תוצאת חיפוש

Polyneuropathy in Critical Illness

M. Mouallem, A. Adunsky, H. Semo, M. Dolgopiat

Depts. of Medicine E, Geriatrics, and Neurological Rehabilitation, Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv University

Critical illness polyneuropathy developed in 8 patients aged 22-84 years in our intensive care units. This acute polyneuropathy, predominantly axonal and motor, develops in the setting of the systemic inflammatory response syndrome and multi-organ failure. It is found in about 50% of patients treated in intensive care units for more than 2 weeks. In those who survive, neurological and functional recovery is the rule.

Attentional Characteristics of Developmental Right Hemi-Sphere Syndrome

Yael E. Landau, Varda Gross-Tsur

Neuropediatric Unit, Shaare Zedek Medical Center, Jerusalem

Developmental right hemisphere syndrome (DRHS) is characterized by emotional and interpersonal difficulties, attention deficit hyperactivity disorder (ADHD), visuo-spatial handicaps, subtle left body neurologic signs and failure in nonverbal academic domains, especially arithmetic. Concurrence of ADHD and DRHS is not surprising because research has implicated dysfunction of the right hemisphere in both syndromes. Furthermore, the right hemisphere has more brain areas devoted to attentional processing, making it more important and more vulnerable in attentional problems.

We describe the clinical parameters of DRHS as exemplified by 2 cases, a boy and a girl, both 13 years old. They participated in a study group in which attention and speed of performance were assessed in children with DRHS and were compared to children with ADHD and to a control group. A tendency to overfocusing, difficulty in inhibition, perseverative behaviors, stereotypy, and slowness and absence of hyperactivity characterized the DRHS group. These behaviors led us to hypothesize that the attentional symptoms in DRHS define a specific subgroup of ADHD which requires a different therapeutic approach.

The Wandering Spleen

A. Ravid, D. Lev, V. Makarin, Y. Klausner, M. Umansky Y. Kluger

Surgery B and C Depts., Sourasky Medical Center, and Sackler Faculty of Medicine, Tel Aviv University

We describe a 26-year-old woman with thrombocytopenia discovered during gestation. On admission for evaluation of abdominal pain, torsion of an ectopic spleen was found. The spleen was removed and the thrombocytopenia resolved.

D-Lactic Acidosis in Short Bowel Syndrome

Ben-Ami Sela, Joseph Zlotnik, Tamar Masos, Joseph Danieli, Sarah Mazia-Beni, Anita Jonas

Institute of Chemical Pathology and Pediatric Intensive Care and Gastroenterology Units, Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv University

A boy of 20 months and a girl of 27 months who had previously undergone massive bowel resection due to congenital intestinal obstruction are described. During grad-ual reduction of total parenteral nutrition and initiation of oral feeding, both developed severe, acute metabolic acidosis, accompanied by encephalopathy and ataxia. After the laboratory identification of massive amounts of the d-Disomer of lactic acid in urine and blood, both were successfully treated with IV bicarbonate, and metronidazole to suppress the overgrowth of colonic lactobacilli responsible for the metabolic crisis.

Ramsay Hunt Syndrome

Doron Aframian, R. Ben-Oliel, Yair Sharav

Depts. of Oral Diagnosis, Medicine and Radiology, Hebrew University- Hadassah School of Dental Medicine, Jerusalem

Ramsay Hunt syndrome is caused by infection of the geniculate ganglion of the seventh cranial nerve by varicella- zoster virus. A case in an 82-year-old woman is described. She presented with oral lesions, right facial palsy and an eruption and pain in her right ear. Oral examination revealed small circumscribed erosions on the right anterior two-thirds of the tongue, with loss of taste. There were also lesions on her right palate. Early diagnosis and treatment are important as immediate treatment is more likely to prevent irreversible complications affecting the facial and other cranial nerves involved.

Muir-Torre Syndrome: Importance of Clinical Diagnosis and Genetic Investigation

Hana Strul, Paul Rozen, Tova Naiman, Ruth Shomrat

Gastroenterology Dept. and Genetics Institute, Tel Aviv Medical Center and Tel Aviv University

Muir-Torre syndrome is a relatively rare cutaneous manifestation of hereditary nonpolypous colorectal cancer (HNPCC). This autosomal dominant syndrome is characterized by a combination of sebaceous gland and malignant visceral tumors. The common sites of internal malignancies are the gastrointestinal tract and urinary system. It appears in early adult life and its clinical course is relatively slow.

In some families genetic diagnosis can identify asymptomatic carriers of the mutation. All first-degree relatives, especially mutation carriers, should be referred from the age of 20 years for routine follow-up and early treatment, as it has been proven to decrease morbidity and mortality.

