Brain death: timing of apnea testing in primary brain stem lesion.

In a 73-year-old patient complete areflexia of the cerebral and peripheral nerves following the rupture of an aneurysm of the basilar artery was diagnosed. During apnea testing the spectral analysis of electroencephalography (EEG) revealed an irreversible shift of peak from 6 to 3 Hz within the low-frequency bands. These findings suggest that apnea testing in patients with primary lesion of the brain stem should be carried out only after an isoelectric EEG.

The activity of the sympathetic nervous system following severe head injury.

The activity of the sympathetic nervous system during the course of severe closed head injury has been evaluated in 15 patients by measuring plasma levels of epinephrine and norepinephrine. With the onset of the transition stage from midbrain syndrome to the apallic syndrome the plasma levels mainly of norepinephrine started to increase and remained high during the further course of the disease. During the remission from the apallic syndrome the elevated norepinephrine levels started to decline. The data indicate that a longlasting overactivity of the sympathetic nervous system is a characteristic feature in the course of severe head injury. As a rational therapy to protect the peripheral tissues against the consequences of a longlasting sympathetic overactivity we suggest the use of beta-adrenergic blocking agents and adrenergic neuron blocking drugs.

Endocrine abnormalities in severe traumatic brain injury--a cue to prognosis in severe craniocerebral trauma?

Patients with severe craniocerebral trauma (sCCT) display metabolic and endocrine changes. The question is raised whether hormonal patterns give cues to the prognosis of outcome or not. In 21 patients the function of the adrenocortical, gonadal, thyroid and human growth hormone (hGH)-insulin system was assessed. LH, FSH, TSH, prolactin and hGH were stimulated. 3 groups of patients were formed. Group I: patients in acute phase with a Glasgow Coma Score (GCS) more than 6 (group Ia) and less than 6 (group Ib). Group II: patients in transition to traumatic apallic syndrome (TAS). Group III: patients with full-blown or resolving TAS. The values of group Ia comprised low T3, T4 and testosterone, elevated insulin, normal hGH. Group Ib had hypothyroid T3 and T4 and an attenuated response of LH, TSH, prolactin and hGH to stimulation. Group III: there was seen an endocrine normalisation with elevated T4 and TBG and an altered response of hGH and prolactin to stimulation. Endocrine abnormalities were not helpful in predicting which course, either to better or to worse, a given patient would follow.

Unusual 'spike-wave stupor' in a patient with manic-depressive psychosis treated with amitriptyline.

'Spike-wave stupor' was observed in a 58-year-old male patient with manic-depressive psychosis. Almost continuous atypical spike-wave activity was seen in conjunction with a stuporous episode with stereotyped automatism. Intravenous diazepam ended both the electroencephalographic epileptiform discharges and the clinical stupor. Before and during this episode the patient was treated with an average-dose amitriptyline monotherapy. There was no family history of epileptic seizures. The patient had had electroconvulsive therapy. The history suggests that the analeptic property of amitriptyline induced the 'spike-wave stupor' in this patient.

Recurrent transient global amnesia in a case with cerebrovascular lesions and livedo reticularis (Sneddon Syndrome).

Eight attacks of transient global amnesia were observed in a female patient who suffered from livedo reticularis and a series of other neurological symptoms, which were transient in most stances. The neurological deficits include focal epileptic attacks, unilateral loss of vision, paresis of left arm and/or leg and dysarthria. The first amnestic attack was seen at the age of 19. The episodes lasted from a few to 3 days. The intervals between the amnestic episodes varied between a few days and 11 years. The livedo reticularis became more obvious during each neurological episode and was less pronounced during the time of remission. A benign type of essential hypertension and parproteinemia (gamma-M) was found. The investigations failed to show any evidence of essential thrombocythemia, polyarteriitis nodosa, lupus erythematodes and other immune complex diseases. The underlaying disease remained unclear.

[The pattern of phospholipids during the course of delirium tremens (author's transl)]. / Das Verhalten der Phospholipide während des Delirium tremens

Plasma phospholipids were examined in patients with delirium tremens in the acute phase of the disease and after recovery. The levels of lysolecithin, phosphatidylinositol, phosphatidylethanolamine, sphingomyelin and the total of phospholipids were decreased in comparison to normals. Phosphatidylethanolamine, phosphatidylinositol and the total phospholipids were diminished in comparison to patients with liver cirrhoses. Only in the plasma of patients with liver cirrhoses with bad prognosis (hepatic coma) similar low levels of total phospholipids were found. It is concluded from these results, that the synthesis of phospholipids is reduced during delirium tremens.

