Effects of Head Elevation on Cerebral Oxygenation in Acute Cerebral Infarction.

This study clarifies the effects of head elevation on cerebral oxygenation in patients with acute cerebral infarction. We included 76 patients with anterior circulation cerebral infarction admitted to the stroke care unit within 7 days of symptom onset. Using near-infrared spectroscopy (NIRS), we measured regional saturation oxygen (rSO2), oxygenated haemoglobin (O2Hb), deoxygenated haemoglobin (HHb), and total haemoglobin (THb) in both the affected hemisphere (AH) and unaffected hemisphere (UH) after raising the head to 0°, 30°, and 60° for 1 minute each. The mean arterial pressure (MAP) was measured non-invasively at each position. Our result demonstrated a significant decrease in O2Hb as the head elevation angle (HEA) increased, with no observable interaction between the AH and UH. A significant decrease in rSO2 interaction was observed. In contrast, the HHb and THb levels changed significantly as the HEA increased, although no interactions were identified. No significant correlations existed between the observed changes in O2Hb, HHb, THb, or rSO2 and changes in MAP at each HEA.

Prefrontal Cortex Oxygenation During Walking in an Acute Ischaemic Stroke Patient with Severe Internal Carotid Artery Stenosis: A Case Report.

This study investigated the oxygenation in the prefrontal cortex (PFC) during walking in a 75-year-old male patient in the acute phase of a left frontal lobe cerebral infarction complicated by severe left internal carotid artery stenosis. The patient regained independent ambulation on the fifth day after the onset of symptoms, and the study was conducted on the eighth day after the onset of symptoms. The patient rested for 10 s before walking for 70 s. Then he rested for 60 s. The levels of oxyhaemoglobin (O2Hb), deoxyhaemoglobin (HHb), and total haemoglobin (THb) in the PFC were quantified during the rest and walking phases using a wearable near-infrared spectroscopy device. Comparative analysis with baseline values during rest revealed decreases in the O2Hb, HHb, and THb in both hemispheres of the PFC during walking (O2Hb: -19.76/ -14.88 µmol/L, HHb: -1.18/ -2.00 µmol/L, THb: -20.96/ -16.88 µmol/L, right/left, respectively). The recovery of the O2Hb was delayed by 15.8 seconds in the affected hemisphere. These findings support the effectiveness of wearable NIRS for the evaluation of cerebral oxygenation during rehabilitation in patients with acute stroke to facilitate evaluations and individualise patient care.

Changes in Cerebral Oxygenation in the Prefrontal Cortex During Cardiopulmonary Exercise Testing in Patients After Cardiovascular Surgery.

Oxygenated haemoglobin (O2Hb) and total haemoglobin (THb) concentrations rise with increasing exercise load during the cardiopulmonary exercise test (CPX); however, this elevated response is impaired in patients with chronic heart failure. Furthermore, the changes occurring in patients during the acute phase of cardiac surgery are unknown. This study aimed to measure cerebral oxygenation in the prefrontal cortex (PFC) during CPX in patients during the acute post-operative phase following cardiovascular surgery. Fourteen patients in the acute phase of post-cardiovascular surgery period were enrolled. CPX was administered between the post-operative period and discharge. The protocol employed the ramp method (10 W/min) after 3-min rest and 3-min warm-up periods. Levels of O2Hb, deoxygenated haemoglobin (HHb), THb, and the regional cerebral oxygen saturation (rSO2) in the PFC were measured from the resting state through the end of CPX using near-infrared spectroscopy. The mean values of O2Hb, HHb, and THb levels and rSO2 were compared at rest, warm-up, anaerobic threshold, and peak points. At the peak, O2Hb and rSO2 declined significantly, and HHb rose significantly compared to the respective values at rest; no significant changes were observed in THb. These findings suggest that the oxygen supply to the PFC is reduced in patients with reduced cardiac function following cardiovascular surgery.

Patient characteristics with negative diffusion-weighted imaging findings in acute lateral medullary infarction.

