Cases reported "Disease Progression"

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1/22. Minimal, progressive, and fluctuating hearing losses in children. Characteristics, identification, and management.

    Referring to specific types of hearing loss as "minimal" or "mild" seems to imply that their effects are equally mild or negligible. A growing body of literature, however, supports the notion that such losses can have a significant impact on the communicative and educational development of young children. Although OME is considered a common childhood ailment, mounting evidence suggests that it is not always benign and may contribute to significant educational and communicative difficulties in some young children when accompanied by conductive hearing loss. Even very mild bilateral and unilateral SNHL seems to contribute to problems in the areas of social and emotional function, educational achievement, and communication in some children. Because these hearing losses are so mild, they may not be immediately recognized as the source of such difficulties. The purpose of this report is to heighten the general pediatrician's awareness of the significance of even very mild or minimal hearing losses in children. As the gatekeepers for children's health care, pediatricians are typically the primary recipients of parental expressions of concern and the initiators of evaluations or referrals to address such.
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2/22. Rapid progression of cardiomyopathy in mitochondrial diabetes.

    Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes.
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3/22. epilepsy surgery in children with pervasive developmental disorder.

    Pervasive developmental disorder (PDD) is occasionally associated with medically intractable complex partial seizures. The outcome of PDD was explored in three males and two females who underwent epilepsy surgery at 32 months to 8 years of age (mean = 4 years) after onset of epilepsy at 1 week to 21 months of age (mean = 11 months). Four children had temporal lobe resections (three right, one left; two for focal cortical dysplasia, and two for tumors), and one had a right temporoparieto-occipital resection (for focal cortical dysplasia). Each child underwent repeated evaluations by a pediatric neuropsychologist and psychiatrist. Fourteen to 47 months (mean = 23 months) after operation, one child with persistent seizures had moderate developmental and behavioral improvement, three children (two seizure free, one with rare staring spells) had mild developmental and behavioral improvement, and the remaining child (seizure free) experienced a worsening of her PDD. The four children with mild-to-moderate improvement in postoperative cognitive and behavioral development still demonstrated persistent delay. Cognitive gains were confirmed by neuropsychologic testing in the oldest patient but were not reflected in test results from the three younger children, who had more modest improvement. The child with worsening of her PDD had cognitive and emotional deterioration to babbling, echolalia, aggressiveness, decreased social interaction, and increased mouthing of objects beginning several months postoperatively. These results suggest that families should be counseled that PDD symptoms in children with focal epileptogenic lesions may or may not improve after epilepsy surgery, even if the surgery is successful with respect to seizure control.
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4/22. 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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5/22. Discussing disease progression and end-of-life decisions.

    Because most patients now want to know the truth about their diagnosis and prognosis, the ability to discuss the cancer diagnosis, disease recurrence, or treatment failure, and to solicit patients' views about resuscitation or hospice care, are important verbal skills for oncologists and other oncology health care providers. Moreover, the ability to clearly articulate a treatment plan or elicit patient preferences for treatment are a prerequisite to informed consent. Despite these imperatives, clinicians do not routinely receive training in key communication skills that could enable them to accomplish these tasks. A body of literature is available, however, that identifies communication strategies that can (1) facilitate the establishment of a close rapport with the patient, (2) identify the patient's information preferences, (3) ensure comprehension of key knowledge and information, (4) address the patient's emotions in a supportive fashion, (5) elicit the patient's key concerns, and (6) involve the patient in the treatment plan. In this article, we use dialogues between a physician and a hypothetical patient with advanced ovarian cancer to illustrate how communication techniques can be applied to accomplish these goals. We identify important benefits of the use of these techniques for both the physician and patient, and pose several questions regarding the training of physicians in this area.
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6/22. hyperthyroidism complicating asthma treatment.

    asthma is one of the most common chronic medical conditions. The usual treatment includes quick relief bronchodilator medications of the sympathomimetic class and controller medications that may include the long-acting inhaled bronchodilator salmeterol. Mild adverse cardiac and central nervous system effects are common with these medications, requiring modifications in dose or occasionally switching to a different medication. Both asthma and thyroid disease are common disorders that occasionally occur together. hyperthyroidism may exacerbate asthma. Many symptoms of hyperthyroidism are identical to the adverse effects of the commonly used inhaled bronchodilators and include tremor, nervousness, tachycardia, wide pulse pressure, palpitations, emotional lability, agitation, nightmares, aggressive behavior, and diarrhea. In this report we describe a patient with hyperthyroidism whose symptoms initially were thought to be adverse effects of the inhaled bronchodilator medications.
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7/22. Segmental wall motion abnormalities in an individual with idiopathic pulmonary hemosiderosis.

