scholarly journals Vietnamese patient with progressive ataxia and palatal tremor syndrome

Author(s):  
A Jahangirvand ◽  
K Sawicka ◽  
S Kennedy

Background: We present clinical and MRI features of progressive ataxia and palatal tremor (PAPT). Case Report: A 67 year-old gentleman visiting from Vietnam presented with intermittent stroke-like episodes consisting of facial weakness, dysarthria, and osillopsia. He reported gradually worsening ataxia and dysequilibrium over 4 years. Examination revealed small amplitude nystagmus towards the right, impaired VOR to the left, palatal tremor, left-sided dysmetria, and an unsteady gait. MRI of the brain demonstrated increased T2/FLAIR signal within the inferior olive. Contrast enhanced and diffusion sequences were normal. MRA was normal. Electrocardiography, telemetry, and echocardiogram were normal. CSF was normal. Glial fibrillary acidic protein (GFAP) and vitamin E levels were normal. Genetic testing for hereditary forms of ataxia including spinocerebellar ataxia was not completed. Conclusion: Palatal tremor is commonly classified into symptomatic or essential subgroups. Symptomatic palatal tremor is frequently caused by a lesion in the triangle of Guillain and Mollaret leading to hypertrophic olivary degeneration. A subgroup of symptomatic palatal tremor form a syndrome of PAPT. Published details of cases of PAPT are sparse and the disorder appears to be mainly sporadic. Common features include progression of ataxia, olivary degeneration, gaze-evoked nystagmus, and internuclear ophthalmoplegia. There is no known effective treatment for progressive ataxia, which is the most disabling symptom of PAPT.

2020 ◽  
Vol 13 (11) ◽  
pp. e236188
Author(s):  
Jagadeesh Sutraye ◽  
Mohan Kannam ◽  
Rajat Kapoor ◽  
Virender Sachdeva

A 44-year-old obese woman presented with decrease in vision in the right eye (RE) for 3 days. She reported a simultaneous onset of holocranial headache that worsened on bending forward. She denied eye pain, pain on eye movements, and other ocular or neurological complaints. On examination, her distance best-corrected visual acuity was counting fingers at 1 m in the RE and 20/20 in the left eye (LE). Colour vision was subnormal in both eyes (BE). There was grade II relative afferent pupillary defect in the RE. Fundus examination showed disc oedema in BE . Visual fields in the LE showed central scotoma extending nasally. A provisional diagnosis of papillitis was considered. However, contrast-enhanced MRI of the brain and orbits showed evidence of elevated intracranial pressure. Cerebrospinal fluid (CSF) opening pressure was 42 cm H2O while rest of the CSF analysis was normal. Diagnosis was revised to fulminant idiopathic intracranial hypertension. Management with medical therapy and urgent thecoperiteoneal shunt improved visual function in BE.


2000 ◽  
Vol 37 (3) ◽  
pp. 271-274 ◽  
Author(s):  
J. Fox ◽  
R. Duncan ◽  
P. Friday ◽  
B. Klein ◽  
W. Scarratt

A 12-month-old American Miniature horse colt was presented to the Virginia Tech Veterinary Teaching Hospital with a 7-month history of progressive ataxia. Physical examination revealed a head intention tremor, base-wide stance, and ataxia. Necropsy findings were confined to the brain. There were bilateral areas of liquefactive necrosis and cavitation corresponding to the dorsal accessory olivary and lateral (accessory) cuneate nuclei. Cerebellar folia of the dorsal vermis were thin. Microscopically, the cerebellar cortex was characterized by patchy areas of Purkinje cell loss with associated variable thinning of the molecular and granule cell layers and astrogliosis. Dorsal accessory olivary and lateral cuneate nuclei were cavitated and had mild glial response around their periphery. Additionally, a focus of necrosis and neuropil vacuolization was found in the right putamen. These findings indicate the presence of a neurodegenerative disorder centered, but not confined to, the cerebellum and its connections in this American Miniature horse colt.


