1/33. Bilateral contemporaneous posteroventral pallidotomy for the treatment of Parkinson's disease: neuropsychological and neurological side effects. Report of four cases and review of the literature.The authors report the underestimated cognitive, mood, and behavioral complications in patients who have undergone bilateral contemporaneous pallidotomy, as seen in their early experience with functional neurosurgery for Parkinson's disease (PD) that is accompanied by severe motor fluctuations before pallidal stimulation. Four patients, not suffering from dementia, with advanced (Hoehn and Yahr Stages III-IV), medically untreatable PD featuring severe "on-off" fluctuations underwent bilateral contemporaneous posteroventral pallidotomy (PVP). All patients were evaluated according to the Core Assessment Program for Intracerebral Transplantations (CAPIT) protocol without positron emission tomography scans but with additional neuropsychological cognitive, mood, and behavior testing. For the first 3 to 6 months postoperatively, all patients showed a mean improvement of motor scores on the Unified Parkinson's Disease Rating Scale (UPDRS), in the best "on" (21%) and worst "off" (40%) UPDRS III motor subscale, a mean 30% improvement in the UPDRS II activities of daily living (ADL) subscore, and 60% on the UPDRS IV complications of treatment subscale. Dyskinesia disappeared almost completely, and the mean daily duration of the off time was reduced by an average of 60%. Despite these good results in the CAPIT scores, one patient experienced a partially regressive corticobulbar syndrome with dysphagia, dysarthria, and increased drooling. No emotional lability was found in this patient, but he did demonstrate severe bilateral postoperative pretarsal blepharospasm (apraxia of eyelid opening), which interfered with walking and which required treatment with high-dose subcutaneous injections of botulinum toxin. No patient showed visual field defects or hemiparesis, but postoperative depression, changes in personality, behavior, and executive functions were seen in two individuals. Postoperative abulia was reported by the family of one patient, who lost his preoperative aggressiveness and drive in terms of ADL, speech, business, family life, and hobbies, and became more sleepy and fatigued. One patient reported postoperative mental automatisms, such as compulsive mental counting, and circular thoughts and reasoning during off phases; postoperative depression was found in two patients. However, none of the patients demonstrated these symptoms during intraoperative microelectrode stimulation. These findings are compatible with previous reports on bilateral pallidal lesions. A progressive lowering of UPDRS subscores was seen after 12 months, consistent with the progression of the disease. Bilateral simultaneous pallidotomy may be followed by emotional, behavioral, and cognitive deficits such as depression, obsessive-compulsive disorders, and loss of psychic autoactivation-abulia, as well as disabling corticobulbar dysfunction and apraxia of eyelid opening, in addition to previously described motor and visual field deficits, which make this surgery undesirable even though significant improvement in motor deficits can be achieved.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
2/33. motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration.The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities. The predominant frontal-temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease-inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. His premorbid diagnosis was cortical-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
3/33. rehabilitation outcome in a patient awakened from prolonged coma.BACKGROUND: This article describes the rehabilitation of a patient recovering from a prolonged coma (defined as lasting longer than 4 weeks). The case is noteworthy because it exemplifies the possibilities and difficulties entailed in treating these patients, who are often regarded as too severely impaired to justify intensive rehabilitation efforts. CASE REPORT: The patient is a 28-year old Polish male, unmarried, who suffered serious closed head injuries in an automobile accident in April of 1999. He was in a comatose state for more than two months, with a GCS score of 5. When admitted for rehabilitation he was bedridden, with global aphasia, agraphia, limb apraxia, and executive dysfunction. The rehabilitation program developed for him is described in detail. RESULTS: Over the course of rehabilitation, which began in December 1999 and continues to this writing, the patient has regained locomotion capabilities (though with impairments), and his speech has improved considerably. The apraxia has largely resolved, and he is able to write his name and copy words. He is now capable of performing many activities of daily living. CONCLUSIONS: A comprehensive program of rehabilitation characterized by a strategic, heuristic approach is capable of achieving a good outcome even in very difficult cases, such as prolonged coma.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
4/33. Orthodontic treatment in handicapped children: report of four cases.Mentally and physically handicapped children show in the orofacial system motor-sensitivity disturbances and malocclusions of varying severity. These dysfunctions affect the breathing and speech ability and inhibit the food intake. Myotherapeutic exercises for strengthening of lip and tongue muscles and orthodontic treatment of the malocclusions help provide esthetic and functional improvements in these patients. The limited compliance necessitates a differentiated procedure during the diagnostic and therapeutic process and demands compromises in some cases.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
