Application of bedside HINTS, ABCD2 score and truncal ataxia to differentiate cerebellar-brainstem stroke from vestibular neuritis in the emergency room.

BACKGROUND AND PURPOSE: Acute vestibular syndrome (AVS) typically manifests as isolated dizziness or vertigo with no apparent neurological impairments. However, distinguishing life-threatening stroke from innocuous peripheral vestibular lesions in the emergency room (ER) remains challenging. This study aimed to explore the ability of the head impulse-nystagmus-test of skew (HINTS) combined with truncal ataxia or ABCD2 score to differentiate stroke from peripheral vestibular disease in patients with AVS in the ER. METHODS: We prospectively recruited 121 patients with AVS from December 2022 to June 2023, 69 of whom presented with vestibular neuritis (VN) and the remaining with posterior circulation stroke (PCS). We analysed the HINTS results, truncal ataxia and ABCD2 score and compared the sensitivity and specificity among HINTS, truncal ataxia, ABCD2 score and their combinations using the McNemar test for paired samples. RESULTS: HINTS combined with grade 2-3 truncal ataxia achieved significantly higher sensitivity than that of isolated HINTS in differentiating PCS from VN (100% vs 88.5%, p=0.031). The specificity of HINTS plus grade 2-3 truncal ataxia did not significantly differ from that of isolated HINTS (p=0.125); however, the combination of ABCD2 score and HINTS did not improve the diagnostic accuracy. The sensitivity of ABCD2 score ≥4 plus grade 2-3 truncal ataxia was significantly higher than those of isolated ABCD2 score ≥4 or isolated grade 2-3 truncal ataxia (p=0.016 and p<0.001, respectively) and not significantly lower than that of isolated HINTS (p=0.508). CONCLUSION: Compared with the ABCD2 score, the truncal ataxia is of more valuable assistance to HINTS in differentiating PCS. Although the combination of ABCD2 score and truncal ataxia has a significant implication, it is not a replacement for HINTS.

Concordant GRADE-3 Truncal Ataxia and Ocular Laterodeviation in Acute Medullary Stroke.

Background: Severe truncal ataxia associated with an inability to sit up without assistance (STA grade 3) is frequent in patients with central acute vestibular syndrome (AVS) involving the brainstem or cerebellum. When these patients have nystagmus, central HINTS excludes peripheral lesions; however, additional localization and lateralization signs are helpful, not only to resolve the peripheral versus central vestibular lesion dilemma, but to zero in on a precise lesion localization/lateralization to the lateral medulla, the most common ischemic lesion localization associated with an initially false-negative stroke MRI. Methods: This is a study of AVS patients with additional inclusion criteria: grades 2 or 3 ataxia with an eventual diagnosis of medullary stroke (MS), either involving the lateral medulla (LMS) or the medial medulla (MMS), and horizontal (h) gaze paralysis was the main exclusion criteria. All patients sat on the side of the bed or stretcher, with assistance if needed. A general neurologic examination followed in the sitting position, the testing protocol included the head impulse, spontaneous nystagmus, and skew deviation (HINTS) tests, followed by observation of the effect of brief 3-5 sec eyelid closure on ocular position, and saccade and pursuit eye movement tests. If they could sit, the protocol included the ability to stand with a wide base, then a narrow base, the Romberg test, and tandem gait. Radiographic lesion localization and horizontal gaze deviation concluded the protocol. Results: A total of 34 patients met the entry criteria, 34 MS (13 in the lateral medulla, 12 previously described, and 1 new patient), and 1 new MMS. Among them, n = 10/12 had grade 3 ataxia, and 3 (1 new patient) had grade 2 ataxia. In addition, overt ocular laterodeviation (OLD) was present in thirteen of them (35.3%). All OLD patients had gaze deviation and ipsilateral saccade and truncal lateropulsion, 1 medial medulla stroke patient had grade 3 truncal contrapulsion and contralateral hemiparesis without OLD, n = 20/21 patients with LMS without OLD had grade 3 truncal ataxia, and 1 had grade 2 truncal ataxia. Discussion: AVS patients with severe truncal ataxia (inability to sit without assistance) potentially have brainstem, cerebellum, or subcortical lesions. All patients had central HINTS; however, simultaneous direction-concordant STA 3 and OLD provided greater lateral medulla localization specificity, affecting the ipsilateral medulla. Future work to explore a practical posterior circulation stroke scale that includes HINTS, STA, and OLD will potentially select cases for thrombolysis even in the event of initially false-negative imaging.