Varieties of the locked-in syndrome.

The locked-in syndrome (LiS) was broken down on the basis of neurological symptoms in 12 patients. The criteria of classical LiS are total immobility except for vertical eye movements and blinking. If any other movements are present one should consider the condition as incomplete LiS. Total immobility, including all eye movements, combined with signs of undisturbed cortical function in the EEG led to the concept of total LiS. The anatomical basis for this condition consists of lesions in both cerebral peduncles which interrupt the pyramidal and corticobulbar tracts, the supranuclear fibers for horizontal gaze and the postnuclear oculomotor fibers. As to the course, chronic and transient LiS have been described.

Prostaglandin F2 alpha levels in human cerebrospinal fluid in normal and pathological conditions.

Prostaglandin F2 alpha concentrations in cerebrospinal fluid (CSF) from normal human subjects and patients with various pathological disorders of the central nervous system (CNS) were measured by radioimmunoassay. The mean PGF2 alpha level in 54 controls with no evidence of organic CNS disease was 67 pg/ml (range: 25-150 pg/ml). A significant increase of PGF2 alpha levels was demonstrated in most samples from patients with CNS diseases. Extremely high values were found in patients with stroke and subarachnoid hemorrhage when samples were collected shortly after the cerebral attack. With the regression of clinical symptoms and radiological findings a decrease of PGF2 alpha levels was demonstrated in this group of patients. In 32 patients with cerebral transient ischemic attacks a mean PGF2 alpha concentration of 170 pg/ml (range: 35-355 pg/ml) was found. Increased PGF2 alpha levels were found in patients with epilepsy when samples were collected within a few days after a convulsion. PGF2 alpha levels of four patients with slow progredient forms of multiple sclerosis without clinical symptoms at the time of sample collection were not different from normal controls while the mean PGF2 alpha level of all other patients with multiple sclerosis was 152 pg/ml (range: 55-325 pg/ml). Moreover, increased values could be demonstrated in patients with cerebral tumors and inflammatory processes.

Myotonic myopathy with painful muscle contractions and decrease of symptoms by cold.

Myotonic reaction and paresis accompanied by painful muscle contractions limited to the upper limbs, which decrease remarkably in the cold, were observed in a 29 year old man. The histological investigation revealed minimal non-specific signs of myopathy. The biochemical studies of muscular tissue contained a normal amount of myophosphorylase, acid maltase and glycogen. Ischemic work induced normal elevation of venous lactate. The activities of CPK, LDH and SGOT in the blood serum were occasionally increased. The EMG showed typical myotonic bursts and electrical silence during painful muscle contractions. Repetitive high frequency stimulation demonstrated a clear initial increase of the amplitude of action potentials followed by a decrease in the contracted muscle. The father of the patient suffered from dystrophia myotonica. This coincidnece suggests that this myotonic myopathy is a variant of dystrophia myotonica.

Treatment of Guillain-Barré syndrome by plasma exchange.

Plasma exchange has been used for therapy in eight patients with the Guillain-Barré syndrome. All patients were severely ill. They became tetraplegic and showed cranial nerve involvement. Five patients received assisted respiration, but the others were also at risk of ventilatory insufficiency. Recovery was abrupt in all cases after the first plasma exchanges. Improvement was more marked when plasmapheresis was done on three successive days with plasma exchanges of 2.0-3.01 each in the initial progressive stage of the disease. A considerable advantage of this therapy is the avoidance of continued artificial respiration and nutrition, which both carry the risk of further complications.

Cerebrovascular lesions and livedo reticularis (Sneddon's syndrome)--a progressive cerebrovascular disorder?

Four cases are described in which livedo reticularis was associated with repeated cerebrovascular accidents, which eventually resulted in severe disability in two cases. Patients with severe disability had a history of many years, whereas two patients with little or moderate residual disability had a follow-up of 3 years each. CT scan revealed multifocal cerebral infarctions and cortical atrophy in all cases. Repeated cerebral angiograms, done in three cases, showed no signs of a vascular disease. There were no parameters that pointed to active immunological or inflammatory disorder. Neither clinical evidence of heart or large vessel disease was found. Observations suggest that a so-far unknown progressive cerebral vessel disease associated with livedo is the cause of a steady increase in multiple small cerebral infarctions. Because of the progressive character of the disease the search for effective therapy is needed.