BACKGROUND: Diffusion-weighted imaging (DWI) on magnetic resonance imaging (MRI) shows limited sensitivity in the acute-phase brainstem infarctions, including lateral medullary infarction (LMI), and the detailed characteristics of acute LMI patients with initially negative DWI-MRI findings have not been reported previously. Therefore, we aimed to investigate the differences in the backgrounds or symptoms of acute LMI patients with initially negative findings in standard axial DWI-MRI and those with positive findings. METHODS: In this retrospective cohort study, we collected the data for 35 consecutive acute LMI patients who were hospitalized in our hospital from January 2011 to December 2018. Initial standard axial DWI-MRI was assessed, and the patients were divided into positive and negative groups. The characteristics of the two groups were compared, and the usefulness of additional thin-slice coronal DWI-MRI was also investigated. RESULTS: Nine (26%) acute LMI patients were initially negative on standard axial DWI-MRI. The patients were independently associated with smoking history (78% vs. 23%, p = 0.021) and headache (78% vs. 31%, p = 0.046). Thin-slice coronal DWI-MRI showed positive findings in 50% of the patients with negative findings in standard axial DWI-MRI. All four patients with negative findings in both standard axial and thin-slice coronal DWI-MRI had smoking history and headache. CONCLUSION: Smoking history and headache were associated with initial negative results in standard axial DWI-MRI in acute LMI. Additional thin-slice coronal DWI-MRI was sometimes useful in detecting acute LMI. Follow-up MRI is important for patients showing negative findings in initial DWI-MRI.

Extensive leukoencephalopathy associated with idiopathic capillary leak syndrome: report of a case with neuropathology.

INTRODUCTION: Idiopathic systemic capillary leak syndrome (ISCLS) is a rare cryptogenic disorder characterized by recurrent hemoconcentration, hypoalbuminemia, edema, and hypotension due to extravascular fluid leakage. This is the first report that details uncommon extensive leukoencephalopathy caused by ISCLS upon a neuropathological investigation. CASE REPORT: A 68-year-old female had recurrent episodes of hemoconcentration, hypoalbuminemia, and generalized edema and was diagnosed with ISCLS. After 9 years, brain magnetic resonance imaging (MRI) incidentally revealed extensive leukoencephalopathy without neurological deficits. Thorough examinations ruled out other disorders, and the cerebral involvement due to ISCLS was finally diagnosed. Three years later, she developed an acute-onset coma and status epilepticus together with hypotension and hemoconcentration, which were compatible with ISCLS recurrence. Electroencephalogram and MRI were correlated with a seizure arising from the left hemisphere. Extensive leukoencephalopathy did not show notable changes for 3 years. Although treatment for ISCLS recurrence temporally improved hemoconcentration and consciousness, consciousness worsened again by marked edema of the left hemisphere, and she died of cerebral herniation. A brain autopsy revealed straggly perivascular plasma leakage around the small vessels of the deep white matter, which supported that the leukoencephalopathy was caused by ISCLS. Widespread myelin pallor and decreased axonal density with sparse astrogliosis and microgliosis were observed in the cerebral white matter and corresponded with a chronic change in the MRI. CONCLUSION: Current radiological and pathological observations revealed that frequent perivascular leakages could cause chronic leukoencephalopathy, were linked with the development of systemic capillary leakage in ISCLS, and provided insights into the mysterious pathophysiology.

Clinical impact of functional independent measure (FIM) on 180-day readmission and mortality in elderly patients hospitalized with acute decompensated heart failure.

Activities of daily living (ADL) are important prognostic factors for heart failure. The functional independent measure (FIM) has emerged as a comprehensive valid measure of ADL from both physical and cognitive perspectives. This study aimed to investigate the prognostic impact of the FIM score on clinical outcomes in hospitalized patients with acute decompensated heart failure (ADHF). We retrospectively analyzed 473 ADHF patients, with available pre-discharge FIM scores, admitted to our institution between May 2018 and May 2020. Primary outcome measures, defined as a composite of 180-day all-cause deaths and readmissions, were compared among three tertiles. The median FIM score was 102 (interquartile range: 85-115). Tertile 1 corresponded to an FIM score > 111 (n = 154), Tertile 2 to that of 90-111 (n = 167), and Tertile 3 to that of < 90 (n = 152). During follow-up, 28 deaths and 114 readmissions occurred. Patients with lower FIM scores were associated with a graded increase in the risk of primary outcome measure (p = 0.001). Even after multivariable adjustment, the results remained significant [Tertile 1 vs 3; adjusted hazard ratio: 3.28 (95% confidence interval: 1.72-6.56), p < 0.001; Tertile 2 vs 3; 2.32 (1.27-4.47), p = 0.006]. FIM scores were significantly associated with readmission or death within 180 days of discharge in hospitalized ADHF patients.

