Simultaneous development of ulcerative colitis in the colon and sigmoid neovagina.

Vaginoplasty using sigmoid colon is a common technique for creation of a neovagina. However, special consideration must be given to potential long term consequences of using a colonic conduit for vaginal replacement. We report on the youngest described case in which a patient developed ulcerative colitis refractory to medical therapy with simultaneous involvement of a sigmoid neovagina requiring total proctocolectomy and neovaginectomy. A 17 year old XY female with a history of gonadal dysgenesis and sigmoid graft vaginoplasty presented with a history of bloody, mucoid vaginal discharge, abdominal pain, bloody diarrhea and weight loss. Colonic and neovaginal biopsies demonstrated active colitis with diffuse ulcerations, consistent with ulcerative colitis. Despite aggressive immunosuppressive treatment she had persistent neovaginal and colonic bleeding requiring multiple transfusions, subtotal colectomy and ultimately completion proctectomy and neovaginectomy. It is imperative to recognize that colectomy alone may be an inadequate surgical intervention in patients with ulcerative colitis and a colonic neovaginal graft and that a concomitant neovaginectomy may be integral in providing appropriate treatment.

Resting tremor secondary to a pineal cyst: case report and review of the literature.

BACKGROUND: Patients with pineal lesions typically present with the classical signs of increased intracranial pressure (headache, nausea, vomiting) and/or Parinaud's syndrome. Rare symptomatology of secondary parkinsonism attributed to pineal lesions has been previously reported in the literature. We describe an unusual case of a pineal cyst in a patient with the presenting sign of a resting tremor. CASE DESCRIPTION: We report an 18-year-old Caucasian female who presented with a 1-month history of a new-onset progressive, unilateral low-frequency right-hand resting tremor with associated headache, nausea, vomiting, and excessive diarrhea. Magnetic resonance imaging demonstrated an atypical appearance with enhancement of a mildly prominent pineal gland, possibly representing a pineal cyst. The patient did not exhibit radiographic signs of hydrocephalus. Based upon the radiographic appearance, one could not exclude with absolute certainty the presence of a malignancy. The patient ultimately underwent a bilateral suboccipital craniotomy with gross total resection of the lesion. Postoperatively, the patient exhibited immediate resolution of her preoperative resting tremor and continues to be symptom free at 1 year. CONCLUSIONS: A new-onset, resting tremor and/or other secondary parkinsonism symptoms should raise clinical suspicions of pineal lesions. Treatment can be guided based on tissue type and the presence or absence of hydrocephalus. We observed that complete surgical resection of the lesion provided the best treatment option for the total resolution of symptoms attributed to the disturbance of the microvasculature surrounding the nigro-striatal-pallidal system.

Altered excitability and distribution of NMDA receptor subunit proteins in cortical layers of rat pups following multiple perinatal seizures.

During a critical period of postnatal development the epileptogenic focus is thought to be of cortical origin. We used immunohistochemistry and Western blotting to elucidate potential mechanisms underlying an increased state of susceptibility to seizures in immature animals. Distribution patterns of N-methyl-D-aspartic acid (NMDA) (NR1 and NR2A/B) and alpha-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA) (GluR1 and GluR2) subunits were analyzed in retrosplenial, parietal and temporal cortices during the first two postnatal weeks following three episodes of status-epilepticus. Rat pups were injected three times with kainic acid (3x KA) on P6, P9, and P13 and subsequently sacrificed 48 h after the third seizure. Cortical electroencephalography (EEG) showed increased number of spikes and bursts of longer duration after 3x KA. Immunodensity measurements after 3x KA revealed a robust increase in NR2A/B labeling specific to cortical layer V throughout the retrosplenial, parietal, and temporal cortices, with no changes noted in piriform cortex. NR1 layer V immunoreactivity was also simultaneously increased in serial sections but to a lesser degree; heightened immunodensities were specific to retrosplenial and temporal cortices. The NR1:NR2 ratio was decreased in cortical layer V of the temporal and retrosplenial cortices but not in parietal cortex despite elevated immunoreactivity. Steady levels of GluR1 and GluR2 subunits were noted in all cortical areas studied in the same animals. Thus, recurrent perinatal seizures led to selective and layer-specific increases in NMDA receptor proteins. These changes may be responsible for lowering the seizure threshold in deeper cortical areas and eventually contribute to the cortical epileptogenic focus.