Rehabilitation of somatic sensation and related deficit of motor control by Mirror Box Therapy: a case report.

Somatosensory disorders are often present after cerebral stroke. These deficits are associated with patients' disability. Therefore, their rehabilitation takes an importance in recovery program. However, the treatment of sensation remains poorly considered during neurorehabilitation and evidence for active sensory training is limited. Mirror Box Therapy is a simple training used to treat upper extremity motor deficits and pain also in patients with stroke. However, the effects of Mirror Box Therapy on somatosensory impairments in post-stroke patients are not deeply investigated and often exclusively motor exercises are provided during therapy.The aim of the present study was to investigate the effects of Mirror Box Therapy sensory training on somatosensory deficits in a stroke patient presenting upper limb impairment.The patient underwent to four weeks of training, five days a week. Before, during and after the Mirror Box Therapy treatment, the patient was assessed by Rivermead Assessment of Somatosensory Performance. Before and after training also upper limb motor function and performance in activities of daily living were assessed.After training patient showed an improvement in somatosensory performance. The gain was maintained at follow-up.This case report shows the effects of Mirror Box Therapy sensory training on the upper extremity for the improvement of sensation and movement in a patient with a thalamo-capsular hemorrhagic stroke during the subacute phase.

Regional White Matter Atrophy Correlates with Spike Activity in Encephalopathy Related to Status Epilepticus During Slow Sleep (ESES) After Early Thalamic Lesions.

Encephalopathy related to Status Epilepticus during slow Sleep (ESES) is an age-related, epileptic syndrome, which associates cognitive/behavioral disturbances with a peculiar pattern of spike activity. One promising line of research is the study of ESES in cases of early thalamic lesions. We studied 7 ESES patients with unilateral thalamic lesions using magnetic resonance imaging to assess regional white matter (WM) and thalamic nuclei volume differences, and long-term electroencephalogram recordings to localize the epileptogenic cortex. N170 event-related potentials were used to demonstrate the dysfunctional character of the WM abnormalities. Diffusion-weighted images in a subset of 4 patients were used to parcellate the thalamus and evaluate volume asymmetries, based on cortical connectivity. Large WM regional atrophy in the hemisphere with the thalamic lesion was associated with both cortical dysfunction and epileptic activity. A correlation was demonstrated between lesions in the pulvinar and the mediodorsal thalamic nuclei and WM atrophy of the corresponding cortical projection areas. We propose that these abnormalities are due to the widespread structural disconnection produced by the thalamic lesions associated to a yet unknown age-dependent factor. Further exploration of WM regional atrophy association with the spike activity in other etiologies could lend support to the cortical disconnection role in ESES genesis.

Anatomical and physiological basis of continuous spike-wave of sleep syndrome after early thalamic lesions.

OBJECTIVE: Early neonatal thalamic lesions account for about 14% of continuous spike-wave of sleep (CSWS) syndrome, representing the most common etiology in this epileptic encephalopathy in children, and promise useful insights into the pathophysiology of the disease. METHODS: We describe nine patients with unilateral neonatal thalamic lesions which progressed to CSWS. Longitudinal whole-night and high-density electroencephalograms (EEGs) were performed, as well as detailed imaging and clinical evaluation. Visual evoked potentials were used to probe cortical excitability. RESULTS: Thalamic volume loss ranged from 19% to 94%, predominantly on medial and dorsal nuclei and sparing the ventral thalamus. Lesions produced white matter loss and ventricle enlargement on the same hemisphere, which in four patients was associated with selective loss of thalamic-cortical fibers. Cortical thickness quantification failed to reveal hemispheric asymmetries. Impact on EEG rhythms was mild, with a volume-loss-related decrease in alpha power and preservation of sleep spindles. The sleep continuous spiking was lateralized to the hemisphere with the lesion. Visual cortex stimulation in five patients with posterior cortex spiking revealed an abnormal frequency-dependent excitability at 10-20Hz on the side of the lesion. SIGNIFICANCE: Unilateral selective thalamic-cortical disconnection is a common feature in our patients and is associated with both a focal pattern of CSWS and a pathological type of frequency-dependent excitability (peak: 10-20Hz). We propose that this excitability represents an abnormal synaptic plasticity previously described as the augmenting response. This synaptic plasticity has been described as absent in the corticocortical interactions in healthy experimental animals, emerging after ablation of the thalamus and producing a frequency-dependent potentiation with a peak at 10-20Hz. Because this response is potentiated by sleep states of reduced brainstem activation and by appropriate stimulating rhythms, such as sleep spindles, the simultaneous occurrence of these two factors in nonrapid-eye-movement sleep is proposed as an explanation for CSWS in our patients.

Impairment of saccade adaptation in a patient with a focal thalamic lesion.

