Cases reported "Movement Disorders"

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11/37. Hemiballismus following general anesthesia. A case report.

    Hemiballismus is characterized by the abrupt onset of violent proximal flinging movements, affecting the limbs, neck and trunk on one side of the body. It is caused by the lesion in the region of the contralateral subthalamic nucleus of the Luys. Usually it is a self-limiting disease, lasting 6-8 weeks. A 49-year-old man has been admitted to the hospital after flinging movements of his right arm and the right side of the trunk occurred. A few days earlier he had undergone general anesthesia prior to a dental procedure. There was trouble in waking the patient afterwards. The movements lasted a few days. MRI of the brain revealed ischemic lesions areas in T2-weighted images localized in the region of globus pallidus bilaterally. EEG was abnormal, and showed slowed background activity with slow waves in left temporal lobe. He was treated with haloperidol, clonazepam and vasoactive medications. In spite of administered treatment, hemiballic movements reappeared occasionally. Due to increased frequency of the movements the patient was hospitalized again two years later. The second MRI revealed changes described earlier and a new ischaemic focus in left parietal lobe. Continuation of treatment with haloperidol was administered.
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12/37. EEG characteristics in juvenile Huntington's disease: a case report and review of the literature.

    The clinical features of Juvenile Huntington's disease (J-HD) differ from those of the more common adult-onset form, and include cognitive decline, parkinsonism, myoclonus and seizures. A paucity of literature is available describing the electroencephalographic (EEG) findings. We describe the clinical and EEG characteristics of a patient with genetically confirmed J-HD. A review of previously published cases yielded EEG descriptions in only 23 patients whose disease onset was prior to the age of 32, and only 14 of these were prior to the age of 20. Epileptiform abnormalities were noted in 17 (74%), which was considerably more common than in the adult form. Generalized discharges were noted in nine, with six having polyspike and wave. The remainder had focal or multifocal epileptiform discharges. With genetic testing now available, refinement of the EEG data will be possible.
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13/37. Bobble-head doll syndrome successfully treated with an endoscopic ventriculocystocisternostomy. Case report and review of the literature.

    The bobble-head doll syndrome (BHDS) is characterized by a back-and-forth movement of the head with a frequency of 2 to 3 Hz, which increases during walking and excitement and decreases during concentration. The head movements are accompanied by macrocephaly, ocular disturbances, psychomotor retardation, and sometimes endocrine dysfunction. The BHDS is frequently associated with a suprasellar arachnoid cyst. The authors present the case of a 4-year-old patient with BHDS; an endoscopic cystoventriculostomy was performed by fenestrating a cyst in the suprasellar region. After wide fenestration of the cyst wall that was protruding and obstructing the foramen of Monro, the cyst was entered with the endoscope and a small, natural, valvelike communication of the cyst with the basal prepontine cistern was seen close to the basilar artery. This communication was widened by balloon dilation. After completion of the ventriculocystocisternostomy, the cyst collapsed and the obstruction of the aqueduct was resolved. In view of the source mechanism and cerebrospinal fluid dynamics of the suprasellar arachnoid cyst, a ventriculocystocisternostomy is an important treatment option for BHDS arising from a suprasellar cyst. Three years after treatment, the head bobbing had resolved completely and psychomotor development was improving. Delay of diagnosis and treatment of this condition can cause permanent neurological dysfunction and psychomotor retardation. The authors recommend early ventriculocystocisternostomy as a physiologically based treatment for BHDS originating from a suprasellar cyst.
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keywords = frequency
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14/37. tongue dyskinesia as an early manifestation of Wilson disease.

    A 15-year-old boy was diagnosed as having Wilson disease. He perceived involuntary tongue movement and speech disorder since March 1990. The tongue movements presented in the resting state and during action. It contracted transversely and bilaterally with an irregular frequency about 1 Hz. As a result, the sides of the tongue moved to form a narrow central groove. This was quite different from the tongue protrusion of tardive dyskinesia. His speech had imprecise consonants, monopitch, low pitch, low volume, harsh voice, and hyponasality. These suggested that tongue dyskinesia could be an early sign of Wilson disease and was not the main cause of his dysarthria.
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15/37. Electroencephalographic findings in neuroacanthocytosis.

    This study reports the electroencephalographic findings in two siblings with neuroacanthocytosis. Slowing of the background activity with diffuse slow waves was the main finding in the daytime EEG. All-night polysomnographic recordings were characterized by high voltage slow activity during drowsy state and REM sleep. One patient with an average of 40 episodes of apnea per night of sleep was treated with imipramine and showed respiratory improvement.
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16/37. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease.