Acute Internal Carotid Artery Occlusion Long after Carotid Revascularization by Vein Graft.

We report the case of a 27-year-old woman with acute internal carotid artery occlusion long after carotid artery revascularization by vein graft. She presented with sudden unconsciousness and left hemiparesis. Her right carotid artery was revascularized with an ipsilateral internal jugular vein graft during a carotid body tumor resection 10 years ago. Computed tomography angiography revealed a right internal carotid artery terminus occlusion. Intravenous rt-PA and mechanical thrombectomy were performed, resulting in successful recanalization. Her neurological symptoms gradually recovered. When examining the embolic source, carotid ultrasonography for the vein graft showed intimal thickening, some high-echoic plaques, and lumen dilation, but no thrombus was observed. Color Doppler imaging showed laminar flow at the graft. Angiography after thrombectomy also showed pooling of contrast at the vein graft. We suspected that the blood flow stagnation at the vein graft induced thrombus formation; therefore, anticoagulation therapy was initiated. One year later, she was independent without recurrence of stroke, and anticoagulation therapy was replaced with aspirin because she went abroad. However, a carotid ultrasonography exam the following year revealed a huge thrombus at the graft. Anticoagulation therapy was resumed; subsequently, the thrombus decreased. In conclusion, we could monitor the long-term change in the vein graft by ultrasonography. Moreover, anticoagulation therapy was more effective.

Multiple Cerebral Hemorrhages With Microbleeds in Intravascular Large B-Cell Lymphoma.

This is an extremely rare reported case of intravascular large B-cell lymphoma (IVLBCL) presenting with acute hemorrhages and numerous microbleeds. An 80-year-old man presented with consciousness disturbances after convulsion. Computed tomography revealed multiple hemorrhages, and susceptibility-weighted imaging (SWI) demonstrated numerous microbleeds. Brain biopsy showed CD20-positive cells in small vessels; accordingly, IVLBCL was diagnosed. IVLBCL should be considered as a differential diagnosis in multiple cerebral hemorrhages and microbleeds.

Low Muscle Mass is Associated with Walking Function in Patients with Acute Ischemic Stroke.

BACKGROUND: Muscle mass might be a possible predictor for walking function in patients with stroke; however, evidence is limited. OBJECTIVE: To investigate whether skeletal muscle mass is associated with walking function at discharge during the acute phase. METHODS: In this observational cohort study, we assessed skeletal muscle mass in patients with acute ischemic stroke using the noninvasive and portable multifrequency bio-impedance device. This device can easily be used in bedridden patients. Appendicular skeletal muscle mass was converted to skeletal muscle index (SMI) standardizing by height squared (kg/m2). The primary outcome was walking function assessed by the modified Rankin Scale score at acute phase hospital discharge. Logistic regression analysis was used to determine the association between skeletal muscle mass and walking function. RESULTS: Of the 107 patients enrolled, low SMI (SMI: male <7.0 kg/m2, female <5.7 kg/m2) was identified in 29.9% (19.7% in men, 48.6% in women). Logistic regression analysis showed that low SMI [OR: 4.02, 95% confidence interval (CI): 1.38-11.7, p = 0.001] independently associated with walking function at discharge. Further, patients with mild and moderate severity had significant difficulty in walking when they had low SMI (p = 0.039). CONCLUSIONS: Low skeletal muscle mass at the onset of ischemic stroke is an independent predictor of walking function at discharge during the acute phase. Our findings highlight the importance of detecting skeletal muscle mass in patients with acute ischemic stroke.

Successful Reperfusion with Endovascular Therapy Has Beneficial Effects on Long-Term Outcome Beyond 90 Days.

