Cases reported "Brain Diseases"

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11/39. Treatment of the agitated patient with an organic brain disorder.

    Agitated patients with organic brain disorders represent relatively common diagnostic and management problems. Therapeutic failures usually result from a failure to understand the patient's disturbed behavior, the staff's emotional response to the patient's behavior, or neglect of the biological cause and ineffective use of medication. Effective management depends on continued monitoring of the patient's mental status and physical condition.
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12/39. The coexistence and differentiation of late onset Huntington's disease and Alzheimer's disease. A case report and review of the literature.

    The case report presented is clinically compatible with late onset HD. The diagnosis was initially obscured by a lack of family history due to the early death of both parents and siblings. The presence of symptoms at the age of 59 in one offspring is consistent with the intrafamilial transmission of late onset HD disease. The early neuropsychometric data were consistent with the cognitive changes of HD, particularly with the loss of higher cognitive functions, memory and the relative decline in the performance IQ with the preservation of language skills. The psychiatric symptoms of emotional lability and apathy were also congruent with the diagnosis of HD. The atypical features of this patient's course, including progressively severe dementia, seizures and rigidity, may have provided clinical clues to the coexistence of both AD and HD. The absence of caudate atrophy on serial CT scans in this patient, although inconsistent with the gross findings reported on postmortem exam, perhaps could be explained by the 3 1/2-year interval between the last CT scan and death. Further, it has been noted previously that pathological changes tend to lag behind the clinical manifestations of the disease. The pathologic findings on autopsy were confirmatory for the presence of both AD and HD in this patient. genetic counseling for this family is now most appropriate.
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13/39. artifacts and diagnostic pitfalls on magnetic resonance imaging: a clinical review.

    High field MRI of the brain occasionally exhibits imaging artifacts; most artifacts are obvious and easily recognized, but some are subtle and mimic disease. A thorough understanding of brain MRI artifacts is important to avoid potential diagnostic pitfalls. Some imaging techniques or procedures could be utilized to remove or identify artifacts. These include additional projections, different pulse sequence, and 90 degree shift of phase-encoding gradient. The use of respiratory gating or cardiac gating may also improve image quality by reducing some of the motion-related artifacts.
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14/39. The care of our children in everyday life.

    The life within a family of a rett syndrome child is usually in a constant state of changing emotion due to the overwhelming responsibility of caring for the afflicted child and meeting the needs of the remaining family. Personal philosophy and care techniques are related to give insight into one family's mixed blessing of having a rett syndrome daughter and their struggle to maintain some normalcy within the seemingly incomprehensible malady. When families share with each other and with those who provide medical, educational and therapeutic care for their daughters, progress is made through mutual understanding and consideration for all persons involved, not the least of whom is the rett syndrome child and her family.
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15/39. Pathological eye-head coordination in roll: tonic ocular tilt reaction in mesencephalic and medullary lesions.

    The fundamental pattern of coordinated eye-head roll motion is based upon utricular/saccular as well as vertical canal input, and mediated by the graviceptive pathways from the labyrinths via pontomedullary vestibular nuclei to the rostral midbrain tegmentum. The tonic bilateral graviceptive input stabilizes the eyes and head in the normal upright position. A unilateral lesion causes imbalance in vestibular tone in the roll plane which results in a tonic ipsiversive ocular tilt reaction (OTR). OTR, the triad of ipsilateral head tilt, skew deviation and ocular torsion, occurred as a tonic response (nonparoxysmal) persisting over months to years and was presumed to be due to upper brainstem lesions in 3 patients. Precise localization was limited because of the nature of the lesions. A reversible tonic OTR was seen with acute infarction of the dorsolateral medulla oblongata in 4 out of 11 patients. It may be explained by an ipsilateral lesion of the posterior canal pathways. Transient OTR seems not to be rare in acute Wallenberg's syndrome and ocular torsion in these patients is dysconjugate with predominant excyclotropia of the ipsilateral hypotropic eye. A deviation of the subjective visual vertical in the direction of the spontaneous head tilt--a previously undescribed feature of OTR--was present in all 7 patients and indicates a pathological shift of the internal representation of the gravitational vector. With respect to perception (subjective vertical) it is proposed that the manifest ipsiversive OTR represents a motor compensation of an apparent eye-head tilt contraversive to the lesioned side. Despite the resulting postural imbalance and the conflicting true vertical of the visual surround, the eyes, head and body are continuously adjusted to what the central nervous system erroneously computes as being vertical.
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16/39. Chronic manganese poisoning: a neuropathological study with determination of manganese distribution in the brain.

