% of recurrent-stroke patients without BSI. We conclude that the brainstem is at high risk for recurrent cerebral infarction in patients with DM.

Occurrence and clinicotopographical correlates of brainstem infarction in patients with diabetes mellitus.

BACKGROUND: The goal of the study was to clarify the association between diabetes mellitus (DM) and brainstem infarctions (BSIs) and to investigate the clinicotopographic characteristics of BSIs in patients with diabetes. METHODS: Data were retrospectively reviewed for 1026 consecutive patients admitted to our hospital because of acute cerebral infarctions from January 2004 to August 2010. Acute symptomatic BSIs were explored on radiologic images and classified into multiple infarctions with BSIs, multifocal BSIs, and monofocal BSIs. Isolated BSIs were further classified based on the vertical distribution into midbrain, pontine, and medullary infarctions, and on the horizontal distribution into anterior-dominant, posterior-dominant, and anterior/posterior BSIs. Neurologic symptoms of BSIs and clinical background were compared between DM and non-DM patients. RESULTS: The prevalence of BSIs was 2.6-fold higher (P < .0001) in DM patients. Logistic regression analysis including age, sex, smoking, previous stroke, atrial fibrillation, other cardiac diseases, hypertension, hyperlipidemia, and DM showed that DM was independently associated with BSIs (odds ratio [OR] 2.814; 95% confidence interval [CI] 1.936-4.090; P < .0001). Compared with non-DM patients, DM patients showed more frequent monofocal BSIs (P < .0001) and multifocal BSIs (P = .0296). Monofocal BSIs (n = 114) more frequently involved the pons (P < .0001) and medulla (P = .0212). Anterior-dominant BSIs (P < .0001) were more common in DM patients than in non-DM patients. Symptoms of BSIs included more frequent motor paresis (P = .0180) and less frequent diplopia (P = .0298) in DM patients than in non-DM patients. CONCLUSIONS: DM is important in the development of BSIs, and the associated clinical characteristics include more frequent motor paresis and less frequent diplopia.

Deep white matter hyperintensities, decreased serum low-density lipoprotein, and dilative large arteriopathy.

Deep white matter hyperintensities (DWMHs) seen on magnetic resonance imaging (MRI) are thought to reflect small-vessel diseases (SVDs) and may have a background that differs from that of stenotic large-vessel diseases. We assessed risk factors for DWMHs and investigated the association between DWMHs and dilative changes in the basilar artery (BA) on MRI in nonstroke patients. We reviewed clinical information and MRI findings for 149 outpatients aged 46-90 years, excluding those with a previous symptomatic cerebrovascular event. DWMHs were graded 0-3, and the maximal BA diameter and area were measured from the flow void on axial T2-weighted MRI to assess dilatation. We divided the patients into groups with and without DWMH grade 2 or 3, and compared clinical information and BA parameters in these groups. The two groups demonstrated significant differences in age, serum low-density lipoprotein (LDL) level, estimated glomerular filtration rate (eGFR), and BA parameters. An adjusted logistic regression analysis including BA diameter found that age (odds ratio [OR], 1.974 per 10 years; 95% confidence interval [CI], 1.030-1.112; P = .0006), LDL (OR, 0.811 per 10 mg/dL; 95% CI, 0.964-0.965; P = .0085), eGFR (OR, 0.835 per 10 mL/min/1.73 m(2); 95% CI, 0.967-0.998; P = .0229), and BA diameter (OR, 2.515 per 1 mm; 95% CI, 1.191-4.098; P = .0119) were independently associated with the presence of DWMHs. An analysis including the BA area yielded similar results. DWMHs are manifestations of SVDs and show a strong association with lower serum LDL level, lower eGFR, and BA dilatation.

Amyotrophic lateral sclerosis and language dysfunction: kana, kanji and a prescient report in Japanese by Watanabe (1893).

Although Charcot described amyotrophic lateral sclerosis (ALS) in the 1870s, he did not focus on language. And language problems in ALS with or without dementia were long ignored. A recent report by Caselli et al. [Ann Neurol 1993;33:200-207] is accurately regarded as a major breakthrough in studies of language in ALS. However, we discovered a Japanese account written by Watanabe in 1893 describing paragraphia of an aphasic nature, and this is interesting for two reasons. (1) Watanabe's paper is, we believe, the first report of an aphasia associated with motor neuron disease, and predates other reports by 100 years. (2) It sheds light on the dissociated involvement of the two Japanese writing systems: kana (Japanese simple phonograms) and kanji (Japanese morphograms with complex character derived from Chinese characters). In the aphasia reported by Watanabe, the phonograms are more affected than the morphograms. Thus, Watanabe's clinical observation may predict current theories of the way in which these two writing systems involve different intrahemispheric pathways.

