Cases reported "Movement Disorders"

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1/87. 18Fluoro-2-deoxyglucose (18FDG) PET scan of the brain in type IV 3-methylglutaconic aciduria: clinical and MRI correlations.

    The clinical, 18fluorodeoxyglucose positron emission tomography (18FDG PET) and the magnetic resonance imaging (MRI) brain scan characteristics of four patients diagnosed to have 3-methylglutaconic aciduria were reviewed retrospectively. The disease has a characteristic clinical pattern. The initial presentations were developmental delay, hypotonia, and severe failure to thrive. Later, progressive encephalopathy with rigidity and quadriparesis were observed, followed by severe dystonia and choreoathetosis. Finally, the patients became severely demented and bedridden. The 18FDG PET scans showed progressive disease, explaining the neurological status. It could be classified into three stages. Stage I: absent 18FDG uptake in the heads of the caudate, mild decreased thalamic and cerebellar metabolism. Stage II: absent uptake in the anterior half and posterior quarter of the putamina, mild-moderate decreased uptake in the cerebral cortex more prominently in the parieto-temporal lobes. Progressive decreased thalamic and cerebellar uptake. Stage III: absent uptake in the putamina and severe decreased cortical uptake consistent with brain atrophy and further decrease uptake in the cerebellum. The presence of both structural and functional changes in the brain, demonstrated by the combined use of MRI and 18FDG PET scan, with good clinical correlation, make the two techniques complementary in the imaging evaluation of 3-methylglutaconic aciduria.
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2/87. 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.
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keywords = visual
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3/87. magnetic resonance imaging findings in corticobasal degeneration.

    Two women (patient 1, 77 years old, and patient 2, 63 years old) with strong clinical evidence for corticobasal degeneration (CBD) are presented. Patient 2 was in an early stage of the disease with only a mild disability of her left hand. In addition to the clinical characteristics, both patients presented the typical cortical reflex myoclonus. magnetic resonance imaging studies for both patients revealed nearly identical hyperintense lesions somatotopic from the left-hand primary motor cortex (M1), extending to the midline and possibly supplementary motor area (SMA) in patient 2. To our knowledge, this has not been previously described in patients with CBD. These lesions may play a role in the etiology and the development of CBD with involvement of the M1 and may correspond to the underlying pathology of demyelination or gliosis.
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4/87. Leptomeningeal melanoma and Creutzfeldt-Jakob disease in a patient with chronic lymphocytic leukaemia.

    A 78-year-old woman with known chronic lymphocytic leukaemia (CLL) was admitted to a psychiatric unit because of rapidly declining cognitive function. Clinical examination also revealed cerebellar signs and she later became akinetic and mute. She deteriorated and died of bronchopneumonia. The histology from the post-mortem confirmed the presence of CLL in the lymph nodes and she was also found to have diffuse leptomeningeal melanoma. In addition, there was extensive prion protein deposition in the cerebral cortex, but without significant spongiosis. The astrocytosis that was present appeared superficial only. Furthermore, prion protein appeared to be co-expressed with betaA4 in the form of plaques. The patient therefore had evidence of sporadic Creutzfeldt-Jakob disease (CJD) in addition to meningeal melanoma and CLL. This case further illustrates the importance of employing prion protein immunohistochemistry in suspected cases of CJD, especially where the histology is atypical.
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5/87. Patterns of motor control reorganization in a patient with mirror movements.

    OBJECTIVE: To explore motor control reorganization in a 40-year-old, left-handed patient with perinatally acquired mirror movements. methods: We performed simultaneous bilateral recordings of motor evoked potentials (MEPs) following focal transcranial magnetic stimulation (fTMS) and of central silent period (cSP) during unilateral voluntary contraction in abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. RESULTS: For both muscles the MEP study showed bilateral fast-conducting corticospinal projections from the right undamaged hemisphere, and residual contralateral projections from the left hemisphere. The cSP findings differed in the two muscles: the mirror phenomenon was bilateral in the ADM, but present only on the right side in the APB muscles; the mirror activity of right ADM and APB muscles was inhibited only by fTMS of the ipsilateral right motor cortex; the mirror phenomenon in the left ADM muscle was inhibited only by fTMS of the contralateral right motor cortex. CONCLUSIONS: Mirror movements of right APB and ADM muscles were sustained by the ipsilateral connections from the undamaged motor cortex, while the mirror phenomenon in the left ADM muscle could be explained by hypothesizing a bilateral activation of motor cortices. This previously unreported electrophysiological picture demonstrates that different patterns of motor control may realize after perinatal cerebral lesions, even in different distal muscles of the same patient.
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keywords = cortex
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6/87. February 2000: dementia with motor dysfunction in a patient with liver disease.