    An autopsy case of a 52-year-old man suffering from chronic manganese poisoning (CMP) is reported with determination of the manganese distribution in the brain. The patient had been working in a manganese ore crushing plant since 1965. In 1967 he began to complain of difficulties in walking and diminished libido. Later, he developed various neuropsychiatric symptoms including euphoria, emotional incontinence, masked face, monotonous speech, "cock-walk", increased muscle tone, weakness of upper and lower extremities, tremor of the eye lids, and exaggeration of knee jerks. The major neuropathological change was degeneration of the basal ganglia, in which the pallidum was severely affected. The pallidum disclosed a loss and degeneration of nerve cells, which was especially marked in the medial segment, a prominent decrease of myelinated fibers, and moderate astrocytic proliferation. The substantia nigra was intact. Distribution of manganese in the brain of the present case of CMP was determined using flameless atomic absorption spectrometry and compared with control cases and also a case of Parkinson's disease (PD). There was no significant difference between the control cases and the case of PD in average concentration of manganese and its distribution in the brain. The present case of CMP showed no elevation in average concentration of manganese in the brain. However, there were some changes in its distribution. Thus, the continuance of neurological disorders in CMP is not linked to an elevated manganese concentration itself in the brain. CMP appears to be different from PD in neuropathology and manganese behavior in brain.
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17/39. Paradoxic vocal cord syndrome with surgical cure.

    Paradoxic vocal cord motion (PVCM) can cause wheezing, stridor, weak or absent voice, and ineffective cough. We have described a patient who had a posterior fossa arachnoid cyst, which was thought to be causing PVCM by compressing the brain stem. The PVCM and all symptoms resolved after surgical treatment of the arachnoid cyst.
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18/39. Myocardial mucormycosis with emboli in a hemodialysis patient.

    A 32-year-old man was found to have disseminated mucormycosis. Echocardiogram demonstrated bilateral ventricular thrombi, but there were no segmental wall motion abnormalities. At autopsy, it was found that these thrombi originated from a myocardial abscess and that death had been caused by septic embolization to the brain. The patient had been on maintenance hemodialysis for 9 years. Two transplants, 7 and 8 years previously, had been unsuccessful and had involved only 3 weeks of immunosuppressive treatment. No factor predisposing to fungal infection could be identified, with the possible exception of chronic uremia.
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19/39. A new lid sign in seventh nerve palsy.

    This paper describes a new lid sign in two patients with facial nerve paralysis. The facial nerve palsy was secondary to a pontine abscess in the first case and a complication of acoustic neuroma resection in the second patient. Both patients were found to have absence of the normal nasal twist of the lower lid during eyelid closure. The nasal twist is felt to help pump tears into the lacrimal drainage system. This paper will describe the absence of an important eyelid function in a patient with facial nerve paralysis. By way of background, Doane's slow motion movies have shown that during a normal blink, the upper lid moves both down and nasally, while the lower lid moves from 2 to 5 mm nasally. As the upper lid descends, the lower lid normally moves 2 to 5 mm in a horizontal and nasal direction. This motion of the lower lid helps produce a partial vacuum in the lacrimal system and is responsible for moving the fluid and debris in the tear mentiscus across the eye and into the lacrimal drainage system. The two cases of facial nerve paralysis to be presented demonstrate absence of tear drainage on the side of the lesion and a build-up and overflow of the tears on that side.
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20/39. Protracted epileptiform encephalopathy: an unusual form of partial complex status epilepticus.

    An 11-year-old previously healthy boy had an abrupt onset of partial complex, focal, multifocal, and generalized seizures, with interictal expressive aphasia, extreme emotional lability, agitation, and complex visual and auditory hallucinations. EEGs showed frequent runs of rhythmic high-voltage delta over the right and subsequently over the right and left temporal and frontal regions. All other studies were negative (repeated computed tomography, spinal fluids, viral titers, and cultures). Nadir during the second month showed virtual unresponsiveness, prolonged rhythmic motor and apneic seizures, total anorexia, and sleeplessness. Remission of the electrical and clinical seizure activity and a gradual improvement through a state of agitation and emotional lability occurred during the third and fourth months. One year later he was entirely normal. Compared with the other previously documented cases of prolonged partial complex status, this case is notable for its florid and severe symptomatology, long duration, and final benign outcome.
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