Proteus syndrome complicated by multiple spinal meningiomas.

Proteus syndrome is a complex and highly variable disorder comprising malformations and overgrowth of multiple tissues. We present a 65-year-old Japanese man who had multiple spinal meningiomas and accompanying neural symptoms. His right leg showed hypertrophy with cerebriform connective-tissue naevus on the sole, and macrodactyly. Chest computed tomography imaging revealed mild cystic and emphysematous lung changes, which were possibly related to Proteus syndrome. Otherwise, he had no particular cutaneous, musculoskeletal or visceral involvements. Because of the rather insignificant clinical features, he had not been accurately diagnosed in the past and yet had survived to this age. In particular, the presence of spinal meningiomas as an exceptional complication was sufficiently confusing to consider that he had neurofibromatosis. Doctors should be familiar with the diverse clinical pictures of this rare syndrome for its correct diagnosis and proper management.

Intramedullary spinal cord germinoma.

STUDY DESIGN: A very rare case of intramedullary spinal cord germinoma is described. OBJECTIVE: To increase knowledge about the pathogenesis and treatment of intramedullary spinal cord germinoma. SUMMARY OF BACKGROUND DATA: Primary intramedullary spinal cord germinoma is very rare and only two cases have been previously reported. METHODS: A case of intramedullary spinal cord germinoma with complete paraplegia is presented. The diagnosis was determined by biopsy. RESULTS: After local irradiation of 46 Gy, the tumor disappeared completely. CONCLUSION: Irradiation therapy effectively treated this tumor, as shown radiologically, although the patient remained paraplegic.

Thoracic flexion myelopathy. Case report.

A case of thoracic flexion myelopathy is reported in a 36-year-old man. The patient had a history of gradually progressing transverse thoracic myelopathy. Conventional myelography in the neutral position failed to reveal cord compression; however, a severe block of the dye column was evident on studies in the flexed position. Excellent recovery from this condition was obtained with posterolateral fusion of the thoracic spine in the neutral position.

[Clinical application of 18F-FDG-PET in patients with brain death].

In order to evaluate glucose metabolism in brain death, 18F-FDG-PET scans were performed in three patients with clinically highly suspected brain death. One case was caused by head trauma and other two cases were by subarachnoid hemorrhage. All of them were in deep coma without spontaneous breathing, whose intracranial pressure was remarkably elevated up to the level of mean arterial pressure. Nineteen frames of dynamic scan were started soon after intravenous injection of 18F-FDG for one minute per frame, followed by 10 minutes of static scan which started 40 minutes after the injection. Both in dynamic and static scan, no significant intracranial accumulation of 18F-FDG was seen in all of three cases. This finding can be interpreted as the evidence that there is no significant glucose utilization from blood in the brain. This is the first report of clinical application of 18F-FDG-PET to brain death. Our results support the clinical diagnosis of brain death and 18F-FDG-PET can be of value for the assessment of glucose metabolism in patients with suspected brain death.

[Intramedullary neurofibroma in the cervical spinal cord; a case report].

A 62-year-old woman was admitted complaining of clumsiness in both hands. On neurological examination, bilateral hand muscles were weak, both legs were spastic and hyperreflexic, all the extremities were hypoesthetic. Urological examination revealed detruser sphincter dyssynergia. Spinal CT scan demonstrated an iso density mass lesion in the cervical spinal cord, and it was markedly enhanced. On MRI, it was also markedly enhanced by Gd-DTPA. The operative finding was that an oval shaped tumor was buried in the spinal cord and was totally removed. Its histological diagnosis was neurofibroma. Intramedullary neurofibroma is rare and only 18 cases have been reported. In this case the tumor seemed to have arisen at the root entry zone and to have grown sub-pially.

Reversibility of cerebral function assessed by somatosensory evoked potentials and its relation to intracranial pressure--report of six cases with severe head injury.