    Acquired (non-Wilsonian) hepatocerebral degeneration (AHCD) is an irreversible neurological condition characterized by dementia, dysarthria, and motor disturbances. It has been described in patients with severe liver disease of many causes, and notably in patients with surgically or spontaneously created porto-systemic shunts. We report a case of AHCD in a patient with end-stage liver disease due to alcohol abuse and hepatitis c. In addition, this patient showed pathologic evidence of the less commonly reported "shunt myelopathy" in the absence of a surgically created porto-systemic shunt. The myelopathy was associated with a dramatic vacuolation involving especially the deep motor cortex. Electron microscopy suggested that the vacuolation was due mainly to disruption of abnormal astrocytes.
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7/87. Involuntary masturbation as a manifestation of stroke-related alien hand syndrome.

    alien hand syndrome is a perplexing and uncommon clinical diagnosis. We report an unusual manifestation of alien hand syndrome in a 73-yr-old man with a right anterior cerebral artery infarct affecting the right medial frontal cortex and the anterior portion of the corpus callosum. We conclude that alien hand syndrome should be considered in patients who present with a feeling of alienation of one or both upper limbs accompanied by complex purposeful involuntary movement.
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keywords = cortex
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8/87. Post operative supplementary motor area syndrome: clinical features and outcome.

    The syndrome of the supplementary motor area (SMA) is not well recognized and its features can easily be confused with pyramidal weakness. The authors describe the SMA syndrome in six patients who underwent surgery for tumours located in the SMA, three in the dominant and three in the non-dominant hemispheres. All of them underwent complete resection of the anatomically described SMA, with partial (n = 4) or total resection (n = 2) of the tumour. In the postoperative period, all these patients exhibited reduction of spontaneous movements and difficulty in performing voluntary motor acts to command in the contralateral limbs, although the tone in the limbs was maintained or increased. The function of these limbs in serial automatic motor activities (for example, dressing and walking) was, however, relatively unaffected. speech deficits were seen in only one of three patients with the dominant SMA syndrome. Besides a severe impairment of volitional movements, the salient features of the deficits in this syndrome are hemineglect and dyspraxia or apraxia involving the contralateral limbs. All patients recovered their motor functions over varying periods of time ranging from one to a few weeks. Long-term follow-up (median 24 months) in five patients revealed complete return of function in the affected limbs. It is important to recognize the entity of the SMA syndrome and differentiate it from the deficits that result from operative damage to the motor cortex as the deficits associated with the former are likely to recover almost completely over a short period of time.
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keywords = cortex
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9/87. Bilateral painful hand-moving fingers: electrophysiological assessment of the central nervous system oscillator.

    We describe a 35-year-old woman who presented with the syndrome of painful hand-moving fingers on the right side. Eight months later, she developed similar finger movements and hand discomfort on the left side. She had a history of hand trauma and recurrent shoulder dislocation on the right side. Kinesiologic electromyography suggested a common central oscillator for finger movements in both hands. Electrophysiological assessment of spinal alpha motor neuron excitability, reciprocal inhibition, and Renshaw cell inhibition failed to show any abnormalities. Somatosensory evoked potential test showed marked attenuation of N20 potential recorded from the left somatosensory cortex; paired transcortical magnetic stimulation of the left motor cortex suggested failure of cortical facilitation. The data suggest that the central oscillator responsible for finger movements is located above the spinal cord level in this patient.
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ranking = 2
keywords = cortex
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10/87. Reorganisation of the sensorimotor cortex after early focal brain lesion: a functional MRI study in monozygotic twins.

    Sensorimotor cortical reorganization after early brain lesions was studied by means of fMRI in two pairs of monozygotic twins, in each of which one member had a focal brain injury. This offered a unique opportunity to reduce the wide intersubject variability of the controls often found in similar studies. Activation images were acquired during a motor task (sequential opposition finger movements) and a sensory task (passive brushing of palm and fingers). During the tasks with the recovered hand, constant findings in the lesioned subjects were the activation of the undamaged areas adjacent to lesion site and the activation of the ipsilateral sensorimotor cortex. Bilateral activation of the primary sensorimotor cortex was never observed in the healthy co-twin controls.
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ranking = 6
keywords = cortex
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