Bedside Testing in Acute Vestibular Syndrome-Evaluating HINTS Plus and Beyond-A Critical Review.

Acute vertigo and dizziness are frequent presenting symptoms in patients in the emergency department. These symptoms, which can be subtle and transient, present diagnostic challenges because they can be caused by a broad range of conditions that cut across many specialties and organ systems. Previous work has emphasized the value of combining structured history taking and a targeted examination focusing on subtle oculomotor signs. In this review, we discuss various diagnostic bedside algorithms proposed for the acutely dizzy patient. We analyzed these different approaches by calculating their area-under-the-curve (ROC) characteristics and sensitivity/specificity. We found that the algorithms that incorporated structured history taking and the use of subtle oculomotor signs had the highest diagnostic accuracy. In fact, both the HINTS+ bedside exam and the STANDING algorithm can more accurately diagnose acute strokes than early (<24 to 48 h after symptom onset) MRI with diffusion-weighted imaging (DWI). An important caveat is that HINTS and STANDING require moderate training to achieve this accuracy. Therefore, for physicians who have not undergone adequate training, other approaches are needed. These other approaches (e.g., ABCD2 score, PCI score, and TriAGe+ score) rely on vascular risk factors, clinical symptoms, and focal neurologic findings. While these other scores are easier for frontline providers to use, their diagnostic accuracy is far lower than HINTS+ or STANDING. Therefore, a focus on providing dedicated training in HINTS+ or STANDING techniques to frontline clinicians will be key to improving diagnostic accuracy and avoiding unnecessary brain imaging.

Acute truncal ataxia without nystagmus in patients with acute vertigo.

BACKGROUND AND PURPOSE: Differentiating between peripheral and central aetiologies can be challenging in patients with acute vertigo, given substantial symptom overlap. A detailed clinical history and focused physical eye movement examination such as the HINTS eye examination appear to be the most reliable approach to identify acute cerebellar/brainstem stroke, outperforming even acute brain imaging. We have observed, however, that isolated vertigo of central cause may be accompanied by acute truncal ataxia, in the absence of nystagmus. METHODS: We explored the frequency of ataxia without concurrent nystagmus in a cross section of patients with acute vertigo who presented to the emergency department at two centres in Argentina (Group A) and the UK (Group B). Patients underwent detailed clinical neuro-otological assessments (Groups A and B), which included instrumented head impulse testing and oculography (Group B). RESULTS: A total of 71 patients in Group A and 24 patients in Group B were included in this study. We found acute truncal ataxia-without nystagmus-in 15% (n = 14) of our overall cohort. Lesions involved stroke syndromes affecting the posterior inferior cerebellar artery, anterior inferior cerebellar artery, and superior cerebellar artery, thalamic stroke, cerebral hemisphere stroke, multiple sclerosis, and a cerebellar tumour. Additional oculomotor deficits did not reliably identify a central cause in these individuals, even with oculography. CONCLUSIONS: We have identified a significant subpopulation of patients with acute vertigo in whom the current standard approaches such as the HINTS examination that focus on oculomotor assessment may not be applicable, highlighting the need for a formal assessment of gait in this setting.

Role of incubitus truncal ataxia, and equivalent standing grade 3 ataxia in the diagnosis of central acute vestibular syndrome.

INTRODUCTION AND OBJECTIVES: acute vestibular syndrome is a diagnostic challenge, requiring a rapid and precise diagnosis to take therapeutic actions. Truncal ataxia, inability to sit still, and Babinski flexor dysergy were evaluated. Material anf methods: 52 patients with central pathology (stroke in aica and pica territory) and vestibular neuritis were prospectively studied. MRI of the brain was used as the gold standard. RESULTS: A combination of grade 2-3 ataxia to differentiate patients with vestibular neuritis from patients with stroke resulted in a 92% sensitivity (95% CI 79-100%), a 67% specificity (95% CI 47-86%). Flexion asynergy had a 70% sensitivity (95% CI 47-92%), and an 88% specificity (95% CI 69-100%). The inability to sit still correlated well with truncal ataxia. CONCLUSIONS: vestibulospinal signs are useful in the differential diagnosis of acute vestibular syndromes, and the inability to sit is a good substitute for truncal ataxia when it cannot be evaluated.