The frequent jumps of the eyeballs-called saccades-imply the need for a constant correction of motor errors. If systematic errors are detected in saccade landing, the saccade amplitude adapts to compensate for the error. In the laboratory, saccade adaptation can be studied by displacing the saccade target. Functional selectivity of adaptation for different saccade types suggests that adaptation occurs at multiple sites in the oculomotor system. Saccade motor learning might be the result of a comparison between a prediction of the saccade landing position and its actual postsaccadic location. To investigate whether a thalamic feedback pathway might carry such a prediction signal, we studied a patient with a lesion in the posterior ventrolateral thalamic nucleus. Saccade adaptation was tested for reactive saccades, which are performed to suddenly appearing targets, and for scanning saccades, which are performed to stationary targets. For reactive saccades, we found a clear impairment in adaptation retention ipsilateral to the lesioned side and a larger-than-normal adaptation on the contralesional side. For scanning saccades, adaptation was intact on both sides and not different from the control group. Our results provide the first lesion evidence that adaptation of reactive and scanning saccades relies on distinct feedback pathways from cerebellum to cortex. They further demonstrate that saccade adaptation in humans is not restricted to the cerebellum but also involves cortical areas. The paradoxically strong adaptation for outward target steps can be explained by stronger reliance on visual targeting errors when prediction error signaling is impaired.

Acute motor-sensory axonal polyneuropathy variant of Guillain-Barré syndrome with a thalamic lesion and COVID-19: a case report and discussion on mechanism.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) primarily affects the respiratory system. During the global coronavirus disease (COVID-19) pandemic, COVID-19-associated neurological diseases have been increasingly reported, including peripheral nervous system diseases, such as Guillain-Barré syndrome (GBS). Acute motor-sensory axonal polyneuropathy (AMSAN), is a GBS variant associated with COVID-19. To date, there are no reports of GBS cases with thalamic injury and dynamic evolution with fluctuating GBS symptoms. In this report, we describe the first case of COVID-19-associated AMSAN accompanied by a thalamic lesion and discuss the magnetic resonance imaging (MRI) findings. Case presentation: A 76-year-old woman, with known co-morbid type 2 diabetes mellitus, presented to the emergency room with complaints of weakness and paraesthesia in both her legs and arms for 4 days, and fever and dry cough for the past 5 days. A nasopharyngeal swab for SARS-CoV-2 returned positive. The patient had not received specific treatment for COVID-19 infection. Neurological examination disclosed symmetric weakness (Medical Research Council grade upper limbs 4/5, lowers limbs 2/5) and areflexia in both the legs and feet. No cranial nerves were involved. Following a neuro-electro-physiology study to evaluate neurological symptoms, AMSAN was suggested. Cerebrospinal fluid (CSF) analysis showed elevated protein levels that confirmed the diagnosis of GBS. The patient was subsequently treated with intravenous immune globulin (IVIG), which improved her neurological symptoms (upper limbs 4/5, lowers limbs 4/5). However, urinary retention, dysarthria, dysphagia, bilateral facial paralysis, facial diplegia, bucking, and motor alalia gradually appeared, followed by aggravated paralysis (upper limbs 3/5, lowers limbs 1/5). After being hospitalized for 16 days, the patient underwent continuous plasma exchange (PE) treatment for a duration of 3 days. Following treatment, the patient's neurological symptoms and paralysis gradually improved (upper limbs 4/5, lowers limbs 4/5) over 2 weeks. Meanwhile, we observed that the patient's cerebral magnetic resonance imaging (MRI) findings dynamically evolved along with the fluctuation of her GBS symptoms, mainly in terms of the changes in T2 hyperintensity in the right thalamus accompanied by microhaemorrhages. The inflammation index was normal. We considered a wide range of possible causes including hypoxia, drugs, toxins, and metabolic derangements but these were excluded. Conclusion: The AMSAN variant of GBS secondary to COVID-19 infection is severe and can cause extensive damage to the peripheral nerves system. The deterioration of symptoms in the patient after early immunotherapy may indicate treatment-related fluctuation (TRF) and could be attributed to immune rebound. Moreover, an excessive immune response post-COVID-19 infection may trigger concurrent damage to the central nervous system, indicating secondary harm to brain small blood vessels and nerve units. For suspected cases of GBS complicated by COVID-19, it is essential to conduct early brain MRI examinations in addition to routine peripheral nervous system evaluations to promptly detect any intracranial lesions. This facilitates appropriate immunotherapy and improves patient prognosis.

Worsening Paraparesis: A Diagnostic Dilemma for Neurosarcoidosis.

