This report describes the case of a patient with cricopharyngeal dysfunction with significant piriform sinus expansion. An 80-year-old man presented with a three-year history of dysphagia. Palsy of the cricopharyngeal chalasis was identified by electromyography under both videofluorography and manofluorography. Although a widening procedure was performed in the cricopharyngeal region using a bougie, the patient gained only minor relief from his dysphagia. After the patient had had adequate time to recover spontaneously (six months), a cricopharyngeal myotomy was performed. As a result, his dysphagia resolved and the post-operative course was uneventful. The clinical and histopathological findings in this case suggested that significant piriform sinus expansion had resulted from the cricopharyngeal dysfunction, due to cricopharyngeal myopathy.

Summary:A 6-year-old girl underwent allogeneic BMT from a matched sibling donor for the treatment of Kostmann syndrome. She suddenly became drowsy on day 30 after BMT, and lost consciousness 2 days later. Cranial CT scan showed symmetrical lesions suggesting bilateral necrosis in the basal ganglia. Clinical and laboratory investigations failed to reveal any evidence of neurometabolic disease. Keywords: bilateral basal ganglial necrosis; allogeneic BMT; Kostmann syndrome Acute encephalopathy with symmetrical lesions in the basal ganglia is a rare neurological disease. Generally either a preceding illness or underlying metabolic disease exists in most reported cases. Bilateral basal ganglial necrosis, especially infantile striatal necrosis has been reported to occur often after a non-specific febrile upper respiratory infection. We describe a child who experienced this disease after allogeneic BMT without any definite preceding episodes or metabolic disorders, and presently suffers from severe neurological sequelae. Case reportA neurologically normal 6-year-old girl underwent allogeneic BMT for Kostmann syndrome from an HLA-identical sibling donor. Conditioning consisted of busulphan 35 mg/m 2 p.o. four times daily from day −9 to day −6, ATG 2.5 mg/kg i.v. daily and cyclophosphamide 50 mg/kg i.v. daily from day −5 to day −2. She developed a high grade fever and generalized tonic clonic seizure for a few minutes during the first administration of ATG on day −5. No neurological sequelae could be found after the seizure resolved. She had no past history of febrile convulsions or epilepsy. Initially phenytoin 10 mg/kg i.v. was given twice Correspondence: Dr J Okamura, Institute for Clinical Research, National Kyushu Cancer Center, 3-1-1, Notame, Minami-ku, Fukuoka, 811-1395, Japan Received 26 May 1998; accepted 3 October 1998 daily for seizure prophylaxis from day −9. Hydrocortisone 125 mg i.v. was added as an antipyretic and no seizure occurred thereafter. Cyclosporin A was used for graftversus-host disease prophylaxis.Engraftment was confirmed on day 12 after BMT when the neutrophil count reached 500/ l. The patient experienced asymptomatic mild hyponatremia which started on day 11 and lasted for 30 days. The serum sodium concentration varied between 121 to 133 milliequivalents per liter (mEq/l). The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) was thus suspected because her urinary osmolarity and serum antidiuretic hormone levels were normal although the plasma osmolarity was mildly decreased below the normal limits. Hyponatremia resolved on day 41 with water restriction and a half correction of sodium. The patient suddenly became drowsy on day 30 and lost consciousness 2 days later. No preceding episodes including fever, vomiting, or seizures were observed. A neurological examination on day 32 showed a comatous state of consciousness without any response to painful stimulation except for slight movement of the left upper limb. The patient remained in the supine position, and showed an asymme...