1/19. dysarthria, progressive parkinsonian features and symmetric necrosis of putamen in a family with painful lipomas (Dercum disease variant).We describe painful subcutaneous lipomatosis in four members of a two-generation family. Lipomas appeared in adulthood, were circumscribed, painful on touch and mainly localized to the trunk and proximal parts of the extremities. The disorder was associated with dysarthria, visual pursuit defect and progressive dystonia. MRI showed bilateral increasing cystic lesions in the basal parts of the putamen. No other abnormalities were detected. The lesions corresponded well with the clinical presentation in the patients. Investigation for mitochondrial disease with muscle biopsy and mitochondrial dna gave normal results. No consistent biochemical changes were found. The disorder in this family was considered to differ from MERRF with lipomatous lesions and multiple symmetric lipomatosis but compatible with a Dercum disease variant.- - - - - - - - - - ranking = 1keywords = parkinsonian (Clic here for more details about this article) |
2/19. Adverse effects of subthalamic nucleus DBS in a patient with multiple system atrophy.A 59-year-old woman with levodopa-responsive parkinsonism complicated by motor fluctuations and generalized levodopa dyskinesia underwent bilateral subthalamic deep brain stimulation (STN DBS) 7 years after symptom onset. DBS improved levodopa-responsive upper extremity bradykinesia but aggravated speech, swallowing, and gait. Motor fluctuations were not improved and levodopa dose remained unchanged. pulse generators were turned off. Clinical features and brain MRI in this case were indicative of multiple system atrophy (MSA). STN DBS is not recommended for patients with MSA.- - - - - - - - - - ranking = 0.21223590193786keywords = parkinsonism (Clic here for more details about this article) |
3/19. "Moebius syndrome": a case report.Moebius syndrome is an extremely rare disorder characterized by a lifetime facial paralysis, involving sixth and seventh cranial nerves with malformations of orofacial structures and the limbs. In this case, an 8 year old girl with Moebius syndrome is presented, clinical findings are described and management aspects are discussed. Early dental evaluation and parental counselling as a part of preventive dental regimen can go a long way in providing complete psychosocial rehabilitation for such physically disabled children.- - - - - - - - - - ranking = 0.00015288111714732keywords = paralysis (Clic here for more details about this article) |
4/19. Bilateral subthalamic nucleus deep brain stimulation in a patient with cervical dystonia and essential tremor.The role of subthalamic nucleus (STN) deep brain stimulation (DBS) is well established in Parkinson's disease, but experience with STN DBS in other movement disorders is limited. We report on a patient with medically refractory cervical dystonia and essential tremor resulting in dystonic head tremor and action tremor of the hands who obtained complete tremor suppression and near resolution of her cervical dystonia with bilateral STN stimulation. The results in this case demonstrate that STN DBS can dramatically improve dystonia and tremor in nonparkinsonian movement disorders.- - - - - - - - - - ranking = 0.25keywords = parkinsonian (Clic here for more details about this article) |
5/19. Changes induced by levodopa and subthalamic nucleus stimulation on parkinsonian speech.levodopa (L-dopa) and subthalamic nucleus (STN) stimulation treatments have been associated with both improvement and exacerbation of dysarthria in Parkinson's disease (PD). We report four cases illustrating variant responses of dysarthria to dopaminergic and STN stimulation therapies. patients' motor disability and dysarthria were perceptually rated by the Unified Parkinson's disease Rating Scale (UPDRS) in four conditions according to medication and STN stimulation. Dedicated software packages allowed acquisition and analysis of acoustic recordings. Case 1, who had a severe off period aphonia, experienced improvement of speech induced by both levodopa and STN stimulation. In Case 2, both treatments worsened speech due to the appearance of dyskinesias. Case 3 had a dysarthria exacerbation induced by STN stimulation with parameters above optimal levels, interpreted as current diffusion from the STN to corticobulbar fibers. In Case 4, dysarthria exacerbation occurred with stimulation at an electrode contact located caudally to the target, also arguing for current diffusion as a potential mechanism of speech worsening. The presented cases demonstrated variant effects in relation to L-dopa and STN stimulation on speech. It seems that motor speech subcomponents can be improved like other limb motor aspect, but that complex coordination of all speech anatomical substrates is not responsive to STN stimulation. These hypotheses may be helpful for better understanding and management of STN stimulation effects on motor speech and skeleton-motor subsystems.- - - - - - - - - - ranking = 1keywords = parkinsonian (Clic here for more details about this article) |