We present a 51-year-old man with Muir-Torre syndrome diagnosed by the presence of multiple adenomas of sebaceous glands, colonic adenoma and adenocarcinoma of the duodenum. The family history was typical for HNPCC. A mutation in the hMSH2 gene on chromosome 2p was found in the patient and in several asymptomatic family members. The aim of this report is to increase awareness of this syndrome and emphasize the importance of referring patients and their families for clinical and genetic counseling and diagnosis.

Iatrogenic Gallstone Ileus: A New Complication of Bouveret's Syndrome

Miguel Iuchtman, Amos Sternberg, Ricardo Alfici, Ehud Sternberg, Tzvi Fireman

Depts. of Surgery and Gastroenterology, Hillel Yaffe Medical Center, Hadera, and Rappaport Medical School, Haifa

Bouveret's syndrome involves gastric outlet obstruction caused by a gallstone in the duodenum. This type of gallstone ileus can be diagnosed and treated endoscopically. Endoscopic stone removal is especially indicated in poor risk patients. A dislodged impacted stone can migrate distally and cause small bowel mechanical obstruction. We report a 51-year-old woman who underwent endoscopic duodenal stone manipulation which resulted in small bowel obstruction.

Bowel-Lengthening in a Newborn with Short Bowel Syndrome

Schmuel Katz, Ilan Erez, Ita Litmanovitz, Ludwig Lazar, Arie Raz, Zipora Dolfin

Depts. of Pediatric Surgery, Neonatology and Pediatrics; Meir Hospital, Kfar Saba

Advances in parenteral nutrition and supportive therapy have led to improvement in survival of babies with short-bowel syndrome. Those whose intestinal mass is very unlikely to be adequate should have surgical therapy as soon as possible, before they develop the complications of long-term parenteral nutrition or significant enteritis.

We present a newborn with short-bowel syndrome due to prenatal midgut volvulus. At operation the remaining viable jejunum, 15 cm long, was anastomosed to the cecum. All feeding attempts failed, and the infant suffered from malabsorption. Calories and proteins had to be supplied by intravenous total parenteral nutrition.

At 3 months of age there was significant widening of the remaining bowel and Bianchi's bowel-lengthening procedure was performed. The postoperative course was uneventful and there was gradual improvement in intestinal absorptive capacity. The patient was weaned from parenteral nutrition at 3 years of age. Now, 2 years later, she eats a normal diet.

Serotonin Re-Uptake Inhibitors as Primary Therapy for Carotid Sinus Hypersensitivity

Amos Katz, Angel Kantor, Alexander Battler

Cardiology Dept., Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Carotid sinus syndrome is a well-recognized cause of unexplained syncope in older patients, and may lead to significant morbidity due to trauma from falls. Dual chamber pacing has been shown to be effective in relieving symptoms due to bradycardia, but not due to vasodepressor response. We report an 84-year-old man with recurrent syncope due to carotid sinus hypersensitivity. He was treated only with a serotonin re-uptake inhibitor and was symptom-free after 3 weeks of therapy. He has remained symptom-free for the past year.

Autonomic Dysregulation in Post-Traumatic Stress Disorder: Power Spectral Analysis of Heart Rate Variability

Hagit Cohen, Uri Loewenthal, Mike A. Matar, Hanoch Miodownik, Zeev Kaplan, Yair Cassuto, Moshe Kotler

Mental Health Center, Israel Ministry of Health; Anxiety and Stress Research Unit, Faculty of Health Sciences; Dept. of Life-Sciences, Ben-Gurion University of the Negev, Beer Sheba

Spectral analysis of heart rate variability (HRV) has been shown to be a reliable noninvasive test for quantitative assessment of cardiovascular autonomic regulatory response, providing a dynamic map of sympathetic and parasympathetic interaction. In a prior study exploring the state of hyperarousal that characterizes the post traumatic stress disorder (PTSD) syndrome, we presented standardized heart rate analyses in 9 patients at rest, which demonstrated clear-cut evidence of a baseline autonomic hyperarousal state.

To examine the dynamics of this hyperarousal state, standardized heart rate analysis was carried out in 9 PTSD patients, compared to a matched control group of 9 normal volunteers. 20-minute ECG recordings in response to a trauma-related cue, as opposed to the resting state, were analyzed. The patients were asked to recount the presumed triggering traumatic event, and the control subjects recounted a significant stressful negative life event.

Whereas the control subjects demonstrated significant autonomic responses to the stressogenic stimulus of recounting major stressful experiences, the patients demonstrated almost no autonomic response to the recounting of the triggering stressful event. The patients demonstrated a degree of autonomic dysregulation at rest comparable to that seen in the control subjects' reaction to the stress model.

