Acute Intermittent Porphyria as a Rare Challenging Neuro-Metabolic Disease; a Case Report.

Porphyria is a challenging metabolic disease due to its heterogeneous presentation symptoms and its difficult diagnosis. Many affected individuals can complain of recurrent neuro-visceral attacks per year, some of which may be persistent and life-threatening, which is confusing if there is no established diagnosis. Although the motor manifestations, autonomic changes and seizure are highly suggestive, the diagnosis is often overlooked and needs confirmatory genetic testing. To the best of our knowledge, the acute intermittent porphyria (AIP) reported in this case, involving severe electrolyte disturbances and rapid severe weakness is a challenging neuro-metabolic case and is extremely rare worldwide. Here, we reported a case of AIP in a young girl who presented to the emergency department of Al-Araby international Hospital, Monufia, Egypt with severe abdominal pain, constipation, and headache which had started 10 days ago. It seems that the diagnosis of porphyria should be considered particularly in those patients with abdominal complaints associated with electrolyte disturbances, seizures, and severe progressive neuropathy.

Vertebral compression fracture treatment with vertebroplasty and kyphoplasty: experience in 407 patients with 1,156 fractures in a tertiary cancer center.

BACKGROUND: Painful vertebral compression fractures (VCFs), whether pathologic or osteoporotic, are a source of morbidity in cancer patients. At our tertiary cancer center, over the past decade we have used vertebroplasty (VP) and kyphoplasty (KP) to treat painful VCFs. More data are needed on the treatment of VCFs in cancer patients with these techniques. METHODS: We retrospectively reviewed the medical records of cancer patients with painful VCFs that had been treated at our institution between January 1, 2001 and May 31, 2008. Information was collected on demographic and clinical characteristics, features of the fractures, procedural details, and complications. Pre- and post-procedural pain and related symptoms were assessed using a subset of patients who had responded to the Brief Pain Inventory and the Edmonton Symptom Assessment Scale. RESULTS: A total of 407 cancer patients had 1,156 fractures that had been treated with VP or KP during 536 surgical procedures. Patients had an average of 2.8 fractures (range, 1-10). The majority of patients had pathologic fractures due to multiple myeloma (43%) or osteoporotic fractures (35%). Most fractures occurred in the thoracolumbar region. Adjacent-level fractures occurred in 18% of patients. Surgery provided significant relief from pain and several related symptoms. Symptomatic, serious complications requiring open surgery occurred in two cases (<0.01%) in our series. CONCLUSIONS: Our single-center experience revealed that a large number of cancer patients suffer from painful VCFs. The use of VP or KP in treating painful VCFs in cancer patients has good efficacy and an acceptably low complication rate.

Preliminary evaluation of an educational model for promoting positive team attitudes and functioning among pain medicine fellows.

OBJECTIVE: In response to the Accreditation Council for Graduate Medical Education's (ACGME) new pain medicine fellowship requirement, which emphasizes multidisciplinary training strategies aimed at providing improved clinical care for pain patients, we developed a multidisciplinary team training education model for trainees in our institution's Fellowship Program in Pain Medicine. Although the biopsychosocial model guides the delivery of care by multidisciplinary pain teams, there is a gap on how to improve team attitudes and functioning within an already extensive pain medicine curriculum. The current study aimed to fill that gap by developing and incorporating an educational model that focuses on interpersonal relationships among team members and strategies for improving team performance over time. DESIGN, SETTING, PARTICIPANTS, INTERVENTION, AND OUTCOME MEASURES: Here, we provide a brief overview of our institution's pain medicine fellowship training program highlighting how we have included a team training component into lectures, interdisciplinary case conferences, and journal club articles that focus on topics in the ACGME pain medicine curriculum. We present data from a team attitude and functioning assessment battery administered to 11 pain medicine fellows at the outset and end of their fellowship. RESULTS AND CONCLUSIONS: Mean assessment scores increased from the beginning of the fellowship to the end of the fellowship on interdisciplinary pain team knowledge, current team skills, and attitude toward health care teams. The current study demonstrated effective ways for assessing team attitudes and functioning and including this educational component into a 1-year pain medicine curriculum. Based on our results, we will continue to teach and model effective teamwork in an effort to enhance our trainees' attitudes toward working on an interdisciplinary pain team.

