Dysgeusia induced and resolved by focused ultrasound thalamotomy: case report

Dysgeusia, or distorted taste, has recently been acknowledged as a complication of thalamic ablation or thalamic deep brain stimulation as a treatment of tremor. In a unique patient, left-sided MR-guided focused ultrasound thalamotomy improved right-sided essential tremor but also induced severe dysgeusia. Although dysgeusia persisted and caused substantial weight loss, tremor slowly relapsed. Therefore, 19 months after the first procedure, the patient underwent a second focused ultrasound thalamotomy procedure, which again improved tremor but also completely resolved the dysgeusia. On the basis of normative and patient-specific whole-brain tractography, the authors determined the relationship between the thalamotomy lesions and the medial border of the medial lemniscus—a surrogate for the solitariothalamic gustatory fibers—after the first and second focused ultrasound thalamotomy procedures. Both tractography methods suggested partial and complete disruption of the solitariothalamic gustatory fibers after the first and second thalamotomy procedures, respectively. The tractography findings in this unique patient demonstrate that incomplete and complete disruption of a neural pathway can induce and resolve symptoms, respectively, and serve as the rationale for ablative procedures for neurological and psychiatric disorders.

Direct brainstem somatosensory evoked potentials for cavernous malformations

OBJECTIVE Brainstem cavernous malformations (CMs) often require resection due to their aggressive natural history causing hemorrhage and progressive neurological deficits. The authors report a novel intraoperative neuromonitoring technique of direct brainstem somatosensory evoked potentials (SSEPs) for functional mapping intended to help guide surgery and subsequently prevent and minimize postoperative sensory deficits. METHODS Between 2013 and 2019 at the Stanford University Hospital, intraoperative direct brainstem stimulation of primary somatosensory pathways was attempted in 11 patients with CMs. Stimulation identified nucleus fasciculus, nucleus cuneatus, medial lemniscus, or safe corridors for incisions. SSEPs were recorded from standard scalp subdermal electrodes. Stimulation intensities required to evoke potentials ranged from 0.3 to 3.0 mA or V. RESULTS There were a total of 1 midbrain, 6 pontine, and 4 medullary CMs—all with surrounding hemorrhage. In 7/11 cases, brainstem SSEPs were recorded and reproducible. In cases 1 and 11, peripheral median nerve and posterior tibial nerve stimulations did not produce reliable SSEPs but direct brainstem stimulation did. In 4/11 cases, stimulation around the areas of hemosiderin did not evoke reliable SSEPs. The direct brainstem SSEP technique allowed the surgeon to find safe corridors to incise the brainstem and resect the lesions. CONCLUSIONS Direct stimulation of brainstem sensory structures with successful recording of scalp SSEPs is feasible at low stimulation intensities. This innovative technique can help the neurosurgeon clarify distorted anatomy, identify safer incision sites from which to evacuate clots and CMs, and may help reduce postoperative neurological deficits. The technique needs further refinement, but could potentially be useful to map other brainstem lesions.

Pontine hemorrhage presenting as Foville’s syndrome

Foville’s syndrome, also known as inferior medial pontine syndrome is one of the rare brainstem stroke syndromes with only few cases reported worldwide occurring due to involvement of the infero-medial aspect of pons. Condition is characterised by various cluster of neurological features as a result of defect in multiple vital areas like cortico spinal tract, medial lemniscus, middle cerebral peduncle, facial nerve and abducens nerve involvement. We reported one such rare case of a patient with no known systemic co-morbidity, who presented with sudden onset diplopia, lagophthalmos and contralateral weakness of limbs. On evaluation with computed tomography imaging, hemorrhage at the level of inferior pons was found. Patient subsequently treated and commenced on physiotherapy for rehabilitation.

Brain Connectivity Affecting Gait Function after Unilateral Supratentorial Stroke

Gait dysfunction is a leading cause of long-term disability after stroke. The mechanisms underlying recovery of gait function are unknown. We retrospectively evaluated the association between structural connectivity and gait function in 127 patients with unilateral supratentorial stroke (>1 month after stroke). All patients underwent T1-weighted, diffusion tensor imaging and functional ambulation categorization. Voxel-wise linear regression analyses of the images were conducted using fractional anisotropy, mean diffusivity, and mode of anisotropy mapping as dependent variables, while the functional ambulation category was used as an independent variable with age and days after stroke as covariates. The functional ambulation category was positively associated with increased fractional anisotropy in the lesioned cortico-ponto-cerebellar system, corona radiata of the non-lesioned corticospinal tract pathway, bilateral medial lemniscus in the brainstem, and the corpus callosum. The functional ambulation category was also positively associated with increased mode of anisotropy in the lesioned posterior corpus callosum. In conclusion, structural connectivity associated with motor coordination and feedback affects gait function after stroke. Diffusion tensor imaging for evaluating structural connectivity can help to predict gait recovery and target rehabilitation goals after stroke.