Writing error may be a predictive sign for impending brain atrophy progression in amyotrophic lateral sclerosis: a preliminary study using X-ray computed tomography.

AIM: To investigate whether writing errors are predictive of longitudinal brain atrophy progression in patients with amyotrophic lateral sclerosis (ALS). METHODS: The frequency of writing errors in 6 ALS patients without dementia was compared with longitudinal changes in lateral ventricular areas of the bilateral anterior and inferior horns on X-ray computed tomography scans. The increase in area per month for the anterior and inferior horns was used as a measure of longitudinal brain atrophy progression, and was calculated as: (area on the initial scan - area on the follow-up scan)/scan interval (month). RESULTS: The longitudinal rate of increase in the area of the anterior horns showed significant associations with the rates of total writing errors (r = 0.886, p = 0.0152), kana errors (r = 0.887, p = 0.0148) and kana omission (r = 0.856, p = 0.0268), whereas that for the inferior horns size showed no significant association with any writing errors. CONCLUSION: The increased area of the anterior horns indicates frontal-lobar atrophy, and writing errors may be a predictive sign for impending brain atrophy progression in the frontal lobes, which reflects the development of anterior-type dementia.

Cerebral microbleeds and dilative remodeling of the basilar artery: a magnetic resonance imaging study.

This study was conducted to examine the relationship between cerebral microbleeds (CMBs), one of the manifestations of small-vessel diseases (SVDs), and basilar artery (BA) dilatation on magnetic resonance imaging (MRI). Clinical information and MRI images were reviewed for 149 outpatients aged 46-90 years, excluding those who had a previous symptomatic cerebrovascular event. CMBs were evaluated on T2∗-weighted MRI, and BA diameters were measured as the maximal width of the flow void on axial T2-weighted MRI to assess dilatation. Patients were divided into 2 groups, with CMBs and without CMBs, and clinical information and BA diameters were compared between the groups. Regression analyses of the data also were performed. The 2 groups had significant differences in mean blood pressure (MBP), low-density lipoprotein (LDL) and uricemic acid levels, and BA diameter. Adjusted logistic regression analysis showed that MBP (odds ratio [OR], 1.059 per 1 mm Hg; 95% confidence interval [CI], 1.019-1.101; P = .0035), LDL (OR, 0.976 per 1 mg/dL; 95% CI, 0.960-0.994; P = .0072), and BA diameter (OR, 3.266 per 1 mm; 95% CI, 1.504-7.103; P = .0028) each had an independent association with the presence of CMB. Adjusted multiple regression analysis showed that only BA diameter (ß coefficient, 0.240; 95% CI, 0.775-3.734; P = .0031) was independently associated with the number of CMBs. Our data indicate that CMB, a manifestation of SVD, shows a strong association with BA dilatation.

Hypertrophic pachymeningitis in an immunocompetent adult with positive Aspergillus DNA in the cerebrospinal fluid.

A 42-year-old immunocompetent man presented with subacute onset unilateral headache and associated lower cranial nerve palsies. Cranial magnetic resonance imaging showed enhancing thickened tentorium cerebelli and subtentorial dura mater. Cerebrospinal fluid examination revealed lymphocytic pleocytosis and positive polymerase chain reaction assay of Aspergillus DNA. While on voriconazole treatment a progressive increase was noted in subtentorial pachymeningeal hypertrophy, which was excised because of critical compression of the medulla. The excision material showed extensive fibrosis, cellular infiltrates and no organisms. With combination therapy with anti-fungal agents and corticosteroids, pachymeningitis showed regression. We hypothesised that intact immune status and less burden of Aspergillus infection in this patient may have resulted in a chronic progressive hypertrophic pachymeningitis.

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) for Japanese ALS and FTD patients.