The critical value and duration of intracranial pressure (ICP) causing cerebral function damage was evaluated in six head injury patients by monitoring the first negative cortical component (N20) of the somatosensory evoked potential (SEP). The SEP was elicited by stimulating the median nerve, and N20 (C3' or C4'-Fz on the affected side) and N13 (C2S-Fz) were monitored serially with a signal processor. The auditory brainstem response (ABR) was simultaneously recorded on the affected side (A1 or A2-Cz). A reversible loss of N20 occurred 7 times in six cases. In all cases, the N20 was restored by emergency decompression or hyperosmolar therapy. The minimum ICP at which N20 disappeared was 30 mmHg, and the N20 was restored when decompression was performed within 4.5 hours. However, when the disappearance persisted for more than 1.5 hours, the N20 latency was markedly prolonged after restoration. These changes appeared before the ABR showed definite abnormalities. These results show that the cerebral function may be damaged when ICP exceeds 30 mmHg, and that emergency decompression is required within 4.5 hours, preferably within 1.5 hours, to restore cerebral function. This critical ICP and duration should be of clinical value in patient management.

[Homicidal poisoning by aconite: report of a case from the viewpoint of clinical forensic medicine].

A case of homicidal poisoning by aconite is reported from the viewpoint of clinical forensic medicine and analytical chemistry. Jesaconitine was detected in the vomitus, stomach contents, plasma and urine at concentrations of 32.2, 5.48, 0.433 and 1.07 micrograms/ml, respectively. The total amount of jesaconitine in the stomach contents was 1.3 mg. Macroscopic autopsy revealed hemorrhagic pulmonary edema, and histologically, diffuse contraction-band necrosis was evident in the myocardium. Exogenous administration of adrenaline or endogenous release of catecholamines are known to induce hemorrhagic pulmonary edema and contraction-band necrosis, and aconitine is reported to cause release of endogenous catecholamines centrally and to induce neurogenic pulmonary edema and ventricular-type arrhythmia. Therefore, the chief cause of death in this case was considered to be aconite-induced centrogenic arrhythmia.

Measurement of serum alkaline phosphatase isozyme I in brain-damaged patients.

The authors measured alkaline phosphatase isozyme I (ALP-I) in sera of 24 brain-damaged patients and four with disorders other than brain damage. The study population comprised three patients with postresuscitation encephalopathy, four with ruptured cerebral aneurysms, 14 with acute subdural hematoma and cerebral contusion, and three with nontraumatic intracerebral hemorrhage. ALP-I detected in brain damage is physicochemically different from the other known ALP-Is that appear in patients with obstructive jaundice or hepatoma. In the brain-damaged patients, ALP-I became elevated about 7 days after admission and markedly increased as secondary brain damage developed. Excluding patients who died within 9 days of admission, the maximum serum ALP-I concentration was well correlated with the functional outcome. In cases in which barbiturate therapy was effective, the appearance of ALP-I was delayed and its elevation was suppressed. The results of this study suggest that measurement of serum ALP-I is useful not only in the management but also in predicting the prognosis of brain damage.

[Analysis of angiographical findings in patients with severe brain disorders progressing to brain death].

In the present study, the authors analysed the serial angiographical findings progressing to brain death and their relation to the intracranial pressure (ICP) and the cerebral perfusion pressure (CPP). Seventy two patients, from four to eighty four years old (fourty six males and twenty six females) admitted in the Department of Emergency Medicine, University of Tokyo Hospital during the period from January, 1981 to April, 1986, were studied. Their underlying diseases were supratentorial primary brain lesions except two cases with asphyxias which progressed to brain death. ICP was continuously measured and CPP was calculated as the pressure gradient between the mean arterial blood pressure (MAP) and ICP. The direct carotid angiography was performed to follow the cerebral circulation. Fourty five patients were subjected to barbiturate (pentobarbital sodium) therapy. The degree of the intracranial filling staged as "Non-filling", "Siphon-filling", "Partial-filling", "Delayed-filling", "All-filling" correlated significantly with ICP and CPP. These relationships, however, disappeared once ICP exceeded MAP. When "Non-filling" angiogram was obtained, clinical signs had already showed brain death. On the other hand, minimal flow ("Siphon-filling", "Partial-filling", "Delayed-filling") were still demonstrated in six brain death cases while ICP was approaching its "peak" value. This study showed that clinical diagnosis of brain death preceded the Non-filling phenomenon, suggesting that, for the demonstration of the cerebral circulatory arrest, the angiograms should be performed after the clinical diagnosis of brain death was established and CPP became zero. The evaluation of the extremely slow and minimal filling is still a matter of discussion.(ABSTRACT TRUNCATED AT 250 WORDS)

Sequential changes of auditory brain stem responses in relation to intracranial and cerebral perfusion pressure and initiation of secondary brain stem damage.