2021 ◽  
Author(s):  
Seyed Amir Zamanpour ◽  
Bahare Bigham ◽  
Mohamad-Hoseyn Sigari ◽  
Hoda Zare

Abstract Introduction: Accurate, fast, and reliable diagnosis of Alzheimer's Disease (AD) from Mild Cognitive Impairment (MCI) is crucial for prescribing proper treatment and prevention of disease progression. At first glance, structural and diffusion MRI images, are affected by neurodegenerative proceedings in AD and MCI. In this study, we are looking for the most effective features to detect and differentiate between healthy normal control (NC), AD, and MCI groups by non-invasive Magnetic Resonance Imaging (MRI) method and propose the automatic multi-class classification using the structural and diffusion MRI Features of the brain. Methods: The structural and diffusion MRI data were downloaded from the Alzheimer’s Disease Neuroimaging Initiative (ADNI) database on three groups including AD, MCI, and NC subjects. Four famous classification models of machine learning were used to discover the best classification as a diagnostic tool for separation of the NC, AD and MCI groups. Results: Taken together, our results from this study lead to classify three groups for differentiation between the NC group and patients with MCI and AD, with average accuracy factor 89.9% for Support Vector Machine (SVM) and 91.9% for Artificial Neural Network (ANN) using selected features. Conclusions: Top 9 regions repetitive of WM based on four types of features are the caudate nucleus, corpus callosum, hippocampus, para hippocampus, temporal gyrus, putamen nucleus, cingulate gyrus, the region of 36 and 3 Brodmann. Therefore, these regions could be considered for identifying, monitoring, and future drug trials that could target this brain region to AD and MCI Management.


Author(s):  
M. Sato ◽  
Y. Ogawa ◽  
M. Sasaki ◽  
T. Matsuo

A virgin female of the noctuid moth, a kind of noctuidae that eats cucumis, etc. performs calling at a fixed time of each day, depending on the length of a day. The photoreceptors that induce this calling are located around the neurosecretory cells (NSC) in the central portion of the protocerebrum. Besides, it is considered that the female’s biological clock is located also in the cerebral lobe. In order to elucidate the calling and the function of the biological clock, it is necessary to clarify the basic structure of the brain. The observation results of 12 or 30 day-old noctuid moths showed that their brains are basically composed of an outer and an inner portion-neural lamella (about 2.5 μm) of collagen fibril and perineurium cells. Furthermore, nerve cells surround the cerebral lobes, in which NSCs, mushroom bodies, and central nerve cells, etc. are observed. The NSCs are large-sized (20 to 30 μm dia.) cells, which are located in the pons intercerebralis of the head section and at the rear of the mushroom body (two each on the right and left). Furthermore, the cells were classified into two types: one having many free ribosoms 15 to 20 nm in dia. and the other having granules 150 to 350 nm in dia. (Fig. 1).


2020 ◽  
Vol 17 (2) ◽  
pp. 110-120
Author(s):  
N.D. Sorokina ◽  
◽  
L.R. Shahalieva ◽  
S.S. Pertsov ◽  
L.V. Polma ◽  
...  

