1/263. akinetic mutism after fourth ventricle choroid plexus papilloma: treatment with a dopamine agonist.BACKGROUND: akinetic mutism is a behavioral state wherein a patient seems to be awake but does not move or speak. Several patients are reported to have developed mutism after posterior fossa surgery. We present a patient who developed akinetic mutism after total excision of a choroid plexus papilloma of the fourth ventricle, and who was treated with bromocriptine. CASE DESCRIPTION: An 18-year-old woman was admitted with akinetic mutism, which had developed 6 days after posterior fossa surgery. She had had no neurologic deficit in the first 5 days after surgery and could communicate with her family. Despite antioedematous therapy and daily lumbar punctures to drain cerebrospinal fluid, there was no clinical improvement after she entered the akinetic mute state. brain magnetic resonance revealed ventriculomegaly; brain single photon emission computed tomography revealed bilateral reduction of perfusion in the frontal region. Because daily lumbar drainage did not result in clinical improvement, shunt placement was not considered. bromocriptine therapy was begun at a dose of 2x2.5 mg; 24 hours later, the patient started to speak and move her upper extremities. Further improvement occurred over the following week when the dose was increased to 3x2.5 mg. bromocriptine was replaced with a placebo to determine whether the neurologic improvement was caused by the medicine. The patient's neurologic status deteriorated progressively; therefore, bromocriptine was restarted and she was discharged from the hospital. During the 6 months of follow-up, the patient has remained in good health. CONCLUSIONS: The etiology of akinetic mutism is not clear. Monoaminergic pathways, particularly dopaminergic cell groups, are most probably involved in this syndrome, because bromocriptine has a dramatic effect on these patients, as demonstrated in our case.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
2/263. Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease.We report a pair of monozygotic Huntington's disease (HD) twins who, although sharing identical CAG repeat lengths, not only present with marked differences in clinical symptoms but also behavioral abilities as measured by our experimental procedures. Both HD twins and two healthy control subjects were tested twice over 2 years. Patient A was generally more impaired at a motor level, whereas Patient B showed greater attentional impairment; Patient B, however, showed more progressive deterioration. The control subjects' performance remained consistent over the 2-year interval. Patient A clinically had the more hyperkinetic hypotonic variant of the disease, whereas Patient B, who was the more impaired, presented with a more hypokinetic hypertonic (rigid) variant. The influences of epigenetic pre- and postnatal environmental factors should not be ignored.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
3/263. Hyperekplexia in a patient with a brainstem vascular anomaly.OBJECTIVES: To describe a patient with a clinical picture suggestive of idiopathic hyperekplexia (IH), who was later found to harbour a subtle brainstem vascular anomaly. PATIENT: A 35-year-old man, 4 years earlier, developed sudden jumping and falling in response to unexpected sensory stimuli. RESULTS: Neurological examination was normal. electromyography showed an excessively large and non-habituating motor startle response. There were no mutations of the alpha1 subunit of the inhibitory glycine receptor which cause hereditary hyperekplexia. Although all these findings were consistent with a diagnosis of IH, a blink reflex study showed an enhanced recovery curve suggestive of a brainstem lesion. A detailed MRI study revealed a subtle vascular anomaly involving the lower brainstem. CONCLUSION: This is the first report of sporadic hyperekplexia related to a brainstem vascular anomaly. Subtle damage to the brainstem should always be excluded in patients with sporadic hyperekplexia, regardless of the coexistence of additional clear-cut neurological symptoms.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
4/263. poems syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma.poems syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of poems syndrome as a paraneoplastic syndrome in myeloma patients.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
5/263. 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 = 3keywords = ms (Clic here for more details about this article) |
6/263. Cerebral arteriovenous malformations and movement disorders.A series of six patients with movement disorders associated with cerebral arteriovenous malformations (AVM) is reported. The AVMs were classified according to the Spetzler-Martin classification as grade V (one patient), grade IV (four patients), and as grade III (one patient). One patient had action-induced hemidystonia caused by a contralateral frontoparietal AVM which compressed the putamen and was supplied partially by enlarged lenticulostriate arteries. Two patients presented with unilateral cortical tremor associated with contralateral high-frontal cortical/subcortical AVMs sparing the basal ganglia. Another patient developed hemidystonia and hemichorea-hemiballism after bleeding of a contralateral temporooccipital AVM and subsequent ischemia. Two patients had focal dystonia after thalamic and basal ganglia hemorrhage from AVMs. Five patients were operated on. The movement disorder was abolished in one patient postoperatively. Different mechanisms were identified that are relevant for the development of AVM-related movement disorders: mass effect, diaschisis, local parenchymal altered cerebral blood flow, and hemorrhagic or ischemic structural lesions.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