Sarcoidosis is a chronic granulomatous disorder mostly known to affect the respiratory system. However, about 5-10% of cases develop neurological complications, either de novo or in patients with known sarcoidosis. The most common complications as cited by current literature include cranial nerve palsies, meningitis, and myelopathy. A unilateral thalamic lesion is an extremely rare presentation of disease. As the neurological manifestations of sarcoidosis are uncommon and variable, it poses a diagnostic challenge. We present a challenging case with worsening paraparesis and a step-by-step approach to how it was diagnosed as neurosarcoidosis. We aimed to create awareness about this uncommon manifestation to avoid misdiagnosis and promote early recognition of neurosarcoidosis.

Primary central nervous system lymphoma presenting with Parkinsonism as an initial manifestation: A case report and literature review.

Primary central nervous system lymphoma (PCNSL) is a rare intracranial neoplasm in older adults. Tumor-associated parkinsonism (TAP) in PCNSL is extremely rare, and its clinical features are unclear. The present report describes the case of a 75-year-old man who presented with parkinsonism due to multiple hyperintense lesions in the thalamus and periventricular white matter as visualized by magnetic resonance imaging (MRI). Due to the rapid progression of parkinsonism and lesion enlargement, the patient underwent stereotaxic biopsy. Subsequently, his condition was diagnosed as TAP in PCNSL at 2 months after onset. The patient completely recovered after treatment and experienced no recurrence of TAP for 8 months. Although it is difficult to distinguish TAP from vascular parkinsonism (VP) at initial consultation, the early diagnosis of PCNSL is important for improving prognosis. In the case of rapidly progressing parkinsonism, one should suspect the possibility of TAP associated with early-stage PCNSL. Early treatment improves the chances of remission and decreases the possibility of recurrence.

[A case suspected of dystonia with marked cerebellar atrophy with torsion dystonia of the neck and cerebellar ataxia that developed during pharmacologic schizophrenia treatment].

A 46 year-old man with schizophrenia had taken several anti-psychotic drugs since 25 years of age. From ~35 years of age, he noticed occasional neck torsion to the left, and later an ataxic gait; both symptoms gradually worsened. On admission, the patient was taking olanzapine (5 mg/day) and biperiden hydrochloride (1 mg/day) because his schizophrenia was well controlled. His parents were not consanguineous, and there was no family history of neuropsychiatric diseases. On neurological examination, he showed mild cognitive impairment, saccadic eye pursuit with horizontal gaze nystagmus, mild dysarthria, dystonic posture and movement of the neck, incoordination of both hands, and an ataxic gait. Deep tendon reflexes were normal except for the patellar tendon reflex, which was exaggerated bilaterally. Pathological reflexes were negative and there was no sign of rigidity, sensory disturbance or autonomic dysfunction. Ophthalmological examinations detected thinning of the outer macula lutea in both eyes, indicative of macular dystrophy. After admission, all anti-psychotic drugs were ceased, but his dystonia was unchanged. Levodopa and trihexyphenidyl hydrochloride were not effective. General blood, urine and cerebrospinal fluid examinations showed no abnormalities. Brain MRI showed cerebellar atrophy and bilateral symmetrical thalamic lesions without brainstem atrophy or abnormal signals in the basal ganglia. I123-IMP SPECT also revealed a decreased blood flow in the cerebellum. Genetic screening, including whole exome sequencing conducted by the Initiative on Rare and Undiagnosed Disease identified no possible disease-causing variants. The patient's dystonia worsened and choreic movements manifested on his right hand and foot. We suspected dystonia with marked cerebellar atrophy (DYTCA), but could not exclude drug-induced dystonia. Macular dystrophy and bilateral thalamic lesions on brain MRI have not been previously described in DYTCA. Whether these features might be primarily associated with dystonia or cerebellar ataxia now remains to be determined.

Adult Langerhans cell histiocytosis of the central nervous system.

Langerhans cell histiocytosis (LCH), a disorder characterized by aberrant function and proliferation of mononuclear phagocytic cells called Langerhans cells, usually occurs in those <15 years of age. Adult-onset LCH is extremely rare. We present a case of a 35-year-old woman who presented with multiple episodes of confusion, rapidly progressive cognitive decline, and multiple endocrinopathies, including diabetes insipidus. Brain imaging showed a hypothalamic lesion, and biopsy results confirmed the diagnosis of LCH. Given the wide variety of presentations and the multiple differential diagnoses of hypothalamic lesions, it is essential to be aware of this uncommon condition, especially in adults, where it may be underdiagnosed.

Longitudinal magnetic resonance imaging changes in Japanese encephalitis.

