Masakatsu Taki

Angioedema: 6 years experience with fourteen cases

OBJECTIVE To study the difference in the findings between the causes of angioedema and the characteristics of angioedema induced by angiotensin receptor II blockers (ARBs), and to investigate whether laboratory examinations for acute phase inflammatory markers can aid in diagnosis and predict airway risk. METHODS We retrospectively reviewed fourteen cases of patients with angioedema that were treated from 2000 to 2006. Data were collected regarding age, sex, location of the edema, cause, time course of resolution and laboratory examinations (leukocyte counts, serum C-reactive protein (CRP) level, complement function and the activity of C1 esterase inhibitor). RESULTS The causes of angioedema were ACEIs in six patients (42.9%), candesartan (ARB) in three (21.4%), HAE (types 1 and 2) in two, and unknown in three. Of these patients, 71.4% exhibited edema in the floor of the mouth, irrespective of the cause. Two patients with edema induced by candesartan exhibited both lingual and laryngeal edemas. The remaining one with candesartan-induced edema exhibited edema in the neck and mediastinum and pleural effusion. The average time to resolution was 4.1 days, ranging from one to twelve days. The edema in eleven patients resolved with conservative therapy, while three patients underwent tracheotomy. In two patients with candesartan-induced edema, although the edemas resolved completely after cessation of candesartan administration, the edemas reappeared in the same locations, two and thirty days after the cessation of candesartan for each patient. None of the patients with angioedema induced by ACEIs exhibited elevation of serum CRP levels. No significant differences were found for leukocyte counts and serum CRP levels between patients with angioedemas induced by ACEIs, ARB and those of unknown cause. No significant differences were observed in the above findings between the patients who underwent tracheotomy and those who did not. Two patients exhibited low C4 levels, and one of the two exhibited no activity of C1 esterase inhibitor. CONCLUSION Consistent with previous reports, angioedema in the floor of the mouth extending to the tongue should be considered as a possible risk factor for airway compromise. Laboratory examinations for acute phase inflammatory markers are not useful for diagnosis and are not predictive for airway intubation and tracheotomy. Angioedema induced by candesartan can present in anomalous sites and reappear following drug cessation even if the edema has resolved completely.

Epidermal cyst of the bony external auditory canal

A previously healthy 3-year-old boy was referred to our hospital because of a mass in the left external auditory canal. He had no complaint and his mother found the mass by chance. On physical examination, left otoscopy showed a white mass in the posterior canal wall (Fig 1A). The mass was smooth-surfaced with unclear borders. The right external auditory canal and both tympanic membranes were normal. Computed tomography scan showed a mass in the posterior wall of the bony external auditory canal, lateral to the tympanic membrane without erosion of the surrounding bony wall (Fig 1B). The middle ear was normal on radiographic examination. With the patient under general anesthesia, the mass was surgically removed via a retroauricular approach. It was ablated from the bony wall and skin, and successfully enucleated en bloc without rupture (Fig 1C). It was lateral to the annulus, and the bone of the external auditory canal was normal in appearance. The enucleated mass was round, firm, and 8 mm 7 mm 5 mm in size. The external auditory canal was packed for 1 week after surgery. The patient had

Low-grade cribriform cystadenocarcinoma of the parotid gland: A case report

Low-grade cribriform cystadenocarcinoma is a rare tumor that is recognized as a variant of cystadenocarcinoma by the 2005 WHO classification. We report a case of low-grade cribriform cystadenocarcinoma. The patient was a 32-year-old woman who presented with a mass in the right parotid region. Computed tomography and magnetic resonance imaging revealed a polycystic mass in the right parotid gland. The preoperative fine needle aspiration biopsy showed no malignancy, however the frozen section during surgery showed carcinoma. The patient subsequently underwent total parotidectomy with preservation of the facial nerve. Microscopically, the tumor had a typical feature of intraductal proliferation. There was a golden brown pigment and PAS-positive/diastase-resistant cytoplasmic granules in the epithelium of the cysts. Immunohistochemically, smooth muscle actin highlighted the cells rimming the cystic spaces. The tumor cells were negative for S-100 protein. The patient has no recurrence and no facial palsy after the treatment.

Opioid-Induced Nausea Involves a Vestibular Problem Preventable by Head-Rest.