BACKGROUND AND PURPOSE: Recent studies have demonstrated that endovascular reperfusion therapy improves clinical outcomes at 90 days after ischemic stroke. However, the effects on long-term outcomes are not well known. We hypothesized that successful reperfusion might be associated with long-term improvement beyond 90 days after endovascular therapy. To assess the long-term effects beyond 90 days, we analyzed the association of successful reperfusion with a temporal change in modified Rankin Scale (mRS) score from 90 days to 1 year after endovascular therapy. METHODS: We retrospectively analyzed a database of consecutive patients with acute ischemic stroke who received endovascular therapy between April 2006 and March 2016 at 4 centers. We compared the incidences of improvement and deterioration in patients with successful reperfusion (i.e., modified thrombolysis in cerebral infarction score of 2b or 3) with those in patients with unsuccessful reperfusion. We defined improvement and deterioration as decrease and increase on the mRS score by 1 point or more from 90 days to 1 year after endovascular therapy respectively. RESULTS: A total of 268 patients were included in the current study. The rate of patients with improvement tended to be higher in patients with successful reperfusion than in patients with unsuccessful reperfusion (20% [34/167 patients] vs. 12% [12/101], p = 0.07). The rate of patients with deterioration was lower in patients with successful reperfusion than in patients with unsuccessful reperfusion (25% [42/167] vs. 42% [42/101], p < 0.01). After adjustment for confounders, successful reperfusion was associated with improvement (adjusted OR 2.65; 95% CI 1.23-5.73; p < 0.05) and deterioration (adjusted OR 0.33; 95% CI 0.18-0.62; p < 0.01), independent of the 90-day mRS score. CONCLUSIONS: Successful reperfusion has further beneficial legacy effects on long-term outcomes beyond 90 days after stroke.

Isolated cortical vasogenic edema and hyperintense vessel signs may be early features of reversible cerebral vasoconstriction syndrome: Case reports.

Background The temporal and anatomical features of vasoconstriction in patients with reversible cerebral vasoconstriction syndrome within hours after symptom onset, in the hyperacute phase, are unclear. Case result Herein we report the cases of two patients with acute severe headache who were diagnosed with reversible cerebral vasoconstriction syndrome. Magnetic resonance imaging within hours after symptom onset revealed multiple areas of isolated cortical vasogenic edema and hyperintense vessel signs of the distal cerebral arteries. Follow-up imaging performed four days later in both cases showed diffuse segmental arterial vasoconstriction in the proximal regions of the cerebral arteries. Both patients received antivasoconstrictive therapy shortly after admission, and neither had neurological sequelae at discharge. The magnetic resonance imaging findings improved gradually within three months after symptom onset. Conclusion Isolated cortical vasogenic edema and hyperintense vessel signs, when observed within hours from sudden severe headache onset, may be useful early markers of reversible cerebral vasoconstriction syndrome.

Alberta Stroke Program Early CT Score-Time Score Predicts Outcome after Endovascular Therapy in Patients with Acute Ischemic Stroke: A Retrospective Single-Center Study.

BACKGROUND: Clinical outcomes after successful endovascular therapy in patients with acute ischemic stroke are associated with several factors including onset-to-reperfusion time (ORT), the National Institute of Health Stroke Scale (NIHSS) score, and the Alberta Stroke Program Early CT Score (ASPECTS). The NIHSS-time score, calculated as follows: [NIHSS score] × [onset-to-treatment time (h)] or [NIHSS score] × [ORT (h)], has been reported to predict clinical outcomes after intravenous recombinant tissue plasminogen activator therapy and endovascular therapy for acute stroke. The objective of the current study was to assess whether the combination of the ASPECTS and the ORT can predict the outcomes after endovascular therapy. METHODS: The charts of 117 consecutive ischemic stroke patients with successful reperfusion after endovascular therapy were retrospectively reviewed. We analyzed the association of ORT, ASPECTS, and ASPECTS-time score with clinical outcome. ASPECTS-time score was calculated as follows: [11 - ASPECTS] × [ORT (h)]. RESULTS: Rates of good outcome for patients with ASPECTS-time scores of tertile values, scores 5.67 or less, scores greater than 5.67 to 10.40 or less, and scores greater than 10.40, were 66.7%, 56.4%, and 33.3%, respectively (P < .05). Ordinal logistic regression analysis showed that the ASPECTS-time score (per category increase) was an independent predictor for better outcome (common odds ratio: .374; 95% confidence interval: .150-0.930; P < .05). CONCLUSIONS: A lower ASPECTS-time score may predict better clinical outcomes after endovascular treatment.

Combined visual and semi-quantitative assessment of 123I-FP-CIT SPECT for the diagnosis of dopaminergic neurodegenerative diseases.