7/263. Interarticulator co-ordination in deaf signers with Parkinson's disease.Motor control deficits in signers with Parkinson's disease (PD) were examined through analysis of their production of American sign language (ASL) fingerspelling, which is sequential and rapid motor behavior that has theoretical models of its underlying structure. Free conversation of two Deaf signers with PD and two Deaf control signers was analysed. In addition, scripted productions of one control signer were also analysed and directly compared to the same productions by the signers with PD. A featural analysis of ASL fingerspelling and a frame-by-frame analysis of multiple articulator movements were used to examine the fingerspelled productions. On the basis of the featural analysis, the signers with PD showed a variety of error patterns, all of which reflected attempts to reduce the motoric demands of coarticulation and thereby facilitate ease of articulation. Signers with PD either held individual segments in a fingerspelling sequence for a long time (segmentation), blended adjacent segments into a single segment (sequential blending), or broke handshapes down sequentially into their component features (featural unraveling). The results of both the featural analysis and the frame-by-frame analysis show that the PD signers have difficulty co-ordinating the movements of independent articulators in complex sequences. For example, the movements of independent articulators for fingerspelling (the thumb, fingers, and wrist) were markedly farther apart in time and more variable for the signers with PD. In addition, the signers with PD used fewer wrist movements while fingerspelling. Such deficits are consistent with claims that patients with PD are impaired in their ability to use ongoing sensorimotor information to program multi-articulator movements.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
8/263. A video review of the diagnosis of psychogenic gait: appendix and commentary.The gait and other clinical features of 22 patients presenting to our hospital over the last 10 years are shown on video. In 12 patients, a diagnosis of psychogenic gait was made; in the remainder, the gait abnormality was the result of a neurologic disease. Psychogenic gaits are compared and contrasted with "organic" gaits. In one patient, the psychogenic gait occurred in the setting of a neurologic disease. The "traditional" approach to psychogenic gait, attempting to exclude underlying neurologic and psychiatric disease and seeking evidence for primary and secondary gain, was found to be of limited value. More useful were the features of the gait itself, in particular, exaggerated effort, extreme slowness, variability throughout the day, unusual or uneconomic postures, collapses, convulsive tremors, and distractibility; certain aspects of the history were also helpful. A list of comments is provided. The diagnosis of psychogenic gait, particularly in the elderly, remains fraught with hazard, and a balance has to be sought between subjecting an anxious patient to needless investigations and yet not losing sight of the fact that the patient may be elaborating on symptoms of genuine disease. The bizarre gait of some neurologic disorders, particularly dystonia and chorea, may be a pitfall for the unwary.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
9/263. Treating severe bruxism with botulinum toxin.BACKGROUND: Locally administered botulinum toxin, or BTX, is an effective treatment for various movement disorders. Its usefulness in treating bruxism, however, has not been systematically evaluated. SUBJECTS AND methods: The authors studied 18 subjects with severe bruxism and whose mean duration of symptoms was 14.8 /- 10.0 years (range three-40 years). These subjects audibly ground their teeth and experienced tooth wear and difficulty speaking, swallowing or chewing. Medical or dental procedures had failed to alleviate their symptoms. The authors administered a total of 241 injections of BTX type A, or BTX A, in the subjects' masseter muscles during 123 treatment visits. The mean dose of the BTX A was 61.7 /- 11.1 mouse units, or MU (range 25-100 MU), per side for the masseter muscles. RESULTS: The mean total duration of response was 19.1 /- 17.0 weeks (range six-78 weeks), and the mean peak effect on a scale of 0 to 4, in which 4 is equal to total abolishment of grinding, was 3.4 /- 0.9. Only one subject (5.6 percent) reported having experienced dysphagia with BTX A. CONCLUSION: The results of this study suggest that BTX administered by skilled practitioners is a safe and effective treatment for people with severe bruxism, particularly those with associated movement disorders. It should be considered only for those patients refractory to conventional therapy. Future placebo-controlled studies may be useful in further evaluating the potential of BTX in the treatment of bruxism.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
10/263. alien hand syndrome: report of two cases.alien hand syndrome (AHS) refers to the occurrence of apparently purposeful movements in the hand which are independent of volitional control. Two subtypes of AHS have been proposed: frontal AHS, with grasp reflex and compulsive manipulation of tools by the dominant hand, and callosal AHS, which occurs in the nondominant hand and is characterized mainly by intermanual conflict. Here, we report two cases of frontal-type alien hand syndrome with symptoms of reflexive grasping, impulsive groping, and apraxia (in case 1), and compulsive manipulation of tools (in case 2). brain computed tomography revealed a left anterior cerebral artery (ACA) territory infarct and multiple small infarcts of both hemispheres in patient 1 and a left ACA infarct in patient 2. The involuntary movements were bothersome to these patients in their daily activities. Both patients attended conventional physical and occupational therapies, and patient 2 received additional biofeedback training. follow-up studies showed the spontaneous grasping behavior was still present in patient 1 and AHS had subsided in patient 2. We also describe a potentially effective technique involving biofeedback for patients with alien hand syndrome.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
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