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) patients might present with cognitive and behavioural abnormalities resembling frontotemporal dementia (FTD). The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was developed as an easy to administer cognitive screen for detecting these symptoms. The aim of the present study was to develop and validate a Japanese version of the ECAS. METHODS: In this single centre observational study, 35 ALS patients and 28 healthy controls were enrolled. Three patients in the ALS group fulfilled the criteria for behavioural variant FTD (ALS-FTD) and the rest were grouped as ALS without FTD. Participants were subjected to the Japanese version of the ECAS. ALS patients were also subjected to the Montreal Cognitive Assessment, Frontal Assessment Battery, ALS Functional Rating Scale-Revised, and respiratory function testing. Demographic and disease characteristics (e.g., sex, age at examination, and years of education) were also recorded. RESULTS: Internal consistency and correlations with general cognitive screenings were sufficient in the Japanese adaptation. Executive functions were the most commonly affected ECAS domain, followed by fluency and language. Compared to control subjects, ALS patients without FTD had low scores in the ECAS ALS-specific functions but not in ALS-nonspecific functions. Meanwhile ALS-FTD patients markedly underperformed both in the ECAS ALS-specific and ALS-nonspecific functions. Furthermore, the Japanese ECAS score correlated positively with years of education and negatively with age at onset. CONCLUSION: The Japanese version of the ECAS is a valid and useful screening tool to identify multiple types of cognitive impairment in ALS patients.

Effect of motor imagery and voluntary muscle contraction on the F wave.

We tested the validity of instructing patients to minimally contract the muscle to facilitate F-wave recording in clinical practice. In 12 healthy subjects, F waves were recorded from the first dorsal interosseous muscle at rest, during motor imagery, and at up to 30% of the maximal voluntary contraction (MVC). F-wave persistence increased significantly from 32.5 +/- 11.9% (mean +/- SD) at rest to 58.3 +/- 15.2% during motor imagery and 90.0 +/- 8.7% during 3% MVC. It then remained the same during stepwise changes to and from 30% MVC before decreasing significantly from 80.8 +/- 18.5% during 3% MVC to 48.7 +/- 23.8% during motor imagery and 27.0 +/- 16.0% at rest. The trial average of F-wave amplitude showed a similar pattern of facilitation. Motor imagery enhances F-wave persistence and amplitude, which further increase with a slight muscle contraction and show no additional change with a stronger effort.

Kana versus kanji in amyotrophic lateral sclerosis: a clinicoradiological study of writing errors.

The frequency of writing errors in samples from 14 patients with amyotrophic lateral sclerosis without manifest aphasia were compared with clinical background and indices from X-ray computed tomography, including the Evans' index (EI) and the cella media index (CMI). The inferior horn index (IHI) was measured as the maximal width of the short axis of the bilateral inferior horn of the lateral ventricles/the maximum transverse distance between the two internal laminae. Overt dementia and disinhibitive behavioral changes were significantly associated with frequency of total errors (p = 0.0280) and kanji errors (p = 0.0025). Significant associations were found for the EI with kana errors (p = 0.0481) and for the IHI with kanji errors (p = 0.0052). Preferential involvement of kana and kanji may reflect involvement of language-related areas in the frontotemporal lobes with frontal lobe or temporal lobe predominance.

Involvement of the basilar artery in diabetes mellitus: an MRI study of brainstem infarctions.

The relationships among diabetes mellitus (DM), brainstem infarctions (BSIs) and involvement of the basilar artery (BA) were investigated in 254 patients with acute cerebral infarctions detected on magnetic resonance (MR) imaging. Radiological findings included lesion topography and size (mm(2)) of BSIs on MR images, and the extent of BA stenosis measured by MR angiography. Adjusted logistic regression analyses showed that DM (OR 4.018; p = 0.0006) and BA stenosis (OR 1.003 per 1 mm; p < 0.0001) had an independent association with the incidence of BSIs, but the lesion size of the BSIs was only associated with BA stenosis (ß coefficient 0.280; p < 0.0001). Diabetic patients showed significantly more frequent isolated pontine infarctions and a lesser degree of BA stenosis (p < 0.005) compared to non-diabetic patients. Preferential involvement of the pons and smaller vessels may be characteristics of diabetic patients.

[Bulbar-onset amyotrophic lateral sclerosis (ALS) with isolated agraphia].