Visual and semi-quantitative assessments of 123I-FP-CIT single-photon emission computed tomography (SPECT) are useful for the diagnosis of dopaminergic neurodegenerative diseases (dNDD), including Parkinson's disease, dementia with Lewy bodies, progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration. However, the diagnostic value of combined visual and semi-quantitative assessment in dNDD remains unclear. Among 239 consecutive patients with a newly diagnosed possible parkinsonian syndrome who underwent 123I-FP-CIT SPECT in our medical center, 114 patients with a disease duration less than 7 years were diagnosed as dNDD with the established criteria or as non-dNDD according to clinical judgment. We retrospectively examined their clinical characteristics and visual and semi-quantitative assessments of 123I-FP-CIT SPECT. The striatal binding ratio (SBR) was used as a semi-quantitative measure of 123I-FP-CIT SPECT. We calculated the sensitivity and specificity of visual assessment alone, semi-quantitative assessment alone, and combined visual and semi-quantitative assessment for the diagnosis of dNDD. SBR was correlated with visual assessment. Some dNDD patients with a normal visual assessment had an abnormal SBR, and vice versa. There was no statistically significant difference between sensitivity of the diagnosis with visual assessment alone and semi-quantitative assessment alone (91.2 vs. 86.8%, respectively, p = 0.29). Combined visual and semi-quantitative assessment demonstrated superior sensitivity (96.7%) to visual assessment (p = 0.03) or semi-quantitative assessment (p = 0.003) alone with equal specificity. Visual and semi-quantitative assessments of 123I-FP-CIT SPECT are helpful for the diagnosis of dNDD, and combined visual and semi-quantitative assessment shows superior sensitivity with equal specificity.

Ultrasonographic diaphragm thickness correlates with compound muscle action potential amplitude and forced vital capacity.

INTRODUCTION: Noninvasive evaluation of respiratory function in patients with various neuromuscular disorders is important for predicting life expectancy. METHODS: We performed B-mode ultrasonography (USG) and nerve conduction studies in 37 patients (16 had amyotrophic lateral sclerosis; 11, myopathy; and 10, neuropathy) and 10 controls. USG of the diaphragm was performed in the supine position using a linear probe over the intercostal space at the anterior axillary line. Diaphragm muscle thickness was measured at end-expiration. The amplitude of diaphragm compound muscle action potentials (CMAP) was obtained by phrenic nerve stimulation with a surface electrode. Respiratory function was measured with standard pulmonary function tests including forced vital capacity (FVC). RESULTS: Diaphragm thickness was significantly correlated with FVC (r = 0.74) and CMAP amplitude (r = 0.53). CONCLUSIONS: Diaphragm USG is useful for objective evaluation of pulmonary function in neuromuscular disorders without requiring undue patient effort or cooperation.

National Institutes of Health Stroke Scale-Time Score Predicts Outcome after Endovascular Therapy in Acute Ischemic Stroke: A Retrospective Single-Center Study.

BACKGROUND: Outcomes after successful endovascular therapy in acute ischemic stroke are associated with onset-to-reperfusion time (ORT) and the National Institutes of Health Stroke Scale (NIHSS) score. In intravenous recombinant tissue plasminogen activator therapy, the NIHSS-time score, calculated by multiplying onset-to-treatment time with the NIHSS score, has been shown to predict clinical outcomes. In this study, we assessed whether a similar combination of the ORT and the NIHSS score can be applied to predict the outcomes after endovascular therapy. METHODS: We retrospectively reviewed the charts of 128 consecutive ischemic stroke patients with successful reperfusion after endovascular therapy. We analyzed the association of the ORT, the NIHSS score, and the NIHSS-time score with good outcome (modified Rankin Scale score ≤ 2 at 3 months). RESULTS: Good outcome rates for patients with NIHSS-time scores of 84.7 or lower, scores higher than 84.7 up to 127.5 or lower, and scores higher than 127.5 were 72.1%, 44.2%, and 14.3%, respectively (P < .01). Multivariate logistic regression analysis revealed that the NIHSS-time score was an independent predictor of good outcomes (odds ratio, .372; 95% confidence interval, .175-.789) after adjusting for age, sex, internal carotid artery occlusion, plasma glucose level, ORT, and NIHSS score. CONCLUSIONS: The NIHSS-time score can predict good clinical outcomes after endovascular treatment.

Are multifocal motor neuropathy patients underdiagnosed? An epidemiological survey in Japan.