The lack of response to the stress model applied in the study appears to imply that PTSD patients experience so great a degree of autonomic hyperactivation at rest, that they are unable to marshal a further stress response to the recounting of the triggering trauma, as compared to control subjects. A subsequent study of the effect of medication on these parameters showed that they are normalized by use of selective serotonin re-uptake inhibitors (SSRI's).

Neither the clinical implications of these findings, nor their physiological mechanisms are clear at present. We presume that they reflect a central effect, as the peripheral automatic effects of SSRI's are relatively negligible.

Trichobezoars

M. Barzilai, N. Peled, M. Soudack, L. Siplovich

Carmel and Rambam Medical Centers, Haifa and Afula Medical Center 

Trichobezoars are accumulations of hair casts in the stomach associated with trichophagia. The typical patient is an adolescent female who presents with alopecia and an upper abdominal mass which on moving can cause intermittent gastric outlet obstruction. Only a minority of patients have severe psychiatric disorders. When hair strands extend from the main mass in the stomach, all along the small bowel and reaching the cecum, the condition is termed the Rapunzel syndrome. In about 5% of patients there are separate hair masses in the stomach. The clinical presentation includes abdominal pain, loss of appetite, weight-loss, vomiting, loose stools, pancreatitis, jaundice, anemia and hypoalbuminemia. These signs and symptoms raise the suspicion of malignancy. Complications of bezoars include ulcers, perforation of the bowel, obstruction and intussusception. Diagnosis can be established either by barium swallow or by CT scan. Ultrasound might suggest the diagnosis, but sonographic features are definitely not pathognomonic. Treatment is surgical, as endoscopic removal is usually unsuccessful. We present 2 children in whom trichobezoars were found.

Cyclic Vomiting Syndrome in Children

G. Ben-Amitay, Y. Nevo, D. Lieberman, R. Mester, S. Harel

Ness Ziona Mental Health Center, Institute for Child Development and Pediatric Neurology Unit, Tel Aviv-Sourasky Medical Center, and Tel Aviv University Medical School

Cyclic vomiting syndrome in children is a manifestation of various etiologies, including gastroenterological and renal disorders, central and autonomic nervous system abnormalities, as well as metabolic and endocrine dysfunction. Frequently no organic cause is found. Personality profiles of children with cyclic vomiting reveal perfectionism, competitiveness, and aggressive behavior. Vomiting attacks have been induced by anxiety and excitement in patients with cyclic vomiting. We describe an 8-year-old girl with cyclic vomiting, frequently associated with occipital headaches, photophobia or dizziness. Psychiatric evaluation indicated a generalized anxiety disorder.

Abdominal Compartment Syndrome

D. Rosin, M. Ben Haim, A. Yudich, A. Ayalon

Dept. of General Surgery and Transplantation, Chaim Sheba Medical Center, Tel Hashomer and SacSchool of Medicine, Tel Aviv University

Abdominal compartment syndrome refers to a complex of negative effects of intra-abdominal hypertension. Its most common cause is complicated abdominal trauma. The syndrome includes mainly hemodynamic and respiratory manifestations but may involve other systems as well. It may present as a life-threatening emergency in the multi-trauma patient. Awareness of the syndrome may enable the surgeon to take preventive measures or to diagnose it earlier and to treat it effectively. We describe a 21-year old man who developed this syndrome after multiple gunshot wounds, with severe liver injury. After 2 operations the typical manifestations of the syndrome were diagnosed. He was re-operated to release intra-abdominal hypertension and then slowly recovered.

Central Cord Syndrome in the Elderly Who Fall: a Diagnostic Trap

A. Adunsky, H. Semo, S. Levenkrohn

Geriatric Rehabilitation Dept., Chaim Sheba Medical Center, Tel Hashomer

Acute post-traumatic spinal injury is common and usually poses no diagnostic difficulties. Following a low-energy fall, the onset of acute central cord syndrome in the elderly is not common and is frequently misdiagnosed. Men aged 60 and 81, and a woman aged 75 are described, in whom central cord syndrome was overlooked. Awareness of this condition is important to avoid incorrect diagnosis and hazardous management.

Gradenigo Syndrome and Cavernous Sinus Thrombosis, in Fusobacterial Acute Otitis Media

S. Hananya, Y. Horowitz

Pediatrics Dept., Central Hospital of the Emek, Afula

In this era of antimicrobial medication, intracranial complications following otitis media are rare. We present a 5-year-old boy who suffered from petrositis (Gradenigo syndrome) and cavernous sinus thrombosis as combined complications of acute otitis media caused by fusobacteria. The diagnosis was made using imaging methods suited to the various structures of the skull. Cure was achieved by prolonged conservative treatment with antibiotics, with gallium scan for evaluation of the bone inflammation.