Central somatosensory conduction time and acoustic brainstem transmission time in post-traumatic coma.

Short-latency evoked potentials (SEPs) of the scalp and neck after median nerve stimulation and acoustic brainstem evoked potentials (BAEPs) were recorded in 85 patients in post-traumatic coma with clinical signs of brainstem impairment between days 2 and 6 after trauma. The central somatosensory conduction time (CCT), the amplitude ratio (AR) N20:N13, the interpeak latencies (IPL) I-III, III-V, I-V, and the ARs between waves I and V (I:V) and between wave I and the wave IV/V complex (I:IV/V) were calculated and related to the outcome of the patients. In cases of coma due to supratentorial lesions, CCT and ARs of SEPs were close to normal in patients with good outcome: CCT increased and ARs decreased with worsening of outcome. In cases of primary brainstem injury, a significant prolongation of CCT was also seen in patients with good recovery, whereas normal CCTs could be found in patients with severe disability and death outcome. In this case, unilateral absent scalp SEPs were frequently found. The IPLs I-III, III-V, I-V, and the ARs of BAEPs increased with worsening of outcome. Significant differences of IPL I-V and III-V (brainstem transmission time) were seen between patients with good recovery or moderate disability outcome and the patients with severe disability or death outcome. There was no difference in BAEPs between patients with primary brainstem lesion and patients with secondary brainstem lesion. Patients with bilateral absent SEPs and bilateral absent BAEPs not related to traumatic or preexisting hearing disorders died or survived severely disabled. Unilateral absence of scalp SEPs and unilateral absence of BAEPs were frequently found in patients who died or who had severe disability. Asymmetries in scalp SEPs appeared to be distributed equally to all outcome categories, but asymmetries in BAEPs increased with worsening of outcome too. In most of the patients who died or survived disabled, both SEPs and BAEPs were abnormal.

BRAINDEX: an interactive, knowledge-based system supporting brain death diagnosis.

BRAINDEX (Brain-Death Expert System) is an interactive, knowledge-based expert system offering support to physicians in decision making concerning brain death. The physician is given the possibility of communicating in almost natural language and, therefore, in terms with which he is familiar. This updated version of the system is implemented on an IBM-PC/AT with the expert system shell PC-PLUS and consists of about 430 rules. The determination of brain death is realized with backward chaining and for the optional coma-scaling a forward-chaining mechanism is used.

[Possible malignant hyperthermia as reaction to an overdose of myotonolytic, antidepressive and sedative drugs (author's transl)]. / Fragliche maligne Hyperthermiekrise bei Medikamentenintoxikation.

A 51-year-old male patient with no history of musculo-skeletal or myopathic abnormalities, but suffering from manic-depressive psychosis, attempted suicide with an overdose of dolpersin hydrochloride (Mydocalm), dipenzepine hydrochloride (Noveril), meprobamate (Mepronox) and nitrazepam (Mogadon). He developed high fever, muscle rigidity, tachycardia, arrhythmias, hypotension and mottled cyanosis, symptoms well-known in persons with malignant hyperthermia, an autosomally inherited disease of skeletal muscle. There is also discussed the manifestation and the symptoms of an acute rhabdomyolysis. The diagnosis was confirmed by chemical pathological laboratory findings, including respiratory and metabolic acidosis, myoglobinaemia accompanied by myoglobin diuresis, and elevated creatine phosphokinase (CPK values up to 2790 U/l). Electron microscopic examination of muscle tissue revealed signs of myolysis and mitochondrial reactions with pleoconic hyperplasia. No inhalation anaesthetics or skeletal muscle relaxants, such as succinyl choline, were used in this case. Therefore, malignant hyperthermia might have been induced by a combination of drugs which were not known to induce this abnormal muscular reaction. However, the muscle relaxant effect of dolpersin hydrochloride may have acted as a possible inducer of the attack.

[An 8-finger hand with ulnar duplication (mirror image deformity)]. / Acht-Finger-Hand mit Ulna-Verdoppelung (Spiegelbild-Deformität).