INTRODUCTION: Our objective was to do an epidemiologic survey of patients with multifocal motor neuropathy (MMN) in comparison with those with amyotrophic lateral sclerosis (ALS) in Japan. METHODS: In this retrospective study, we examined 46 patients with MMN and 1,051 patients with ALS from major neuromuscular centers in Japan from 2005 to 2009. Diagnosis was based on the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) and the revised El Escorial criteria. The efficacy of intravenous immunoglobulin (IVIg) was also taken into consideration in the diagnosis of MMN. RESULTS: The ratio of MMN to ALS patients (0­0.10) varied among the centers, but mostly converged to 0.05. The prevalence was estimated to be 0.29 MMN patients and 6.63 ALS patients per 100,000 population. CONCLUSIONS: The frequency of MMN patients was around 1 out of 20 ALS patients, and MMN was possibly underdiagnosed in some centers.

[Enhancing Neuromuscular Disease Diagnosis through Electrophysiology].

Electrophysiologic testing plays an important role in evaluating peripheral nerve, muscle, and neuromuscular junction diseases, aiding in diagnosis and treatment strategies by offering real-time assessment. Demyelination of peripheral nerves results in increased conduction delay, temporal dispersion, conduction block, and stimulation threshold. The localization or diffusion of these changes is crucial in understanding disease pathogenesis, necessitating stimulation at multiple points along nerve pathways. When axonal degeneration occurs, the amplitude is reduced, with mild conduction delay. Acute axonal degeneration may require 1 week to develop into Wallerian degeneration. During this time, conductivity was preserved in the nerve peripheral to the lesion. When MG or LEMS is suspected, repetitive nerve stimulation tests and single-fiber EMG are valuable for the diagnosis and pathophysiological evaluation. Notably, the latter is highly sensitive but not specific. Needle electromyography (EMG) assists in differentiating between myopathies and neurogenic diseases, and in determining whether the patient is in an acute or chronic stage. Integration of these tests contribute to an accurate diagnosis when considering the presenting symptoms.

Activities of daily living limitation and functional decline during hospitalization predict 180-day readmission and mortality in older patients with pneumonia: A single-center, retrospective cohort study.

BACKGROUND: The role of activities of daily living (ADL) as a predictor of adverse outcomes in patients with pneumonia is unclear. This study aimed to assess the association between ADL, including physical and cognitive function, and death or readmission in older inpatients with pneumonia. METHODS: This retrospective, single-center, observational study included consecutive older inpatients with pneumonia between October 2018 and December 2019. ADL was assessed using the Functional Independence Measure (FIM). Functional decline during hospitalization was defined as a decrease of at least 1 point in FIM at discharge from admission. The primary outcome was the time to composite 180-day mortality and readmission from any cause after discharge. RESULTS: In total, 363 patients (median [interquartile range] age: 80 [73-86] years, male: 68 %) were divided according to the median FIM scores (≥100, n = 183 and < 100, n = 180). Among the patients, 25 experienced functional decline during hospitalization, 69 were readmitted, and 17 died. In the Kaplan-Meier analysis, both the lower FIM group and the functional decline group had significantly lower event-free rates than the higher FIM groups and the non-functional decline groups (log-rank test, p < 0.001), respectively. After multivariate analysis, both the lower FIM (adjusted HR, 2.11; 95 % CI, 1.24-3.58; p = 0.006) and functional decline (adjusted HR, 3.18; 95 % CI, 1.44-7.05; p = 0.005) were significantly associated with the primary outcome. CONCLUSIONS: In older patients hospitalized with pneumonia, ADL limitations at discharge and a decline in ADL were associated with poor outcomes.

Subacute Progressive Severe Ataxic Sensory Neuropathy with Sjögren's Syndrome.

We herein report a 79-year-old woman with subacute progressive ataxic sensory neuropathy. The patient's symptoms began with numbness in the lower extremities, which rapidly deteriorated, resulting in gait disturbance and abnormal sensations in the extremities, reaching a peak over a period of approximately two months. Nerve conduction studies revealed pure axonal-type sensory polyneuropathy. The expeditious progression of the disease initially prompted suspicion of Guillain-Barré syndrome or paraneoplastic syndrome. Nevertheless, after comprehensive evaluations, the conclusive diagnosis was confirmed as ataxic sensory neuropathy with Sjögren's syndrome. Intensive immunotherapy was administered; however, it was ineffective in halting disease progression. Consequently, this case underscores the significance of an early comprehensive diagnosis and prompt immunotherapy for ataxic sensory neuropathy associated with Sjögren's.