scholarly journals Progressive Supranuclear Palsy with Wall-Eyed Bilateral Internuclear Ophthalmoplegia Syndrome: Authors’ Second Case

2019 ◽  
Vol 11 (2) ◽  
pp. 205-208 ◽  
Author(s):  
Hideyuki Matsumoto ◽  
Tatsurou Inaba ◽  
Toshiyuki Kakumoto ◽  
Ryoji Miyano ◽  
Naohiro Uchio ◽  
...  

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome has previously been reported in only 2 patients with progressive supranuclear palsy (PSP). Herein, we report a third case of WEBINO syndrome with PSP. The patient was an 81-year-old man who had experienced gradually increasing gait disturbance and occasional falls since the age of 78 years. At 80 years of age, he presented with cognitive impairment, parkinsonism, and oculomotor abnormalities. The oculomotor abnormalities consisted of vertical gaze palsy and loss of eye convergence. Brain magnetic resonance imaging demonstrated marked atrophy of the midbrain. He was diagnosed with PSP. At the age of 81 years, he presented with alternating extropia in his forward gaze and adduction paresis and outward nystagmus of the abducted eye in his horizontal gaze, both of which were compatible with WEBINO syndrome. Previously, we reported the first case of PSP with WEBINO syndrome, and another group recently reported a second case. In light of the previous cases and the present case, WEBINO syndrome in PSP should not be considered extremely rare. Furthermore, WEBINO syndrome has not been reported in other neurodegenerative disorders, which suggests that it might be a useful and specific diagnostic finding in PSP.

Neurosurgery ◽  
2002 ◽  
Vol 51 (5) ◽  
pp. 1290-1294 ◽  
Author(s):  
Samuel Tobias ◽  
Richard A. Prayson ◽  
Joung H. Lee

Abstract OBJECTIVE AND IMPORTANCE Necrotizing sarcoid granuloma (NSG) has been recognized as a histological variant of sarcoidosis. Two cases of neurosarcoidosis (NS) with NSG with concomitant systemic disease have been described previously. We present an unusual case of primary NS-NSG that resembled an en plaque cranial base meningioma. CLINICAL PRESENTATION A 51-year-old man presented with a 3-month history of progressive left visual deterioration and proptosis. Brain magnetic resonance imaging demonstrated a large cranial base lesion occupying the left anterior clinoid process and sphenoid wing, extending to the left frontotemporal convexity. A second dura-based lesion was observed in the right parietal convexity. Both lesions enhanced homogeneously after administration of intravenous contrast medium. Magnetic resonance imaging characteristics were consistent with a typical clinoidal meningioma with an en plaque extension laterally. INTERVENTION A left frontotemporal craniotomy with extradural removal of the anterior clinoid process was performed. When the dura was opened, a red fibrous mass was identified. Intraoperative histological analysis revealed the presence of necrotizing and noncaseating granulomas. Postoperatively, cerebrospinal fluid, erythrocyte sedimentation rate, C-reactive protein, and serum and cerebrospinal fluid angiotensin-converting enzyme values were normal. The search for acid-fast bacilli or fungi was negative. A diagnosis of primary NS-NSG was made. The patient began long-term high-dose corticosteroid therapy. One and a half years after surgery, his vision had improved significantly and the lesions were stable as revealed by magnetic resonance imaging. CONCLUSION The first case of primary NS-NSG in the absence of systemic sarcoidosis is reported. NS should be included in the differential diagnosis of dura-based lesions resembling meningioma.


Author(s):  
Julia Marian ◽  
Firdous Rizvi ◽  
Lily Q. Lew

AbstractNonketotic hyperglycemic chorea-ballism (NKHCB), also known as diabetic striato-pathy (DS) by some, is a rare complication of diabetes mellitus and uncommon in children. We report a case of a 10 11/12-year-old boy of Asian descent with uncontrolled type 1 diabetes mellitus (T1DM), Hashimoto's thyroiditis, and multiple food allergies presenting with bilateral chorea-ballism. His brain magnetic resonance imaging revealed developmental venous anomaly in right parietal lobe and right cerebellum, no focal lesions or abnormal enhancements. Choreiform movements resolved with correction of hyperglycemia. Children and adolescents with a movement disorder should be evaluated for diabetes mellitus, especially with increasing prevalence and insidious nature of T2DM associated with obesity.