5/33. Early ictal speech and motor inhibition in fronto-mesial epileptic seizures: a polygraphic study in one patient.OBJECTIVE: To investigate ictal motor inhibition occurring during seizures in a patient with a tumor located in the left fronto-mesial pre-central cortex. methods: Awake and sleep video-polygraphic monitoring, recording scalp EEG and EMG activities from several cranial, trunk and limbs muscles, was performed in a patient with drug-resistant recurrent focal motor seizures before surgical treatment. speech/motor tasks were repeatedly administered to the patient during the recording sessions in order to evaluate the occurrence of early ictal motor inhibition. RESULTS: Thirty-four seizures were recorded during wakefulness showing a stereotyped pattern of inhibition of speech and voluntary movements followed by sequential activation of upper limb-trunk-lower limb muscles contralateral to the tumor. Polygraphic recordings showed that: (1) initial speech and motor arrest were associated with the EMG evidence of progressive muscle tone suppression in cranial and right distal upper limb muscles; (2) tonic contraction of right deltoid, biceps brachii, intercostalis and paraspinalis muscles appeared after motor inhibition; (3) tonic-clonic activity in the right tibialis anterior muscle occurred at the end of seizures. Eleven subclinical seizures were recorded during sleep showing mild focal tonic EMG activity in right side trunk muscles. CONCLUSIONS: Our findings evidenced early and somatotopically organized inhibition of voluntary movement at the beginning of epileptic seizures with fronto-mesial onset. The demonstration that speech and motor arrest were associated with progressive EMG suppression in cranial and limb muscles supports the hypothesis of motor inhibitory seizures originating in the mesial aspect of pre-motor frontal cortex.- - - - - - - - - - ranking = 7keywords = speech (Clic here for more details about this article) |
6/33. A novel movement disorder of the lower lip.Four patients, aged 25 to 42 years presented with acute onset of a movement disorder characterized by a tonic, sustained, lateral and outward protrusion of one half of the lower lip. The movement disorder was present at rest, while in some patients, it was also present during speech. In all cases, the abnormal lip posture could be suppressed voluntarily. Neurological examination was otherwise normal. Extensive laboratory investigation failed to reveal any causative factors for secondary focal dystonia. Treatment with oral medications and botulinum toxin was mostly ineffective. Spontaneous remissions were frequent.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
7/33. stuttering and gait disturbance after supplementary motor area seizure.Acquired stuttering is an uncommon speech disorder. Supplementary motor area (SMA) lesions have been reported to be directly or indirectly related to acquired stuttering and various types of motor dysfunction. We report on a patient who presented with both acquired stuttering and long-lasting gait disturbance after SMA seizure.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
8/33. Parkinsonism following bilateral lesions of the globus pallidus: performance on a variety of motor tasks shows similarities with Parkinson's disease.OBJECTIVES: The authors report the results of detailed investigations into the motor function of a patient who, after a heavy drinking binge and subsequent unconsciousness, respiratory acidosis, and initial recovery, developed parkinsonism characterised by hypophonic speech and palilalia, "fast micrographia", impaired postural reflexes, and brady/akinesia in proximal (but not distal) alternating upper limb movements. methods: In addition to brain magnetic resonance imaging (MRI), different aspects of motor function were investigated using reaction time (RT) tasks, pegboard and finger tapping tasks, flex and squeeze tasks, movement related cortical potentials (MRCPs), and contingent negative variation (CNV). Cognitive function was also assessed. The results were compared to those previously reported in patients with Parkinson's disease (PD). RESULTS: brain MRI showed isolated and bilateral globus pallidus (GP) lesions covering mainly the external parts (GPe). These lesions were most probably secondary to respiratory acidosis, as other investigations failed to reveal an alternative cause. The results of the RT tasks showed that the patient had difficulties in preparing and maintaining preparation for a forthcoming movement. MRCP and CNV studies were in line with this, as the early component of the MRCP and CNV were absent prior to movement. The patient's performance on pegboard and finger tapping, and flex and squeeze tasks was normal when performed with one hand, but clearly deteriorated when using both hands simultaneously or sequentially. CONCLUSIONS: In general, the present results were similar to those reported previously in patients with PD. This provides further indirect evidence that the output of globus pallidus is of major importance in abnormal motor function in PD. The possible similarities of the functional status of GP in PD and our case are discussed.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
9/33. Cerebral folate deficiency: life-changing supplementation with folinic acid.Cerebral folate deficiency is characterized by low cerebrospinal fluid (CSF) concentrations of 5-methyltetrahydrofolate and a broad spectrum of clinical signs and symptoms. A patient with progressive spasticity, gait disturbance, speech difficulties, initially diagnosed as a recessive spastic paraplegia recovered on folinic acid (15-30 mg/day) and her 5-methyltetrahydrofolate in CSF normalized. This report demonstrates the importance of CSF investigation in the diagnosis of cerebral folate deficiency and efficiency of folinic acid (5-formyltetrahydrofolate) supplementation.- - - - - - - - - - ranking = 1keywords = speech (Clic here for more details about this article) |
10/33. Influence of motor disorders on the visual perception of human movements in a case of peripheral dysgraphia.We report the case of a 71 year-old female patient (DC) with a left parietal lesion resulting in a peripheral dysgraphia essentially characterized by difficulties in letter sequences writing. The aim of our experiments was to analyze the influence of motor difficulties on the visual perception of both writing and reaching movements. Results showed a strong link between motor and perceptual performance. For reaching movements, performances in both production and perception tasks conform to the motor principles identified in healthy subjects (Fitts' law and motor anticipation).By contrast, for handwriting movements, DC's productions do not follow the motor principles usually observed in normal subjects (isochrony principle, motor anticipation) and in perception the same results were observed. The motor references used by DC in the visual perception of writing movement were not the laws of movement but rather her own way of writing. Taken together these data strongly suggest that motor competences is involved in the visual perception of human movements. They are discussed in the general framework of the simulation theory.- - - - - - - - - - ranking = 0.92296566242587keywords = perception (Clic here for more details about this article) |
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