6/19. Sporadic rapid-onset dystonia-parkinsonism presenting as Parkinson's disease.We report on a 38-year-old patient with rapid-onset dystonia-parkinsonism (RDP) with a missense mutation in the Na/K-ATPase alpha3 subunit (ATP1A3). Asymmetrical parkinsonian symptoms evolved over a year. After a stable episode of another 2.5 years, overnight he developed oromandibular dystonia and more severe parkinsonian symptoms. We conclude that RDP should be considered as a rare cause of levodopa-unresponsive parkinsonism even if there is no family history and the classic presentation is lacking.- - - - - - - - - - ranking = 1.7734154116271keywords = parkinsonian, parkinsonism (Clic here for more details about this article) |
7/19. manganese-induced Parkinsonism in a patient undergoing maintenance hemodialysis.We report a rare case of manganese (Mn)-induced parkinsonism in a patient on maintenance hemodialysis therapy who complained of gait disturbance and dysarthria. His symptoms and abnormal magnetic resonance imaging (MRI) findings of the brain were thought to be caused, at least in part, by long-term ingestion of a health supplement (chlorella extract) that contained 1.7 mg of Mn in the usual daily dose. Elevated serum and cerebrospinal fluid Mn levels were detected, and brain MRI showed areas of abnormal intensity in the bilateral basal ganglia (low intensity on T1-weighted images and high intensity on T2-weighted images). edetic acid infusion therapy dramatically improved the MRI abnormalities, after which his symptoms gradually improved 4 months later.- - - - - - - - - - ranking = 0.21223590193786keywords = parkinsonism (Clic here for more details about this article) |
8/19. Progressive anarthria with secondary parkinsonism: a clinico-pathological case report.The pathological process and lesion topography in patients with the syndrome of progressive aphasia are heterogeneous and few necropsy examination cases have been investigated. This is a case report of a 53 year old right handed man with progressive anarthria and secondary Parkinsonism over a period of six years. Positron emission tomography (PET) showed a decreased cerebral blood flow and metabolism in the frontal cortex, which was more pronounced on the left. Neuropathology disclosed a spongiform vacuolation in layer II of the frontal cortex, mostly in the Broca area, and neuronal loss in the substantia nigra. This original case reinforces the view that there are different entities of the syndrome of progressive aphasia which can be identified on the basis of clinical, neuroimaging and anatomical data.- - - - - - - - - - ranking = 0.84894360775142keywords = parkinsonism (Clic here for more details about this article) |
9/19. Traumas of the middle skull base with TMJ involvement. Case report.The authors report their experience with temporomandibular joint (TMJ) traumas involving breakage of the roof of the glenoid cavity, an infrequent event that occurs in those cases in which, as a result of the condylar neck not fracturing, the traumatic energy is transmitted to the middle skull base. As the literature contains no valid series for establishing standardized protocols for the treatment of these fractures, we propose our own orthopedic-functional approach. The patient observed by us had suffered a cranio-facial trauma and presented the classical symptoms and signs of TMJ traumas and complete bilateral Bell paralysis. He was subjected to a CAT scan and then to 2-stage treatment consisting of functional rest with liquid diet followed by physiotherapy. An almost total recovery in TMJ function was observed after 1 month. At 1-year follow-up the facial paralysis had resolved completely. On the basis of our experience, breakages of the glenoid cavity can be compared, in terms of treatment procedure, to intracapsular fractures of the TMJ with surgery confined to cases of ankylosis sequelae. To avoid the onset of ankylosis careful control of clinical, functional and radiological follow-up is required.- - - - - - - - - - ranking = 0.00030576223429464keywords = paralysis (Clic here for more details about this article) |
10/19. A case of frontotemporal lobar degeneration with progressive dysarthria.We investigated the evolution of the neurological and neuropsychological characteristics in a right-handed woman who was 53-years-old at the onset and who showed personality changes and behavioral disorders accompanied by progressive dysarthria. She had hypernasality and a slow rate of speech with distorted consonants and vowels, which progressed as motor disturbances affecting her speech apparatus increased; finally, she became mute two years post onset. Her dysarthria due to bilateral voluntary facio-velo-linguo-pharyngeal paralysis accompanied with automatic-voluntary dissociation fit the description of anterior opercular syndrome. She showed personality changes and behavioral abnormalities from the initial stage of the disease, as is generally observed in frontotemporal degeneration (FTD), and her magnetic resonance image showed progressive atrophy in the frontotemporal lobes; thus, she was clinically diagnosed with FTLD. This patient's symptoms suggest that FTLD, including bilateral anterior operculum degeneration, causes progressive pseudobulbar paretic dysarthria accompanied by clinical symptoms of FTD, which raises the possibility of a new clinical subtype in the FTLD spectrum.- - - - - - - - - - ranking = 0.00015288111714732keywords = paralysis (Clic here for more details about this article) |
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