Patient with adult‐onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation

We report a 51‐year‐old female patient with adult‐onset type II citrullinemia (CTLN2) who had a history of pancreatoduodenectomy for duodenal somatostatinoma with metastases to regional lymph nodes at age 49 years, paying special attention to indications for liver transplantation. At age 50 years, she developed hepatic encephalopathy with elevation of plasma ammonia and citrulline levels. A diagnosis of CTLN2 was made by DNA analysis of the SLC25A13 gene and treatment with conservative therapies was begun, including a low‐carbohydrate diet and supplementation with arginine and sodium pyruvate. However, despite these treatments, frequent attacks of encephalopathy occurred with markedly elevated plasma ammonia levels. While we were apprehensive regarding the risk of recurrence of somatostatinoma due to immunosuppressive therapy after liver transplantation, the patient was in a critical condition with CTLN2 and it was decided to perform living‐donor liver transplantation using a graft obtained from her son. Her postoperative clinical course was uneventful and she has had an active life without recurrence of somatostatinoma for 2 years. This is the first case of CTLN2 with somatostatinoma. As the condition of CTLN2 patients with rapidly progressive courses is often intractable by conservative therapies alone, liver transplantation should be considered even after surgery for malignant tumors in cases with neither metastasis nor recurrence.     

Pulmonary histoplasmosis in a Japanese male: Report of a case

(Received for publication on June 23, 1997; accepted on Jan. 6, 1998)     

Cervical mediastinoscopy after total laryngectomy and radiotherapy: its feasibility.

OBJECTIVE: The feasibility of cervical mediastinoscopy after total laryngectomy and radiotherapy has not been documented. METHODS: We describe our experience with cervical mediastinoscopy in two patients with total laryngectomy and radiotherapy for squamous cell carcinoma and had a Blom-Singer speaking tracheostomy valve in situ. Both patients presented with mediastinal lymphadenopathy and radiographic features suggesting a new pulmonary malignancy. RESULTS: Rigid bronchoscopy and cervical mediastinoscopy were undertaken and paratracheal and subcarinal lymph nodes were biopsied. Both patients made uneventful post operative recovery. Mediastinal lymph node biopsy was definitive in deciding their further care. CONCLUSION: Cervical mediastinoscopy is feasible in patients with total laryngectomy, tracheostomy and radiotherapy.     

Preoperative diagnosis of Rathke's cleft cyst

Two cases of Rathke's cleft cyst are presented. The cysts showed as high-density lesions on plain CT with slight enhancement with contrast medium. They appeared as a hyperintense mass in the T1-weighted magnetic resonance images. Preoperative diagnosis is very important because different sellar cystic lesions require different treatment.     

A case of successful resection of pulmonary arteriovenous fistula with a high shunt ratio

We report on a 25-year-old cyanotic man who was diagnosed as having a pulmonary arteriovenous fistula. His chief complaint had been shortness of breath since childhood. Polycythemia (Hb 21.4 g/dl) was detected during a health checkup at his company. A chest X-ray showed an abnormal mass in the left lung. Blood gas analysis showed severe hypoxia with PaO₂ of 38.6 mmHg at room air. Angio-CT showed a large aneurysmal lesion at S6 of the left lung with a large feeding artery and vein. Oxygen saturation was 75.2% in the radial artery and 62.5% in the right atrium. The right-to-left shunt ratio was therefore calculated as 62%. The aneurysmal lesion was resected by segmentectomy of the left S6 following division of A6 and V6. After a successful operation, the patient no longer had shortness of breath or cyanosis and blood gas analysis showed PaO₂ as 84.3 mmHg at room air. Pulmonary angiography showed no residual shunt lesion.     

Effects of allocation of visuo-spatial attention to visual stimuli triggering unilateral arm abduction on anticipatory postural control

OBJECTIVE: We investigated the effects of allocation of visuo-spatial attention to visual stimuli triggering arm movement on anticipatory postural control. METHODS: Fourteen healthy right-handed subjects participated in the study. Visual stimuli were randomly presented to the left or right visual field on a screen. An attention-directing cue or a non-directional cue was presented at 1000ms before visual stimulus onset. Subjects focused or divided visuo-spatial attention (focused- and divided-attention conditions, respectively) based on types of cues, and abducted the left or right arm rapidly in response to target stimuli (with 30% probability of visual stimuli) according to the side of presentation. Peak-to-peak amplitude of P1 and N1 components of event-related potentials (P1-N1 amplitude) elicited by visual stimuli and activation timing of postural muscles with respect to the middle deltoid were measured. RESULTS: Compared with divided-attention condition, in focused-attention condition P1-N1 amplitude at occipital electrodes was enhanced and activation of the left hip abductors during right arm abduction was hastened. A significant correlation was observed between the attention-related changes in these two measurements. CONCLUSIONS: Anticipatory postural control during right arm abduction was influenced by attention-related modulation of sensory-perceptual processing. SIGNIFICANCE: These findings are important for understanding of the effects of visuo-spatial attention on anticipatory postural control.     

Effects of Amantadine on Dopaminergic Neurons in Discrete Regions of the Rat Brain

The effect of a dopamine agonist, amantadine, on dopaminergic neurons was investigated in rat brains. Amantadine (40 mg/kg, i.p.) tended to increase DA (16%) and DOPAC (24%) levels. Further, amantadine (40 mg/kg, i.p.) significantly increased HVA levels in frontal cortex (44% above baseline after 40 mg/kg, i.p.) but not in corpus striatum and nucleus accumbens. Amantadine significantly increased DA levels at doses of 10, 20 and 40 mg/kg, i.p., in corpus striatum. On the other hand, amantadine decreased the L-DOPA accumulation by 30% in frontal cortex. This decreasing effect of amantadine may be attributable to a negative feedback mechanism by DA autoregulation. Our findings, therefore, suggest that amantadine may accelerate dopaminergic neurotransmission by increasing DA release from the frontal cortex and may possibly improve senile dementia.