2002 ◽  
Vol 97 ◽  
pp. 533-535 ◽  
Author(s):  
Jin Woo Chang ◽  
Jae Young Choi ◽  
Young Sul Yoon ◽  
Yong Gou Park ◽  
Sang Sup Chung

✓ The purpose of this paper was to present two cases of secondary trigeminal neuralgia (TN) with an unusual origin and lesion location. In two cases TN was caused by lesions along the course of the trigeminal nerve within the pons and adjacent to the fourth ventricle. Both cases presented with typical TN. Brain magnetic resonance imaging revealed linear or wedge-shaped lesions adjacent to the fourth ventricle, extending anterolaterally and lying along the pathway of the intraaxial trigeminal fibers. The involvement of the nucleus of the spinal trigeminal tract and of the principal sensory trigeminal nucleus with segmental demyelination are suggested as possible causes for trigeminal pain in these cases. It is postulated that these lesions are the result of an old viral neuritis. The patients underwent gamma knife radiosurgery and their clinical responses have been encouraging to date.


2021 ◽  
Vol 11 (7) ◽  
pp. 679
Author(s):  
Vincenzo Alfano ◽  
Mariachiara Longarzo ◽  
Giulia Mele ◽  
Marcello Esposito ◽  
Marco Aiello ◽  
...  

Apathy is a neuropsychiatric condition characterized by reduced motivation, initiative, and interest in daily life activities, and it is commonly reported in several neurodegenerative disorders. The study aims to investigate large-scale brain networks involved in apathy syndrome in patients with frontotemporal dementia (FTD) and Parkinson’s disease (PD) compared to a group of healthy controls (HC). The study sample includes a total of 60 subjects: 20 apathetic FTD and PD patients, 20 non apathetic FTD and PD patients, and 20 HC matched for age. Two disease-specific apathy-evaluation scales were used to measure the presence of apathy in FTD and PD patients; in the same day, a 3T brain magnetic resonance imaging (MRI) with structural and resting-state functional (fMRI) sequences was acquired. Differences in functional connectivity (FC) were assessed between apathetic and non-apathetic patients with and without primary clinical diagnosis revealed, using a whole-brain, seed-to-seed approach. A significant hypoconnectivity between apathetic patients (both FTD and PD) and HC was detected between left planum polare and both right pre- or post-central gyrus. Finally, to investigate whether such neural alterations were due to the underlying neurodegenerative pathology, we replicated the analysis by considering two independent patients’ samples (i.e., non-apathetic PD and FTD). In these groups, functional differences were no longer detected. These alterations may subtend the involvement of neural pathways implicated in a specific reduction of information/elaboration processing and motor outcome in apathetic patients.


NeuroSci ◽  
2020 ◽  
Vol 1 (2) ◽  
pp. 115-120
Author(s):  
Jacques De Reuck ◽  
Florent Auger ◽  
Nicolas Durieux ◽  
Claude-Alain Maurage ◽  
Vincent Deramecourt ◽  
...  

Introduction and Purpose: Cerebral amyloid angiopathy (CAA) can be observed in patients with progressive supranuclear palsy (PSP), though to a lesser degree than in Alzheimer’s disease. The present post-mortem 7.0-tesla magnetic resonance imaging (MRI) evaluates whether CAA has an influence on the degree of hippocampal atrophy (HA) and on the incidence of associated micro-infarcts (HMIs) and cortical micro-bleeds (HMBs). Material and Methods: Eight brains with PSP-CAA were compared to 20 PSP brains without CAA. In addition to the neuropathological examination, the hippocampus was evaluated on the most representative coronal section with T2 and T2*-weighted MRI sequences. The average degree of HA was determined in both groups. The incidence of HMIs and HMBs was also compared as well as the frequency of cortical micro-infarcts (CoMIs) and cortical micro-bleeds (CoMBs) in the hemispheric neocortex. Results: The neuropathological examination showed a higher incidence of lacunar infarcts in the PSP-CAA brains compared to the PSP ones. With magnetic resonance imaging (MRI), the severity of HA and the incidence of HMIs and HMBs was similar between both groups. Additionally, the frequency of CoMIs and CoMBs in the neocortex was comparable. Conclusions: The association of CAA in PSP brains has no influence on the degree of HA and on the incidence of the small cerebrovascular lesions in the hippocampus as well as in the neocortex.


2020 ◽  
Author(s):  
Adelaide Valluzzi ◽  
Salvatore Donatiello ◽  
Graziana Gallo ◽  
Monica Cellini ◽  
Antonino Maiorana ◽  
...  

AbstractOsteoid osteoma is a benign osteoblastic tumor, quite uncommon in the spine. We report a case of an osteoid osteoma involving the atlas in a 6-year-old boy, who presented with suboccipital pain and torticollis. Initial radiological findings were ambiguous as magnetic resonance imaging showed mainly edema of upper cervical soft tissues. The subsequent computed tomography depicted a lesion of left lamina of C1. As conservative treatment failed, the lesion was surgically resected and the patient became pain free. To our knowledge, this is the first case of osteoid osteoma involving the atlas associated with abnormal soft tissue reaction reported in literature.


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