Brain White Matter Abnormality Induced by Chronic Spinal Cord Injury in the Pediatric Population: A Preliminary Tract-based Spatial Statistic Study

Objectives: Tract-based spatial statistics (TBSS) is a diffusion tensor imaging (DTI)–based processing technique that aims to improve the objectivity and interpretability of analysis of multisubject diffusion imaging studies. This study used TBSS to measure quantitative changes in brain white matter structures following spinal cord injury (SCI). Methods: Eighteen SCI subjects aged 8–20 years old (mean age, 16.5 years) were scanned using a conventional single-shot EPI DTI protocol using a 3.0T Siemens MR scanner. All participants underwent a complete International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) examination to determine the level and severity of injury. Five participants were classified as American Spinal Injury Association Impairment Scale (AIS) A, nine as AIS B, and four as AIS C/D. Imaging parameters used for data collection were as follows: 20 directions, b = 1000 s/mm2, voxel size = 1.8 mm × 1.8 mm, slice thickness = 5 mm, TE = 95 ms, TR = 4300 ms, slices = 30, TA = 4:45 min. To generate TBSS, nonparametric permutation tests were used for voxel-wise statistical analysis of the fractional anisotropy (FA) skeletons between AIS groups. A two-tailed t test was applied to extract voxels with significant differences at p < .05. Results: Notable significant changes occurred throughout the corticospinal, spinothalamic, and dorsal column/medial lemniscus tracts. Altered regions in the temporal, occipital, and parietal lobes were also identified. Conclusion: These results suggest that white matter structures are altered differently between people with different AIS classifications. TBSS has the potential to serve as a screening tool to identify white matter changes in regions of interest.

Successful Application of an Insole with a Metatarsal Inhibition Bar and Deep Heel Cup for Improving Gait Dysfunction in a Patient with Poor Coordination with Disrupted Corticoreticular Tracts: A Case Report

We report the successful management of gait dysfunction in a patient with coordination problems using an insole with a metatarsal inhibition bar (MIB) and a deep heel cup. Furthermore, we investigated the state of the neural tracts via diffusion tensor tractography (DTT). A 23-month-old boy with gait dysfunction presented with toe walking with a wide base and decreased hip flexion. Motor weakness or spasticity was not observed. Conventional brain magnetic resonance imaging did not reveal any abnormal findings, but DTT revealed disrupted bilateral corticoreticulospinal tracts (CRTs). No abnormalities were observed in the corticospinal tract or the medial lemniscus. We applied a custom-made insole with an MIB and a deep heel cup. Immediately after application, the patient’s gait pattern stabilized significantly and was nearly normalized. Our therapeutic experience indicates that the application of an insole with an MIB and deep heel cups could be beneficial for patients with coordination problems and gait dysfunction. Our DTT results showed that CRTs could be the causative brain pathology for gait dysfunction in patients with coordination problems.

Endovascular repair and open repair surgery of thoraco-abdominal aortic aneurysms cause drastically different types of spinal cord injury

Both endovascular repair (EVR) and open repair (OR) surgery of thoraco-abdominal aortic aneurysms cause spinal cord (SC) injury that can lead to paraparesis or paraplegia. It has been assumed that mechanisms responsible for SC damage after EVR are similar to those after OR. This pilot study compared the pathophysiology of SC injury after EVR versus OR using a newly developed EVR dog model. An increasing number of stents similar to those used in patients were inserted in the aorta of three dogs to ensure thoracic or thoracic plus lumbar coverage. The aorta of OR dogs was cross-clamped for 45 min. Behavior assessment demonstrated unique patterns of proprioceptive ataxia and evolving paraparesis in EVR versus irreversible paraplegia in OR. MRI showed posterior signal in lumbar SC after EVR versus central cord edema after OR. Histopathology showed white matter edema in L3–L5 localized to the dorsal column medial lemniscus area associated with loss of myelin basic protein but not neurons after EVR, versus massive neuronal loss in the gray matter in L3–L5 after OR. Metabolome analysis demonstrates a distinctive chemical fingerprint of cellular processes in both interventions. Our results call for the development of new therapeutics tailored to these distinct pathophysiologic findings.