Background and Aims Opioids are indispensable for pain treatment but may cause serious nausea and vomiting. The mechanism leading to these complications is not clear. We investigated whether an opioid effect on the vestibular system resulting in corrupt head motion sensation is causative and, consequently, whether head-rest prevents nausea. Methods Thirty-six healthy men (26.6±4.3 years) received an opioid remifentanil infusion (45 min, 0.15 μg/kg/min). Outcome measures were the vestibulo-ocular reflex (VOR) gain determined by video-head-impulse-testing, and nausea. The first experiment (n = 10) assessed outcome measures at rest and after a series of five 1-Hz forward and backward head-trunk movements during one-time remifentanil administration. The second experiment (n = 10) determined outcome measures on two days in a controlled crossover design: (1) without movement and (2) with a series of five 1-Hz forward and backward head-trunk bends 30 min after remifentanil start. Nausea was psychophysically quantified (scale from 0 to 10). The third controlled crossover experiment (n = 16) assessed nausea (1) without movement and (2) with head movement; isolated head movements consisting of the three axes of rotation (pitch, roll, yaw) were imposed 20 times at a frequency of 1 Hz in a random, unpredictable order of each of the three axes. All movements were applied manually, passively with amplitudes of about ± 45 degrees. Results The VOR gain decreased during remifentanil administration (p<0.001), averaging 0.92±0.05 (mean±standard deviation) before, 0.60±0.12 with, and 0.91±0.05 after infusion. The average half-life of VOR recovery was 5.3±2.4 min. 32/36 subjects had no nausea at rest (nausea scale 0.00/0.00 median/interquartile range). Head-trunk and isolated head movement triggered nausea in 64% (p<0.01) with no difference between head-trunk and isolated head movements (nausea scale 4.00/7.25 and 1.00/4.5, respectively). Conclusions Remifentanil reversibly decreases VOR gain at a half-life reflecting the drug’s pharmacokinetics. We suggest that the decrease in VOR gain leads to a perceptual mismatch of multisensory input with the applied head movement, which results in nausea, and that, consequently, vigorous head movements should be avoided to prevent opioid-induced nausea.

Case report: Two cases of hearing impairment due to intracranial hypotension

We report two cases of spontaneous intracranial hypotension (SIH) that presented with orthostatic hearing impairment. Pure tone audiometry in case 1 and distortion-produced otoacoustic emissions (DPOAEs) in case 2 clearly revealed orthostatic low-frequency hearing impairment. The symptoms resolved with conservative therapy in case 1, but they did not resolve completely after three treatments with epidural blood patch in case 2. The orthostatic changes at low-frequency that were present in both examinations indicated a relative endolymphatic hydrops due to SIH. Both otological examinations were useful to infer the pathophysiology of hearing impairment associated with SIH.

Middle ear malformations in identical twins

The majority of the congenital anomalies of middle ear are solitary and a non-hereditary. We report cases of identical twins with congenital incudo-stapedial disconnection. Case 1 was an 8-year-old girl. Hearing impairment was identified at the age of three. She was referred to our university hospital in April 2005. Pure-tone audiogram showed conductive hearing impairments. Computed tomography (CT) revealed the incudo-stapedial disconnections in both ears. The exploratory tympanotomies on the right and left ears were performed in May and July 2005, respectively. The surgical findings showed absence of the long process and presence of the lenticular process of the incus in both ears. After the reconstructions of ossicular chain, the hearing of both ears improved. Case 2 was an 11-year-old girl. The hearing impairment of the right ear was identified in May 2008. She was referred to our university hospital three months later. Pure-tone audiogram showed the conductive hearing impairment in the right ear. CT revealed the incudo-stapedial disconnection in the right ear. The surgery showed the same findings as those of case 1. Anomalies of both cases suggest that the lenticular process of the incus and the stapes originate from a common primordium.

A case report of HTLV-I associated myelopathy presenting with cerebellar ataxia and nystagmus.

