INTRODUCTION: Ulcerative colitis (UC) is an inflammatory disease that predominantly involves the colon and rectum often requiring treatment with corticosteroids and immunomodulators. However chronic use of immunomodulators is usually associated with increased risk for lymphoma. Extranodal involvement of GI tract is typically seen however primary GI lymphoma is very rare. Here we present a unique case of B-cell lymphoma diagnosed from stomal biopsies. CASE DESCRIPTION/METHODS: A 76-year-old caucasian male with history of Ulcerative Colitis for almost 28 years, status post multiple abdominal surgeries including ventral hernia repair, ileostomy and colostomy presented with ongoing stoma complications for about 6 months. These complications included new fistula formation around the colostomy and ileostomy sites. Despite aggressive wound care, lesions did not regress, and continued to grow producing sharp, burning type of pain. There was ongoing purulent discharge from new fistula formation. Peri-ostomal biopsy from both ileostomy and colostomy was positive for B-cell lymphoma. On further history, patient endorsed fever, night sweats, and 30-lb weight loss over 5 months. PET scan showed increased uptake in both the stomas as well as liver. Chemotherapy options were discussed with Oncology, however patient opted for hospice as the prognosis was deemed poor. DISCUSSION: Primary GI lymphoma is common in stomach. Small intestine and colonic lymphoma account for 9% and 1% respectively. There have been multiple reports published citing association of lymphoma in IBD patients treated with immunomodulators. Our patient had two stomas (ileostomy and colostomy). Both periostomal biopsies were positive for B cell lymphoma. Periostomal lymphoma has been rarely reported. Parnesh et al (Case 1) reported a similar case of stomal lymphoma in a patient with history of Ulcerative Colitis. Levecq et al (Case 2) reported a stromal lymphoma in a patient with transfusion related AIDS who underwent total colectomy and ileostomy for indeterminate colitis. The lymphoma was diagnosed at 20 years (Case 1) and 2 years (Case 2) post stoma formation. Lymphoma should be in the differential of stoma being complicated by peristomal tissue proliferation, fistula and infection.
Introduction Narcolepsy with atypical unilateral cataplexy is a rare phenotype. We would like to share single center, single patient experience over the last 13 years of managing this patient. Report of Cases: 64-year-old female diagnosed with narcolepsy with unilateral cataplexy in 2008 with MSLT. Her cataplexy syndrome has been atypical, in that it has a strictly unilateral presentation for the past 13 years, with right face, arm, and leg weakness with strong emotions(anger/laughter). Her right-side extremities go limp for a few seconds to a minute. Her cataplexy syndrome has never evolved to become bilateral or unilaterally on her left side. Her physical examination has been largely unremarkable. For Narcolepsy, was on Modafinil (2008) which was up titrated to 600 mg with no side effects, eventually switched to Armodafinil 250 mg (2013). Eventually did not control EDS fully, methylphenidate (2014) was added. The patient reported cognitive impairment with gradual memory loss on stimulants (starting in 2012) and nocturnal insomnia on methylphenidate SR. Due to worsening cognition and memory, stimulants were discontinued in 2014 and patient referred to neurology and had neuropsychiatric evaluation for memory loss. Dementia lab workup and MRI with spectroscopy were unremarkable. Due to continuation of EDS was restarted on Modafinil until May 2021. In May 2021, she reported no longer fully controlled daytime sleepiness and Pitolisant was added with improvement in ESS from 17/24 to 9/24. For the management of unilateral cataplexy, patient was well controlled initially on venlafaxine (2008), eventually up titrated with an additional dose a few times. After cognitive impairment, patient stopped refilling it in 2014 and subsequently slept better without the venlafaxine. She reported that she would fall when she got very upset/mad so learnt not to get mad at people. She had many episodes of unilateral cataplexy mainly with laughing or excitement so was started on Fluoxetine 20 mg and eventually up titrated to 40 mg. Her unilateral cataplexy symptoms have been well controlled with the addition of Pitolisant. Conclusion Management of Narcolepsy with atypical unilateral cataplexy is challenging and requires use of multiple medications with different sites of action. Support (If Any)
Introduction Since iron is a cofactor used in the synthesis of CNS dopamine, some of which is then converted into norepinephrine, CNS iron deficiency will have an impact on the availability of dopamine and norepinephrine. Medications for narcolepsy and hypersomnia, such as stimulants and wakefulness medications, are dependent on the availability of these neurotransmitters to achieve their clinical response. Unrecognized and untreated nutritional deficiencies may contribute to inadequate responses to treatment of these sleep disorders. Report of Cases: A 26-year-old female with a history of iron deficiency since her pre-kindergarten health evaluation had a history of inadequate responses to stimulants and wakefulness medications. Additional labs were drawn to evaluate for nutritional deficiencies that could be contributing to the combination of fatigue and hypersomnolence. These revealed continued severe iron deficiency along with vitamin B6, B12, and D deficiencies. The treatment plan then expanded to include treatment of the nutritional deficiencies and a trial of pitolisant, which works to increase histamine levels in the brain. Sadly, pitolisant resulted in intolerable headaches, so it was discontinued. Conclusion This author feels that patients with hypersomnia warrant a broader approach that includes evaluating for nutritional deficiencies which may contribute to fatigue and hypersomnolence. In this case, the plan was to address the patient’s nutritional deficiencies and switch to pitolisant which mediates increased CNS histamine levels. This was unsuccessful due to the side effect of increased frequency and intensity of headaches related to pitolisant. Treating her iron deficiency with iron infusions should increase CNS synthesis of dopamine and norepinephrine. She may now have a better response to stimulants or wakefulness medications which depend on the availability of these neurotransmitters. She may also benefit from combination therapy by adding sodium oxybate to one of these medications. Support (If Any)
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