Two-tuohy needle and catheter technique for fluoroscopically guided percutaneous drainage of spinal epidural abscess: a case report.

BACKGROUND: The incidence of spinal epidural abscess has increased in the past decades. Traditionally, management was based on surgical decompression. More recent studies have shown conservative management has successful outcomes in selected patients. CASE REPORT: We present a case, in which an elderly woman presented with new onset radicular pain and mild leukocytosis more than a week after a complicated revision of an intrathecal catheter in place for management of chronic axial low back pain. Magentic resonance imaging (MRI) revealed a posterior epidural abscess from T12 to L2. Two Touhy needles were placed in the epidural space with fluoroscopic guidance for drainage of the abscess. A catheter was then advanced into the epidural space for irrigation with saline and an antibiotic solution. Intravenous antibiotics were continued for a total of 6 weeks. Radicular pain resolved immediately post-procedure. Serial MRIs also showed decreasing size of the abscess. CONCLUSION: Posterior spinal epidural abscesses may be successfully treated by way of the two Touhy needle and catheter technique for drainage and irrigation. This procedure should be reserved for patients that present with no neurological deficits or deemed nonsurgical candidates. Patients should continue on prolonged intravenous antibiotics and be monitored closely for clinical deterioration and undergo serial follow-up MRIs.

Kyphoplasty and vertebroplasty.

Percutaneous vertebroplasty is the injection of a vertebral compression fracture (VCF) with bone cement, generally polymethylmethacrylate. Percutaneous kyphoplasty is the placement of balloons into the vertebral body with an inflation/deflation sequence to create a cavity before the cement injection. These procedures are most often performed in a percutaneous fashion on an outpatient (or short stay) basis. The procedure is indicated for painful VCFs due to osteoporosis or malignancy, and painful hemangiomas. The procedure may have efficacy in painful vertebral metastasis and traumatic compression fractures. Much evidence favors the use of this procedure for pain associated with these disorders. The overall risks of the procedure are low, but serious complications (including spinal cord compression) can occur. With good patient selection and careful technique, these complications are avoidable, making the risk-to-benefit ratio highly favorable.

Cerebral hemorrhage as the initial manifestation of cystic fibrosis.

The authors present a case of an infant boy, otherwise healthy, who presented with acute irritability and obtundation. A head computed tomography scan revealed massive intracerebral hemorrhage. Investigations of the etiology revealed abnormal clotting times and reduced activity of all vitamin K-dependent factors. All etiologies of vitamin K deficiency were ruled out except malabsorption. The diagnosis of cystic fibrosis was confirmed by sweat chloride and genetic testing. Cystic fibrosis could present with cerebral bleed and should be considered in its differential diagnosis when other etiologies are not clear, as the other manifestations of vitamin K deficiency could be very subtle and unnoticed. Screening and early diagnosis of cystic fibrosis may prevent serious complications.

Spinal cord stimulator relieves neuropathic pain in a patient with radiation-induced transverse myelitis.

We present a patient with intractable neuropathic pain because of radiation-induced transverse myelitis unresponsive to medical treatment. After a successful trial of spinal cord stimulation, a permanent stimulator was implanted. Improvement was noted in verbal pain score, medication usage and function. Spinal cord stimulation may offer a therapeutic option for patients with neuropathic pain resulting from transverse myelitis and should be considered when other treatments fail.

Ganglionic local opioid application (GLOA) for treatment of chronic headache and facial pain.

OBJECTIVE: This report describes the effects of ganglionic local opioid application (GLOA) in patients with chronic headache and persistent idiopathic facial pain. CASE REPORT: We present 2 patients with chronic headaches and 1 patient with persistent idiopathic facial pain who were refractory to medical treatment. These patients responded well to a series of ganglionic local opioid applications (GLOAs) by administration of buprenorphine. The beneficial effect of GLOA was manifested by a decrease in pain intensity, reduction of pain medications, and improvement in quality of life. CONCLUSIONS: These results support the theory of sympathetically mediated pain in the head and face, the presence of opioid receptors on the sympathetic ganglia, and a possible beneficial role of opioids in modulation of this process. To our knowledge, this case series is the first case series in the English literature of the use of GLOA at the stellate ganglion for head-and-face pain.