The authors present a congenital arm- and hand deformity in a four year old boy. This "mirror deformity" includes aplasia of the radius, doubled carpus and metacarpus, polydactyly (eight fingers) and aplasia of the thumb. The hypoplastic radial four fingers show brachysyndactyly, the whole upper extremity is hypoplastic but both shoulders are normally developed. The elbow has an extension contraction with a range of movement of 20 degrees in the double olecranons. The wrist has a flexion contracture. At first, pollicisation of the third finger of the hypoplastic radial part of the left hand with resection of three hypoplastic fingers was performed. The thenar muscles were reconstructed with interosseous muscles and the flexion contraction corrected by shortening and doubling of the extensor tendons. Preoperative EMG studies demonstrated two ulnar nerves with cross-innervation of the median nerve. The postoperative situation was recorded again by EMG.

[Electro-neurological correlations in early stages of posttraumatic comatose states. I. The EEG at different stages of acute traumatic secondary midbrain and bulbar brain syndrome (author's transl)]. / Elektro-neurologische Korrelationen in den frühen Phasen des posttraumatischen Komas. I. Das EEG in den verschiedenen Phasen des akuten traumatischen sekundären Mittelhirn- und Bulbärhirnsyndroms.

165 EEG were analysed from 140 patients in posttraumatic coma with secondary traumatic midbrain and bulbar brain syndrome. The EEG-pattern was related to the stage of midbrain or bulbar brain syndrome caused by supratentorial brain shift. There was a close relation between EEG-pattern and the grade of rostro-caudal deterioration. A decrease in the number of different EEG-patterns was associated with increasing intracranial pressure. An unfavourable state of coma was indicated by the disappearance of sleep or sleep-like activities, alternating pattern and loss of reactivity. In deep stages of midbrain syndrome the neurological examination reveals no lateralizing signs at all. The EEG gives the only hint to a local cerebral lesion. EEG abnormalities due to the herniation itself interferred with the EEG-changes due to secondary circulatory, respiratory and metabolic encephalopathies. In such cases the above mentioned regularities were slurred.

[Electro-neurological correlations in early stages of post-traumatic comatose states. II. The EEG at the transition stage to, and at the full stage of the traumatic apallic syndrome (author's transl)]. / Elektro-neurologische Korrelationen in den frühen Phasen des posttraumatischen Komas. II. Das EEG im Ubergang zum und im Vollbild des traumatischen apallischen Syndroms.

The EEG patterns of 36 patients (out of 140 patients with severe brain injury) in the transition stage from midbrain to the traumatic apallic syndrome and/or in the full stage of apallic syndrome are compared with the EEGs in the acute stages of traumatic secondary midbrain syndrome. There is a clear decrease in the variety of EEG-patterns, especially of sleep or sleep-like potentials and alternating patterns, and a loss of rectivity in patients in the alternating patterns, and a loss of reactivity in patients in the transition stage. Therefore these changes not only indicate the increasing rostro-caudal deterioration, but also indicate the increasing rostro-caudl deterioration, but also the transition to the traumatic apallic syndrome. in these cases the EEG-changes mentioned above don't carry a worse prognosis. The reduction of different EEG patterns is less marked in patients in the full stage of the apallic syndrome. The variety of EEg patterns increases by the onset of sleep and waking activity, the appearance of spindles and of long runs of delta.

Carotid occlusion caused by seat belt trauma.

Surgical reconstruction should be considered in patients showing episodes of transient cerebral ischaemia or progressive neurological deterioration secondary to carotid arterial injuries proved by arteriography. Even complete thrombosis of the internal carotid artery may be managed successfully, if thrombectomy and intimal repair are undertaken within some hours after the onset of neurological symptoms. Unger reported a mortality rate of 21% following carotid arterial trauma, 34% of the patients improved if they underwent surgical repair, whereas only 14% of the patients improved if they had ligation or were not treated surgically. Statistical data have shown that both shock and coma are bad prognostic omens and patients presenting with these signs have less than a 50% chance leaving the hospital alive and well, even if they receive optimum emergency management. Although coma has been suggested as a contraindication to carotid repair, review of the literature has shown the data to be too limited to warrant such a conclusion.

Drug induced double peak waveforms in the N20-component of somatosensory evoked potentials.

The double peak waveform of the N20 component of the early somatosensory evoked potentials is a rare finding. In the present paper we investigated 9 patients (mean age +/- SD: 31.3 +/- 11.3 years; range 18-52 years) with the phenomenon of the two subcomponents of the primary cortical complex, after stimulation of the median nerve in a retrospective analysis. The results of the heterogenous patient group showed that the generation of the second subcomponent is not pathognomonic for patients with severe head injury, that it could be reversible and that pharmacological induced effects are presumably responsible for this phenomenon.