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is characterized by spastic paraparesis in the lower extremities, and urinary disturbance. HAM/TSP has also been less frequently associated with cerebellar syndromes and nystagmus. We report a case of HAM/TSP presenting with cerebellar ataxia and nystagmus. The patient was a 73-year-old woman who was born in southern Japan. At age 41, she developed pain and spasticity in the bilateral lower limbs and gradually progressive gait disturbance. At age 57, she was diagnosed with HAM/TSP based on spastic paraparesis in the lower limbs, urinary disturbance and positive anti HTLV-I antibody in serum and cerebrospinal fluid. In June 2008, she was referred to our university and hospitalized for rehabilitation. Twenty days later, she experienced rotatory vertigo sensation. Magnetic resonance imaging revealed pontocerebellar atrophy. The patient presented with cerebellar signs in the upper limbs, gaze-evoked nystagmus in the sitting position and right-beating horizontal nystagmus in the supine and head-hanging positions. Electronystagmography (ENG) showed horizontal saccadic overshoot dysmetria and horizontal saccadic pursuit. Nystagmus is rare among the literature on HAM/TSP. ENG is helpful to evaluate and confirm the cerebellar syndromes of HAM/TSP.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a novel ataxic disorder consisting of the triad of cerebellar impairment, bilateral vestibular hypofunction, and a somatosensory deficit. We report the first Japanese case of CANVAS. The patient is a 68-year-old Japanese male. He was referred to our university for further evaluation of progressive gait disturbance and ataxia. He exhibited horizontal gaze-evoked nystagmus and sensory deficit. Nerve conduction studies showed sensory neuronopathy. Magnetic resonance imaging showed the atrophy of vermis but not of the brainstem. The caloric stimulation and video head impulse test (vHIT) showed bilateral vestibulopathy. The visually enhanced vestibulo-ocular reflex (VVOR) was also impaired. In addition to neurological and electrophysiological examinations, simple neuro-otological examinations (i.e., caloric stimulation, vHIT, and VVOR) may reveal more non-Caucasian cases.

End-tidal CO2 relates to seasickness susceptibility: A study in Antarctic voyages

OBJECTIVE To investigate the relationship between end-tidal CO2 (EtCO2) and seasickness (motion sickness at sea) during an Antarctic voyage. METHODS In this study, we measured EtCO2 and severity of seasickness using the subjective symptoms of motion sickness (SSMS). We sampled EtCO2 and SSMS every 3-4h for 3 days from the date of sail in 16 healthy subjects. This experiment was performed on an icebreaker (standard displacement: 12,650t). RESULTS Since 2 subjects dropped out because of severe motion sickness, available data were collected from 14 subjects. On analysis of all data of all subjects grouped together, there seemed to be a significant negative correlation between EtCO2 and SSMS (R=-0.27, P=0.0005). However, in individual subjects, this correlation was not obvious. During the voyage, EtCO2 level in the seasickness susceptible group was lower than that in the non-susceptible group (P=0.018). Both EtCO2 increasing in the non-susceptible group and decreasing in the susceptible group contribute to the difference in EtCO2 levels. We suggest that the cause of this increase in EtCO2 level in the non-susceptible group was unwitting slow and deep breathing to resist seasickness. CONCLUSION We revealed that for seasickness during an Antarctic voyage, EtCO2 level relates to susceptibility, but not occurrence or severity. Measurement of EtCO2 levels may be useful to identify seasickness-susceptible persons and to efficiently prevent seasickness.

Periodic alternating nystagmus during caloric stimulation

Periodic alternating nystagmus (PAN) is a form of horizontal jerk nystagmus characterized by periodic reversals in direction. We report a case who exhibited transient PAN induced by caloric stimulation. The patient was a 75-year-old male. He had experienced floating sensation in January 2010. Eight months later, he was referred to our university hospital. Gaze nystagmus and positional tests revealed no nystagmus. Only weak right-beating horizontal nystagmus was observed during left Dix-Hallpike maneuver. Electronystagmography showed normal saccadic and smooth pursuit eye movements. The optokinetic nystagmus pattern test was also bilaterally normal. However, during the caloric stimulation to the right ear, at 166 s from the start of irrigation, the direction of nystagmus alternated from leftward to rightward, and thereafter this reversal of direction repeated 15 times. Magnetic resonance imaging showed no significant lesion except for chronic ischemia in the brain. The patient probably had some kind of latent lesion of impaired velocity storage and exhibited transient PAN induced by caloric stimulation. Caloric stimulation is useful and simple examination to disclose latent eye movement disorders of which velocity storage mechanism is impaired.