Microstructural White Matter Alterations in Cognitively Impaired Patients at Early Stages of Multiple Sclerosis

Purpose As conventional quantitative magnetic resonance imaging (MRI) parameters are weakly associated with cognitive impairment (CI) in early multiple sclerosis (MS), we explored microstructural white matter alterations in early MS or clinically isolated syndrome (CIS) comparing patients with or without CI. Methods Based on a preceding tract-based spatial statistics analysis (3 Tesla MRI) which contrasted 106 patients with early MS or CIS and 49 healthy controls, diffusion metrics (fractional anisotropy, FA, mean diffusivity, MD) were extracted from significant clusters using an atlas-based approach. The FA and MD were compared between patients with (Ci_P n = 14) and without (Cp_P n = 81) cognitive impairment in a subset of patients who underwent CI screening. Results The FA was reduced in Ci_P compared to Cp_P in the splenium of corpus callosum (p = 0.001), right parahippocampal cingulum (p = 0.002) and fornix cres./stria terminalis (0.042), left posterior corona radiata (p = 0.012), bilateral cerebral peduncles, medial lemniscus and in cerebellar tracts. Increased MD was detected in the splenium of corpus callosum (p = 0.01). The CI-related localizations overlapped only partially with MS lesions. Conclusion Microstructural white matter alterations at disease onset were detectable in Ci_P compared to Cp_P in known cognitively relevant fiber tracts, indicating the relevance of early treatment initiation in MS and CIS.

Decreased Microstructural Integrity of the Central Somatosensory Tracts in Diabetic Peripheral Neuropathy

Context Although diabetic peripheral neuropathy (DPN) is predominantly considered a disorder of the peripheral nerves, some evidence for central nervous system involvement has recently emerged. However, whether or to what extent the microstructure of central somatosensory tracts may be injured remains unknown. Objective This work aimed to detect the microstructure of central somatosensory tracts in type 2 diabetic patients and to correlate it with the severity of DPN. Methods A case-control study at a tertiary referral hospital took place with 57 individuals with type 2 diabetes (25 with DPN, 32 without DPN) and 33 nondiabetic controls. The fractional anisotropy (FA) values of 2 major somatosensory tracts (the spinothalamic tract and its thalamocortical [spino-thalamo-cortical, STC] pathway, the medial lemniscus and its thalamocortical [medial lemnisco-thalamo-cortical, MLTC] pathway) were assessed based on diffusion tensor tractography. Regression models were further applied to detect the association of FA values with the severity of DPN in diabetic patients. Results The mean FA values of left STC and left MLTC pathways were significantly lower in patients with DPN than those without DPN and controls. Moreover, FA values of left STC and left MLTC pathways were significantly associated with the severity of DPN (expressed as Toronto Clinical Scoring System values) in patients after adjusting for multiple confounders. Conclusion Our findings demonstrated the axonal degeneration of central somatosensory tracts in type 2 diabetic patients with DPN. The parallel disease progression of the intracranial and extracranial somatosensory system merits further attention to the central nerves in diabetic patients with DPN.

Improvement of Gait Dysfunction after Applying a Hinged Ankle–Foot Orthosis in a Hemiplegic Cerebral Palsy Patient with Disrupted Medial Lemniscus: A Case Report

We describe the successful application of hinged ankle−foot orthoses (AFOs) in a cerebral palsied (CP) patient with gait instability due to a disrupted medial lemniscus (ML). The patient was a 27-month-old male CP child with gait instability who presented with reduced knee flexion and ankle dorsiflexion, with severe genu recurvatum on his right lower extremity during gait. The patient had no motor weakness or spasticity. Conventional magnetic resonance imaging (MRI) revealed no definite abnormal lesion. However, diffusion tensor tractography (DTT) showed disruption of the left ML, consistent with right hemiplegic symptoms. The integrity of the major motor-related neural tracts, including the corticospinal and corticoreticulospinal tracts, was preserved. We considered that the patient’s abnormal gait pattern was related to the disrupted ML state. We applied hinged AFOs, which immediately resulted in a significantly stabilized gait. The angles of knee flexion and ankle dorsiflexion increased. Our findings indicate that the application of hinged AFOs could be a useful therapeutic option for CP patients with gait instability related to ML disruption. In addition, we showed that DTT is a useful tool for identifying the causative brain pathology in CP patients, especially when conventional brain MRIs show no specific lesion.