We reported the two cases with bulbar-onset ALS showing isolated agraphia without overt dementia and aphasia. Patient 1 was a 69-year-old man and patient 2 was an 81-year-old woman, and both were right-handed. Each patient developed dysarthria as an initial symptom followed by dysphagia, and neurological examinations showed atrophy and fasciculation of the tongue with upper and lower motor-neuron involvement of the extremities. These characteristic features with electromyographic evidence including widespread acute and chronic denervation led to a diagnosis of bulbar-onset ALS. Around 1 year after the onset of ALS, dysarthria was mild enough to allow oral communication enabling the determination that aphasia was absent with well preserved confrontation naming, repetition, reading and comprehension. The patients were polite without abnormal behavior or character change, and their general intelligence was also well preserved with excellent scores on the Mini Mental State Examination (30 and 27 points for patients 1 and 2, respectively) and Frontal Assessment Battery (16 points for each patient). However, spontaneous writing and dictation revealed abundant writing errors characterized by omission of kana letters and paragraphia of kana and kanji letters in both patients. Some syntactic errors were also observed in writing but in spoken language. A letter-number effect on writing errors was observed in patient 1. Copying of letters or words was intact and structure and orientation of written letters was well preserved, indicating the absence of constructional, apraxic or spatial feature of agraphia. Single photon emission computed tomography demonstrated reduced uptake in the bilateral frontotemporal lobes, predominantly in the left hemisphere, with less evident alternation in magnetic resonance imaging. Our results suggest that patients with bulbar-onset ALS may develop isolated agraphia as a single-domain cognitive impairment, preceding the clinical manifestation of aphasia or dementia. We speculate that the main responsible region might be the posterior part of the middle and inferior frontal gyri including Exner's writing center and Broca's area beyond the primary motor cortex.

[Intramedullary spinal cord metastasis presenting with acute onset longitudinally extensive spinal cord lesion].

A 34-year-old previously healthy man presented with acute transverse lumbar myelopathy and MRI evidence of a longitudinally extensive spinal cord lesion (LESCL) from the upper thoracic cord extending down to the conus medullaris. Gadolinium-DTPA enhancement revealed a clearly demarcated enhanced nodule confined to the level of the 11th thoracic vertebral body (T11), which might have caused longitudinally extensive edema in the spinal cord. Histopathological appearance of adenocarcinoma of the nodule led to the ultimate diagnosis of lung cancer. Intramedullary spinal cord metastasis in a young patient without previously-diagnosed malignancy is a rare disorder, but should be listed up as a cause of LESCL.

[Progressive cerebral infraction initially presenting with pseudo-ulnar nerve palsy in a patient with severe internal carotid artery stenosis].

A 63-year-old man with hypercholesterolemia developed sensory and motor disturbances in the ulnar side of the right hand, and over three days the weakness evolved to entire right arm. Examination on the 6th day after onset showed mild lower facial palsy in addition to the upper limb weakness on the right. The weakness involved entire right arm sparing shoulder girdle muscles, which was worse in the 4th and 5th digits with claw hand deformity of the hand. Magnetic resonance imaging showed multiple small infracts in the centrum semiovale as well as in the medial side of the precentral knob on the left. Magnetic resonance angiography, ultrasonography, and 3D-CT angiography of the neck showed severe stenosis associated with unstable plaque of the left internal carotid artery. Hemodynamic mechanisms including microemboli and hypoperfusion associated with severe internal carotid artery stenosis are likely to cause stroke in evolution after initial presentation of pseudo-ulnar palsy in the present case.

Cognitive and behavioral status in Japanese ALS patients: a multicenter study.

OBJECTIVES: Amyotrophic lateral sclerosis (ALS) patients may present with cognitive and behavioral abnormalities similar to frontotemporal dementia (FTD). In this multicenter study we examined Japanese ALS patients with and without FTD in order to characterize the full extent of cognitive and behavioral abnormalities, including associations with functional motor status, anxiety and depression. METHODS: Patients were evaluated using the Montreal Cognitive Assessment (MoCA), Frontal Assessment Battery (FAB), Hospital Anxiety and Depression Scale, ALS Functional Rating Scale-Revised, spirometry, and verbal fluency tests. Caregivers were asked to complete the ALS-FTD-Questionnaire (ALS-FTD-Q), a behavioral screen. We defined severe cognitive impairment (MoCA < 21 or FAB < 11), mild impairment (11 ≤ MoCA ≤ 25 or 11 ≤ FAB ≤ 15), and normal cognition (MoCA > 25 or FAB > 15). Severe and mild behavioral impairments and normal behavior were defined by the ALS-FTD-Q scores. RESULTS: In 145 ALS patients, better cognitive scores were correlated with earlier age at onset, whereas a worse behavioral score was associated with a longer disease duration and higher level of anxiety and depression. Around seventy percent of all ALS patients showed mild (40-45%) or severe cognitive impairment with cognitive impairment outnumbering behavioral impairment fivefold. Cognitive functions were more impaired in patients with age of onset over 65 years, while behavioral scores were not related to age. CONCLUSIONS: Considering the high prevalence of in particular cognitive impairment, and the diversity of impairments, the cognitive and behavioral aspects of Japanese ALS patients should be given more attention clinically.