BACKGROUND: Japanese encephalitis is a flavivirus that can cause pandemic encephalitis, and is prevalent in Southeast Asia and Australia. Brain images of patients with Japanese encephalitis are characterized by thalamic lesions, distinct from those seen in viral encephalopathies caused by the herpes simplex virus and West Nile virus. AIM: Herein, we describe for the first time a time-dependent magnetic resonance imaging pattern in Japanese encephalitis in a 10-month-old Japanese boy. CASE: The patient was a previously healthy 10-month-old Japanese boy, who exhibited acute-onset flaccid tetraplegia and loss of tendon reflexes. RESULTS: Brain MRI showed characteristic thalamic changes on diffusion weighted images from spotty to uniform and from the left to the right side, associated with low apparent diffusion coefficient maps. These images suggest that the Japanese encephalitis virus may first affect the unilateral thalamus, possibly expanding to the other side, with characteristic patterns changing from spotty to uniform in a manner consistent with the presentation of cytotoxic edema. CONCLUSION: This report first showed longitudinal magnetic resonance changes in Japanese encephalitis, which may help in accurate diagnosis and in discrimination from other etiologies.

Cranial imaging findings in dengue virus infection.

BACKGROUND: The aim of this study is to evaluate cranial CT or MRI changes in dengue encephalitis and their correlation with clinical and biochemical findings. METHODS: Twenty-one serologically confirmed patients with dengue with altered sensorium were included who underwent MRI (20)/CT (1) scan study. Their clinical details including seizure, hypotension, bleeding diathesis, focal neurologic deficit, and Glasgow Coma Scale (GCS) score were noted. Blood counts, hematocrit, renal and liver function tests, electrolytes, cerebrospinal fluid (CSF) and ECG were done. MRI findings on T1, T2, FLAIR, DWI and T1 gadolinium contrast were noted. RESULTS: The median age of the patients was 30 (5-69) years and 5 were females. MRI was abnormal in 9(45%) and CT scan in 1 patient with dengue shock syndrome revealed cerebellar and subdural hematoma. The MRI lesions were in thalamic and basal ganglia in 3, focal cortical areas in 3, white mater in 2 and meningeal enhancement in 3 patients. Seven of these patients had CSF pleocytosis. The presence of abnormal imaging was not related to outcome. One patient died, 1 was bed ridden and 19 had complete recovery on discharge. CONCLUSION: Cranial imaging reveals nonspecific changes and is not related to hematological and biochemical changes or outcome.

Thalamic lesions in acute encephalopathy with biphasic seizures and late reduced diffusion.

BACKGROUND: We aimed to assess the characteristics of thalamic lesions in children with acute encephalopathy with biphasic seizures and late reduced diffusion. METHODS: Using the Tokai Pediatric Neurology Society database, we identified and enrolled 18 children with acute encephalopathy with biphasic seizures and late reduced diffusion from 2008 to 2010. Using diffusion-weighted images, we identified patients with thalamic lesions and compared their clinical factors with those of patients without thalamic lesions. We analyzed the time sequence of thalamic, sucortical, and cortical lesions. To study the topography of thalamic lesions, we divided the thalamus into five sections: anterior, medial, anterolateral, posterolateral, and posterior. Subsequently, we analyzed the relationship between the topography of thalamic lesions and the presence of central-sparing. RESULTS: Seven children presented with symmetrical thalamic lesions associated with bilateral subcortical or cortical lesions. No statistical difference in the clinical features was observed between individuals with and without thalamic lesions. These lesions were observed only when subcortical or cortical lesions were present. In 5 children, thalamic lesions were present in bilateral anterior or anterolateral sections and were associated with subcortical or cortical lesions in bilateral frontal lobes with central-sparing. In the other two children, thalamic lesions were extensive and accompanied by diffuse subcortical and cortical lesions without central-sparing. CONCLUSION: Thalamic lesions in patients with acute encephalopathy with biphasic seizures and late reduced diffusion involve the anterior sections. The thalamocortical network may play a role in development of thalamic lesions in patients with acute encephalopathy with biphasic seizures and late reduced diffusion.

Thalamic alexia with agraphia.

Alexia with agraphia is defined as an acquired impairment affecting reading and writing ability. It can be associated with aphasia, but can also occur as an isolated entity. This impairment has classically been associated with a left angular gyrus lesion In the present study, we describe a case involving a patient who developed alexia with agraphia and other cognitive deficits after a thalamic hemorrhage. In addition, we discuss potential mechanisms of this cortical dysfunction syndrome caused by subcortical injury. We examined a patient who presented with alexia with agraphia and other cognitive deficits due to a hemorrhage in the left thalamus. Neuropsychological evaluation showed attention, executive function, arithmetic and memory impairments. In addition, language tests revealed severe alexia with agraphia in the absence of aphasia. Imaging studies disclosed an old thalamic hemorrhage involving the anterior, dorsomedial and pulvinar nuclei. Tractography revealed asymmetric thalamocortical radiations in the parietal region (left