    We describe two patients and a previously reported patient who acquired unique pendular vergence oscillations of the eyes and concurrent contractions of the masticatory muscles, i.e., oculomasticatory myorhythmia (OMM). The smooth disjunctive eye movements cycled with a frequency of 0.8 to 1.2 Hz. An analysis of peak velocities (15 to 200 degrees/sec) with respect to peak amplitudes (5 to 25 degrees) revealed dynamics characteristic of normal vergence movements. The pathological alterations resulting in pendular vergence oscillations implicate a separately functioning, physiologically normal vergence system within the brainstem. In addition to paralysis of vertical gaze, each patient also experienced progressive somnolence and intellectual deterioration. An intestinal biopsy in 1 patient established a diagnosis of Whipple's disease, which led to appropriate treatment and amelioration of the OMM. A pathological diagnosis of Whipple's disease of the central nervous system was made in the other 2 patients; results of an intestinal biopsy in one of these patients were normal. No patient had palatal myoclonus, and olivary pseudohypertrophy was not found in two autopsy examinations. Thus, OMM is a distinct movement disorder and has been recognized only in Whipple's disease. We conclude that patients with OMM should be treated presumptively for Whipple's disease of the central nervous system, even if a jejunal biopsy is normal.
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17/37. Secondary generalized epilepsy in childhood: EEG patterns and correlation with responsiveness to benzodiazepines or ACTH (preliminary note).

    Secondary generalized epilepsy in childhood, characterized by absences or minor motor seizures, occurs in the forms of various syndromes, as defined by current classifications. EEG often shows continuous or subcontinuous paroxysmal activity associated with partly reversible psychomotor or mental regression. The paroxysmal activity can exhibit one of two distinct patterns: "organized" or "disorganized," although intermediate forms are common. The two patterns differ not only morphologically but also in the responsiveness to drug or hormone therapy, reactivity to stimuli, sleep changes and frequency of disordered slow rhythms. These features are illustrated by means of a survey of 10 cases.
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keywords = frequency
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18/37. Auditory processing deficit in a patient with rett syndrome.

    Because of the consistent and prominent language failure in rett syndrome, a patient with the characteristic phenotype was tested for subtle auditory processing deficits by visual reinforcement audiometry. In spite of excellent detection threshold and frequency discrimination, frequency sweep discrimination was at a chance level. This discrimination is normally performed by 5-8 month old infants. This observation suggests a defect in the specialized units of the inferior colliculus or higher auditory centers which subserve frequency sweep discrimination.
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19/37. Therapeutic effects of a ketogenic diet in rett syndrome.

    Seven girls (age 5 to 10 years) with rett syndrome were investigated extensively. In 6 patients elevations of blood pyruvate were found. Blood lactate levels were marginally elevated. Two patients had variably elevated blood glucose levels. Metabolic studies were otherwise normal apart from minimally elevated blood ammonia levels in 3 of 5 patients tested, 2 of whom were on valproic acid. All 7 patients had anticonvulsant resistant seizures. EEG changes included generalized slowing and multifocal spike wave discharges, and pseudo-periodic burst-suppression patterns during sleep. Respiratory monitoring revealed apneic episodes only during the waking record. Six patients were below the 5th centile for weight despite normal caloric intake. Treatment with ketogenic diets, using medium chain triglyceride (MCT) oil when possible, has improved seizure control in the 5 patients who could tolerate the diet. Slight behavioral and motor improvement has occurred in these 5 patients and 6 of 7 patients on high fat diets have gained weight. With a possible defect in carbohydrate metabolism and a difficult seizure disorder, use of a ketogenic diet is logical and appears to produce clinical benefit in patients with rett syndrome.
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keywords = wave
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20/37. Limb shaking--a carotid TIA.

    Eight patients are described with an unusual form of carotid transient ischemic attack, limb shaking. The basic features included a brief, involuntary, coarse, irregular, wavering movement or tremble involving arm-hand alone, or arm-hand and leg together. In 2 patients limb shaking was the initial manifestation of carotid occlusive disease, and all but one patient had other typical carotid transient ischemic attacks. Major atheromatous carotid occlusive disease was present in all patients on the side opposite the limb movements. Four patients had bilateral carotid occlusive disease. Cerebral ischemia from a carotid territory low-perfusion state may be the pathogenesis of these limb movements, an idea supported by the apparent benefit of surgical revascularization in abolishing or reducing the limb shaking in 6 patients. There was no clinical or EEG evidence to document an epileptiform etiology. Recognition of this uncommon form of carotid transient ischemic attack may be important in the early diagnosis and treatment of carotid occlusive disease.
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