The relationship of supratentorial intracranial pressure (ICP) and cerebral perfusion pressure (CCP) with serial changes in auditory evoked brain stem responses was investigated. Eighty-one patients without primary brain stem damage admitted to our emergency unit were studied. When ICP over 50 mm Hg persisted for 4 hours, the I-V interpeak latency was significantly prolonged. The threshold of this prolongation was 8 hours for the ICP over 45 mm Hg and 24 hours for that of over 40 mm Hg. The ICP of 35-40 mm Hg for 24 hours was the border zone. CCP did not show a significant relation with I-V interpeak latency changes. The loss of wave V was observed in a wide range of the ICP (30-147 mm Hg) and CPP (0-60 mm Hg). Wave III disappeared when the ICP exceeded 50 mm Hg. Wave I became undetectable with an ICP above 50 mm Hg or a CPP below 40 mm Hg. These results indicate that an increase of ICP over 40 mm Hg definitely initiates secondary brainstem dysfunction if it lasts for more than 24 hours and that the ICP should be reduced below this level, preferably below 35 mm Hg, to maintain brain function. The fact that both low CPP and high ICP were involved in the loss of wave I clearly shows that both ischaemia and displacement of the brain stem are the important pathophysiological factors for the disappearance of wave I.

[Clinical significance of serial monitoring of short-latency somatosensory evoked potential in head injury with special reference to intracranial hypertension].

Short-latency somatosensory evoked potential (SEP) was serially monitored in head-injured patients and its clinical significance was evaluated in relation to auditory brain stem response (ABR), prognosis and other parameters such as intracranial pressure (ICP). SEP, ABR, and ICP were monitored serially and automatically in 16 patients with acute head injury. Glasgow Coma Scale (GCS) score was equal to or less than 8 in 14 cases. High dose barbiturate therapy was performed in 7 cases and intracranial hematoma was removed operatively in 14 cases. SEP was recorded from C3'or C4'-Fz leads on the lesion side following stimulation of the contralateral median nerve, and the difference between the two potentials (N20, N13) defined as central conduction time (CCT) was followed at a basic time interval of 10-20 min. The patients were divided into 3 groups according to N20 finding and the prognosis of each group was analyzed. The final outcome (assessed 6 months after injury) was graded according to Glasgow Outcome Scale as follows: good (good recovery, moderate disability), poor (severe disability, persistent vegetative state), death (death). Three patients with no CCT change showed good recovery (table, 11). Out of 7 patients with no N20 on admission, 5 died due to severely increased ICP and the prognosis for the other 2 cases was poor. The last group consisted of 6 patients in whom CCT varied during hospitalization. In 3 out of these 6 cases N20 finally disappeared due to increased ICP and they died in the acute stage.(ABSTRACT TRUNCATED AT 250 WORDS)

[Myelopathy caused by hypertrophy of the posterior longitudinal ligament (HPLL): case report].

A case of myelopathy caused by hypertrophy of the posterior longitudinal ligament at the cervical spine is reported. A 71-year-old man was hospitalized with myelopathy with progressed during the last two months. Plain X-ray of the cervical spine revealed only mild spondylotic change. Myelography demonstrated completely blocked contrast medium at C4 to C5 vertebral height due to epidural mass. This epidural mass was localized ventral to the spinal cord from C3 to C5. CT scan revealed slightly high density area behind the vertebral body. On surgery, no evidence of disc fragment but only hypertrophied posterior longitudinal ligament which was removed was found to be a cause of cord compression. Excellent recovery of neurological symptoms after operation was obtained. On histological study, remarkable hypertrophy, edema, hyalinoid degeneration and a little calcification of the ligament was found, which did not show any evidence of new bone formation. HPLL was characterized by thickened and broad epidural mass in the ventral side of cervical cord with Hounsfield number of 90-156 on CT scan, which is not very easy to differentiate from metastatic epidural tumor. The cause of HPLL is not well elucidated, though, this may be a new category of compression myelopathy.