One of the most common causes of chronic pain in the facial region, including in the trigeminal nerve link, which is not associated with dental diseases, is pain dysfunction of the temporomandibular joint. At the same time, there is evidence in the literature that there are relationships between pain dysfunction of the temporomandibular joint, abnormal occlusion, cervical-muscular tonic phenomena, postural disorders, dysfunction of the Autonomous nervous system and cochleovestibular manifestations. At the same time, neurophysiological indicators of functional disorders in the maxillofacial region and intersystem interactions in pain dysfunction of the temporomandibular joint are insufficiently studied.Goal. The aim of the work is to evaluate the neurophysiological features of trigeminal afferentation in terms of trigeminal somatosensory evoked potentials (TSWP) and the auditory conducting system of the brain in terms of acoustic stem evoked potentials (ASVP) in distal occlusion of the dentition with pain dysfunction of the temporomandibular joint (TMJ) in comparison with physiological occlusion in students 18-21 years old. Material and methods. The main study included 41 students with distal occlusion (21 girls and 20 boys), (grade II Engl, symmetrically right and left in 14 people, and grade II Engl on the left and grade I on the right in 12 people, grade I on the left and grade II on the right in 15 people). All respondents with distal occlusion and who were practically healthy signed an informed consent to participate in the study. We used complex orthodontic methods of examination, subjective degree of severity and intensity of pain in the TMJ, assessment of the Autonomous nervous system (samples and tests), and neurophysiological methods for assessing TSVP and ASVP. Results. Significant differences in ASEP parameters were found in the group of respondents with distal occlusion in the form of a decrease in the latency period of peak I, III, and V compared to physiological occlusion, that correlated with the subjective assessment (in points) of cochleovestibular disorders. According to the TSVP study, a decrease in the duration of latent periods was found, which indicates an increased excitability of non-specific brain stem structures at the medullo-ponto-mesencephalic level compared to the control group. Conclusions. The results obtained are supposed to be used for differential diagnostics, including such dental diseases as TMJ pain dysfunction, occlusion abnormalities accompanied by pain syndrome. Additional functional diagnostics of multi-modal VP of the brain (acoustic evoked potentials, trigeminal evoked potentials) can be performed in conjunction with indicators of autonomic nervous system dysfunction, with parameters of severity of clinical symptoms of cochleovestibular disorders, musculoskeletal dysfunction the maxillofacial area, with indicators of pain, which will determine the tactics and effectiveness of subsequent treatment.


2021 ◽  
pp. 088307382199128
Author(s):  
Hafize Emine Sönmez ◽  
Ferhat Demir ◽  
Semanur Özdel ◽  
Şerife Gül Karadağ ◽  
Esra Bağlan ◽  
...  

Objective: Takayasu arteritis is a rare granulomatous chronic vasculitis that affects the aorta and its main branches. Neurologic manifestations can accompany the disease; however, there is no study on neuroimaging in children with Takayasu arteritis. Therefore, we aimed to evaluate cranial magnetic resonance imaging (MRI) in pediatric Takayasu arteritis patients. Materials and Methods: Demographic, clinical, and laboratory data were obtained retrospectively. Results: The study included 15 pediatric Takayasu arteritis patients. All patients presented with constitutional symptoms. Additionally, 6 patients suffered from headache, 2 had syncope, 1 had loss of consciousness, and 1 had convulsion. All patients underwent cranial and diffusion MRI a median 12 months after diagnosis. Cranial MRI findings were normal in 12 patients, whereas 3 patients had abnormal findings, as follows: stenosis in the M1 and M2 segments of the left middle cerebral artery (n = 1); diffuse thinning of the right internal carotid, middle cerebral, and right vertebral and basilar artery (n = 1); as a sequela, areas of focal gliosis in both the lateral ventricular and posterior periventricular regions (n = 1). Among these 3 patients, 1 had no neurologic complaints. Conclusion: Abnormal MRI findings can be observed in pediatric Takayasu arteritis patients, even those that are asymptomatic; therefore, clinicians should carefully evaluate neurologic involvement in all pediatric Takayasu arteritis patients.


2021 ◽  
Vol 10 (4) ◽  
pp. 887
Author(s):  
Guenther Schneider ◽  
Alexander Massmann ◽  
Peter Fries ◽  
Felix Frenzel ◽  
Arno Buecker ◽  
...  