Acute truncal ataxia in a healthy adult with varicella zoster virus cerebellitis: A case report and literature review.

Acute cerebellitis is a well recognized complication of varicella zoster virus (VZV) infection in children. It has been described in adults in the setting of virus reactivation with a preceding herpes zoster rash, but it is exceedingly rare in adults who are not elderly or immunocompromised, particularly in the absence of a rash. To our knowledge, there has been only one reported case of acute cerebellitis in an immunocompetent adult less than age 65 with virological confirmation of acute VZV infection. We describe a 59-year-old immunocompetent man who presented with acute truncal ataxia without rash and was diagnosed with VZV cerebellitis, supported by anti-VZV IgM and anti-VZV IgG antibodies in the serum and a positive VZV polymerase chain reaction in cerebrospinal fluid. He had robust improvement with intravenous acyclovir treatment and was free of neurologic disability at two month follow-up. This case highlights the importance of virological evaluation in patients with acute ataxia, even in the absence of typical features of infection.

A Unique Case with Oral Dyskinesia, Chorea, Ataxia, and Mild Cognitive Impairment with Caudate Atrophy and Characteristic Brain Calcifications.

The authors report a man who developed oral dyskinesia at 46 years of age, followed by slowly progressive choreic movement and mild cognitive impairment over 20 years. He showed caudate atrophy and four types of intracranial calcification in the hippocampus (dot-like), cerebellar white matter (vague-mass), occipital cortices (laminar), and cerebral white matter (linear). Linear-calcification in the corona radiata seems to be deposition along small veins, which may be related to the white matter changes and to the decreased regional cerebral blood flow in the frontal and parietal lobes. The present case shows a slowly progressive disease with caudate atrophy and characteristic brain calcifications.

Pitfalls in the diagnosis of pupil-sparing oculomotor nerve palsy without limb ataxia: A case report of a variant of Claude's syndrome and neuroanatomical analysis using diffusion-tensor imaging.

Midbrain infarction causing oculomotor nerve palsy with contralateral ataxia is named Claude's syndrome. Herein we report the case of a variant of Claude's syndrome, which shows pupil-sparing oculomotor nerve palsy without the accompanying neurological deficits other than subtle truncal ataxia. MRI and Diffusion Tensor Imaging revealed that midbrain infarction was located rostrally above the decussation of the superior cerebellar peduncle (SCP) and might have partially destructed the tectospinal tract, which resulted in the absence of limb ataxia and presence of subtle truncal ataxia. In this variant of Claude's syndrome, we should carefully assess truncal ataxia to avoid misdiagnosing it as isolated pupil-sparing oculomotor nerve palsy because the patient showed apparently normal gait and truncal ataxia was only revealed by unstable tandem gait.

Enteroviral rhombencephalitis presenting with ocular flutter.

Ocular flutter is a dramatic clinical sign that poses multiple diagnostic considerations. The case description outlines a well young male that presented with ocular flutter and truncal ataxia. The clinical syndrome was subsequently attributed to enteroviral rhombencephalitis. The mechanism and neuroanatomical correlates are discussed, and potential treatments considered.

Clinical Study of Seven Patients with Infarction in Territories of the Anterior Inferior Cerebellar Artery.