Intracranial dilative arteriopathy is associated with chronic kidney disease and small vessel diseases in the elderly.

OBJECTIVE: We sought to determine the responsible factors and clinical significance of dilatation of the internal carotid artery (ICA) and basilar artery (BA). METHODS: ICA and BA diameters were measured using magnetic resonance imaging (MRI) and their association with age, sex, conventional atherosclerotic factors, and estimated glomerular filtration rate (eGFR) were evaluated in 175 outpatients aged 40 to 89 years. The arterial diameters were measured as the maximal width of the flow void on T2-weighted MRI around the brainstem. The relationship between the arterial diameters and deep white matter hyperintensities (DWMHs) on MRI graded from 0 to 3 was also examined. Comparisons were performed between groups with diameters smaller and larger than the mean value for younger (40-69 years) and older (70-89 years) patients, and multiple regression analysis was conducted. RESULTS: Age differed significantly between the larger- and smaller-diameter groups in younger patients, but not in older patients. The larger-diameter group of older patients had a significantly lower eGFR (P = .0002 for ICA, P = .0035 for BA) and a higher DWMH grade (P = .0021 for ICA, P < .0001 for BA) compared to the smaller-diameter group. In multiple regression analysis adjusted for age and sex, a lower eGFR was an independent factor associated with larger arterial diameters (P = .0002 for ICA, P = .0021 for BA). CONCLUSION: Kidney dysfunction is an independent factor that is related to ICA and BA dilatation, which is also associated with DWMHs that reflect small vessel diseases in older patients.

[A 25-year-old woman with primary Sjögren syndrome who developed optic neuritis and encephalomyelitis associated with an anti-aquaporin 4 antibody].

We report a 25-year-old woman who developed optic neuritis and encephalomyelitis following primary Sjögren's syndrome (SjS). SjS began with Sicca syndrome when she was 8 years old, and neurological involvement subsequently developed at the age of 10 with right hemiparesis. Based on clinical symptoms, serum positive for SS-A and SS-B antibodies and pathological findings of the salivary gland, we confirmed a diagnosis of primary SjS. Magnetic resonance imaging (MRI) revealed multiple lesions in the brain and the spinal cord. These led diagnosis of SjS with central nervous system involvement (CNS-SjS) and initiated steroid therapy. At the age of 25, the patient developed left visual loss due to retrobulbar optic neuritis, left lower quadrantic hemianopia, numbness of the right upper limb, and weakness of both legs. Laboratory examinations showed that her serum was positive for SS-A and SS-B antibodies, and her cerebrospinal fluid had elevated levels of total protein and myelin basic protein without pleocytosis. Her brain MRI revealed multiple T2-high-intensity lesions bilaterally in the frontal subcortical white matter and in the right temporo-parietal subcortical white matter. The lesions included a tumefactive lesion and an active lesion. Additionally, the spinal MRI revealed a severely atrophied spinal cord with T2-high-intensity lesions extending longitudinally and centromedullary in the spinal cord. These findings led us to examine the patient's serum for anti-aquaporin (AQP) 4 antibodies and the test confirmed that her serum was positive for the antibodies. After administering intravenous high-dose methylprednisolone (1,000 mg/day for 3 days), her symptoms markedly improved with normalization of myelin basic protein. However, her serum remained positive for AQP 4 antibodies. We think that the patient's diagnosis belongs to the neuromyelitis optica (NMO) spectrum disorders associated with autoimmune disorders. This is a rare case in that the initial presentation was SjS and occurred at a very young age.