Background. This paper aimed to prospectively evaluate the safety of embolization therapy of pulmonary arteriovenous malformations (PAVMs) for the detection of cerebral infarctions by pre- and post-interventional MRI. Method One hundred and five patients (male/female = 44/61; mean age 48.6+/−15.8; range 5–86) with pre-diagnosed PAVMs on contrast-enhanced MRA underwent embolization therapy. The number of PAVMs treated in each patient ranged from 1–8 PAVMs. Depending on the size and localization of the feeding arteries, either Nester-Coils or Amplatzer vascular plugs were used for embolization therapy. cMRI was performed immediately before, and at the 4 h and 3-month post-embolization therapy. Detection of peri-interventional cerebral emboli was performed via T2w and DWI sequences using three different b-values, with calculation of ADC maps. Results Embolization did not show any post-/peri-interventional, newly developed ischemic lesions in the brain. Only one patient who underwent re-embolization and was previously treated with tungsten coils that corroded over time showed newly developed, small, diffuse emboli in the post-interventional DWI sequence. This patient already had several episodes of brain emboli before re-treatment due to the corroded coils, and during treatment, when passing the corroded coils, experienced additional small, clinically inconspicuous brain emboli. However, this complication was anticipated but accepted, since the vessel had to be occluded distally. Conclusion Catheter-based embolization of PAVMs is a safe method for treatment and does not result in clinically inconspicuous cerebral ischemia, which was not demonstrated previously.


Medicina ◽  
2021 ◽  
Vol 57 (1) ◽  
pp. 50
Author(s):  
Jun-Ho Ha ◽  
Byeong-Ho Jeong

Foreign body (FB) aspiration occurs less frequently in adults than in children. Among the complications related to FB aspiration, pneumothorax is rarely reported in adults. Although the majority of FB aspiration cases can be diagnosed easily and accurately by using radiographs and bronchoscopy, some patients are misdiagnosed with endobronchial tumors. We describe a case of airway FB that mimicked an endobronchial tumor presenting with pneumothorax in an adult. A 77-year-old man was referred to our hospital due to pneumothorax and atelectasis of the right upper lobe caused by an endobronchial nodule. A chest tube was immediately inserted to decompress the pneumothorax. Chest computed tomography with contrast revealed an endobronchial nodule that was seen as contrast-enhanced. Flexible bronchoscopy was performed to biopsy the nodule. The bronchoscopy showed a yellow spherical nodule in the right upper lobar bronchus. Rat tooth forceps were used, because the lesion was too slippery to grasp with ellipsoid cup biopsy forceps. The whole nodule was extracted and was confirmed to be a FB, which was determined to be a green pea vegetable. After the procedure, the chest tube was removed, and the patient was discharged without any complications. This case highlights the importance of suspecting a FB as a cause of pneumothorax and presents the possibility of misdiagnosing an aspirated FB as an endobronchial tumor and selecting the appropriate instrument for removing an endobronchial FB.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii460-iii460
Author(s):  
Mayuko Miyata ◽  
Masahiro Nonaka ◽  
Akio Asai

Abstract BACKGROUND If new lesions are observed during follow-up of the malignant tumor after treatment, it is difficult to distinguish whether the tumor is a recurrent lesion, secondary cancer, or radiation necrosis of the brain. We have encountered a patient with symptomatic radiation necrosis of the cerebellum 16 years after treatment of medulloblastoma. CASE PRESENTATION: A 24-year-old man who had received a tumor resection and chemoradiotherapy for cerebellar medulloblastoma at the age of 8 presented with dizziness. For the past 16 years, there was no recurrence of the tumor. He subsequently underwent MRI scan, and T1-Gd image showed enhanced lesion in the right cerebellar peduncle. Cerebrospinal fluid cytology analysis was negative for tumor. We suspected tumor reccurence or secondary cancer, and performed lesion biopsy. The result of the pathological examination was radiation necrosis of the cerebellum. DISCUSSION: The interval of radiation necrosis of the brain and radiotherapy can vary from months to more than 10 years. So, whenever a new lesion is identified, radiation brain necrosis must be envisioned. According to guidelines in Japan, there is no absolute examination for discriminating tumor recurrence from radiation brain necrosis and diagnosis by biopsy may be required. CONCLUSION We experienced a case of symptomatic radiation necrosis of the cerebellum 16 years after treatment. In patients showing new lesion after long periods of time, the possibility of radiation necrosis to be considered.


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