    Idiopathic pulmonary hemosiderosis (IPH) is a rare condition characterized by diffuse pulmonary hemorrhage of unknown etiology. Cardiac involvement in the form of myocarditis and right ventricular hypertrophy have been reported to occur in association with IPH, although findings on echocardiography have not been described. Herein is presented a case of an adult with IPH and echocardiographic abnormalities.
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8/22. Existential issues in palliative care: interviews of patients with amyotrophic lateral sclerosis.

    For the individual as well as for those caring for the patients, the diagnosis of amyotrophic lateral sclerosis (ALS) provides a great emotional challenge. Many factors, including existential distress, contribute to the emotional strain of patients with ALS. This study focuses on patients diagnosed with ALS and how they communicate existential issues related to meaning and guilt, relations, diagnosis and information, physical inability, and dying with dignity and respect for the person. The results of the present study indicate that (1) patients experience a number of problems, particularly in connection with physical inability, (2) the need to confide in someone is not particularly strong, (3) central for the value of life is to be respected as a person, (4) existential issues are of great importance to the patients.
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9/22. Disc herniation after lumbar fusion.

    STUDY DESIGN: Eight patients with a herniated disc after lumbar spinal fusion are reported. Their clinical features, imaging studies, and management are reported. OBJECTIVES: To identify the incidence and features of disc herniation above a spinal fusion, and to describe their management. SUMMARY OF BACKGROUND DATA: Late complications of lumbar spinal fusions have been reported in the literature, but disc herniation has not been specifically addressed in detail. The motion segment above a spinal fusion undergoes additional stresses, as documented by increased pressure and excessive motion, resulting in degenerative changes. These factors likely predispose to disc herniation. methods: Of 601 consecutive lumbar fusion cases over an 8-year period, herniated nucleus pulposus above the fusion was diagnosed in 8 patients. The clinical findings and imaging studies were reviewed, including a myelogram computed tomography scan, a magnetic resonance image with positive documentation of the herniation, or both. The management of these cases was reviewed. RESULTS: Eight patients (1.3%) (4 men and 4 women) were identified, whose average age was 56.4 years. Nonoperative treatment failed in six patients. Two of these patients underwent simple discectomy, and the remaining four underwent discectomy and fusion. All four patients went on to fusion. The average time from disc herniation onset to fusion was 28.4 months. CONCLUSIONS: Herniated disc after lumbar spinal fusion was found in approximately 1.3% of patients. Although rare, this entity that should be considered when patients complain of recurring back pain after a lumbar spinal fusion.
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10/22. alzheimer disease: "It's okay, Mama, if you want to go, it's okay".

    About 4 million people in the united states have alzheimer disease (AD) and the number of incident cases is expected to more than double from 377,000 in 1995 to 959,000 in 2050. patients, their families, and health care professionals struggle with a relentless and irreversible neurological syndrome that can last from 2 to 20 years. alzheimer disease causes both cognitive and functional impairments that predispose the patient to behavioral symptoms, destroy intellectual capacity and personality, erase the ability to communicate one's wishes for care, and lead to life-threatening consequences. At the close of life, family members and clinicians face decisions regarding degrees of intensive medical care to be provided for treatment of the late-stage consequences of AD, including withdrawal of invasive interventions, initiation of hospice, and treatment of a range of progressive medical conditions. physicians can assist patients with AD and their loved ones through the terminal phases of the illness by preparing them for the relentless progression of the disease and by supporting them through the intellectual and emotional conflicts accompanying the end of life.
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