BACKGROUND: The prominent features of anterior inferior cerebellar artery (AICA) infarction are vertigo, cerebellar ataxia, and impaired hearing. The present study investigated neurological characteristics associated with AICA infarction. MATERIALS AND METHODS: The locations of infarcts in 7 patients (age, 32-72 years) with AICA infarction were divided into the lower lateral pons, the middle cerebellar peduncle (MCP), and the cerebellum. RESULTS: Ischemic lesions were located in the MCP in 6 patients, spread to the lower lateral pons in 3, and involved the cerebellum in 4 patients. Standing posture and gait were impaired in all patients. Five and 4 patients had impaired hearing and vertigo, respectively. Two patients had only symptoms of labyrinthine disease, and 1 had these symptoms accompanied by impaired hearing. The symptoms in 2 patients with the lesion in the lateral pons were consistent with those in Gasperini syndrome. Two of 3 patients without vertigo had ataxia of the extremities. Stenosis of the vertebral artery or basilar artery in 5 patients indicated that the etiology was branch atheromatous disease. CONCLUSIONS: The most prominent symptom of truncal and gait ataxia and the frequent association between vertigo and impaired hearing were consistent with the characteristics of AICA infarction. Two patients without vertigo had ataxia of the trunk and extremities that might have been due to involvement of the dorsal spinocerebellar tract in the inferior cerebellar peduncle.

The Diagnostic Accuracy of Truncal Ataxia and HINTS as Cardinal Signs for Acute Vestibular Syndrome.

The head impulse, nystagmus type, test of skew (HINTS) protocol set a new paradigm to differentiate peripheral vestibular disease from stroke in patients with acute vestibular syndrome (AVS). The relationship between degree of truncal ataxia and stroke has not been systematically studied in patients with AVS. We studied a group of 114 patients who were admitted to a General Hospital due to AVS, 72 of them with vestibular neuritis (based on positive head impulse, abnormal caloric tests, and negative MRI) and the rest with stroke: 32 in the posterior inferior cerebellar artery (PICA) territory (positive HINTS findings, positive MRI) and 10 in the anterior inferior cerebellar artery (AICA) territory (variable findings and grade 3 ataxia, positive MRI). Truncal ataxia was measured by independent observers as grade 1, mild to moderate imbalance with walking independently; grade 2, severe imbalance with standing, but cannot walk without support; and grade 3, falling at upright posture. When we applied the HINTS protocol to our sample, we obtained 100% sensitivity and 94.4% specificity, similar to previously published findings. Only those patients with stroke presented with grade 3 ataxia. Of those with grade 2 ataxia (n = 38), 11 had cerebellar stroke and 28 had vestibular neuritis, not related to the patient's age. Grade 2-3 ataxia was 92.9% sensitive and 61.1% specific to detect AICA/PICA stroke in patients with AVS, with 100% sensitivity to detect AICA stroke. In turn, two signs (nystagmus of central origin and grade 2-3 Ataxia) had 100% sensitivity and 61.1% specificity. Ataxia is less sensitive than HINTS but much easier to evaluate.

Clinical study of 46 patients with lateral medullary infarction.

BACKGROUND: Lateral medullary infarction (LMI) exhibits a variety of clinical features. Various bulbar symptoms can occur in LMI. METHODS: Neuroradiologic findings of 46 LMI patients were examined. Their infarcts were categorized into the rostral, middle, and caudal groups and were further subdivided into the anteromedial, anterolateral, lateral (L), and posterior regions. RESULTS: The middle medulla was the most common site (27 patients). Most lesions affected the L region alone (25 patients). Dysarthria and facial palsy occurred significantly more frequently in the rostral group than those in the caudal group. Severe truncal ataxia was significantly more common in the caudal group than that in the rostral group. Twenty-five of the 28 patients with severe truncal ataxia displayed vestibular symptoms; otherwise, the other 3 patients showed absence of vestibular symptoms. Soft palate paralysis occurred at a significantly high frequency in the patients with dysphagia and hoarseness compared with the patients without these 2 symptoms. Segmental sensory disturbance occurred in 5 patients, 4 of whom exhibited atypical patterns. CONCLUSIONS: The results of our comparisons between the rostral and caudal groups were consistent with those of previous studies. The presence of severe truncal ataxia without vestibular symptoms in LMI was atypical. An analysis of the bulbar symptoms indicated that the extent to which soft palate paralysis contributed to dysphagia was associated with the severity of ischemia in the nucleus ambiguus. The present study showed variability in clinical features of LMI, which was related to differences in the severity and the extent of ischemia in the lateral medulla.