E. Rumpl

Varieties of the locked-in syndrome

SummaryThe 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.ZusammenfassungAnhand der klinischen Symptome von 12 Patienten wurde das Locked-in-Syndrom (LiS) in das klassische LiS mit vollständiger Bewegungslosigkeit, ausgenommen vertikaler Blickbewegungen und Blinzeln, und in das inkomplette LiS mit weiteren Bewegungsresten unterteilt. Bei absoluter Immobilität mit Einschluß aller Augenbewegungen, jedoch Zeichen für ein erhaltenes Bewußtsein im EEG, wurde ein totales LiS angenommen. Die anatomische Grundlage für diese Form sind bilaterale Läsionen in den Pedunculi cerebri mit einer Unterbrechung der Pyramidenbahn, der corticobulbären Bahnen, der zentralen Fasern für horizontale Blickbewegungen und der postnukleären Oculomotorius-Fasern. Als Verlaufsvarianten wurden das chronifizierte und das transiente LiS unterschieden.

Central somatosensory conduction time and short latency somatosensory evoked potentials in post-traumatic coma.

Short latency evoked potentials (SEPs) were elicited by stimulation of the median nerve at the wrist and recorded simultaneously from the neck and the contralateral scalp in 44 comatose patients with signs of brain stem impairment due to head injury. Thirty-four patients were studied in acute coma on day 1 or 2 after brain injury. Twenty-three patients were studied in prolonged coma during days 3-12 after trauma. Six patients were examined in brain death. Brain stem involvement was divided clinically and by CT scan into secondary lesions due to supratentorial mass displacement and primary lesions due to direct violence to the brain stem. The central somatosensory conduction time (CCT) was measured by subtracting the peak latency of the major response from the neck (N14) from that of the primary scalp response (N20). The amplitude ratios (ARs) N20/N14 were calculated for each trace. Further asymmetries and absence of SEP over one or both hemispheres were noted. In cases in coma due to supratentorial lesions CCT and AR were close to normal in patients with good outcome. CCT increased and AR decreased with the worsening of outcome both in acute and prolonged coma. Asymmetries of SEPs indicated moderate or severe final disability. Patients with absent SEPs over one or both hemispheres due to supratentorial lesions died or survived severely disabled (1 case). In patients suffering from primary brain stem dysfunction, confirmed by a normal or slightly abnormal CT scan, prolonged CCT, asymmetric but also absent SEPs were also found in patients with good outcome both in acute and prolonged coma. AR was generally low in these cases. Early appearance of SEPs or early recovery of initially distorted SEPs and decrease of CCT in prolonged coma or during recovery was a favourable prognostic sign. Therefore even absent or severely distorted SEPs should be interpreted cautiously in patients who may suffer from primary brain stem involvement. Scalp SEPs were totally absent in patients with brain death.

Prostaglandin F2? levels in human cerebrospinal fluid in normal and pathological conditions

SummaryProstaglandin F2α 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 PGF2α level in 54 controls with no evidence of organic CNS disease was 67 pg/ml (range: 25–150 pg/ml). A significant increase of PGF2α 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 PGF2α levels was demonstrated in this group of patients. In 32 patients with cerebral transient ischemic attacks a mean PGF2α concentration of 170 pg/ml (range: 35–355 pg/ml) was found. Increased PGF2α levels were found in patients with epilepsy when samples were collected within a few days after a convulsion. PGF2α 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 PGF2α 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.ZusammenfassungMittels Radioimmunoassay wurden PGF2α-Konzentrationen im Liquor cerebrospinalis von normalen Personen und von Patienten mit verschiedenen Erkrankungen des ZNS gemessen. Der mittlere Prostaglandin-F2α-Spiegel von 54 Normalpersonen betrug 67 pg/ml (Bereich: 25–150 pg/ml). Bei den meisten Erkrankungen des ZNS unseres Patientengutes konnten deutliche Erhöhungen der PGF2α-Konzentration im Liquor cerebrospinalis festgestellt werden. Auffallend hohe Werte fanden wir bei Patienten mit ischämischen und hämorrhagischen Insulten als auch bei Subarachnoidalblutungen, wenn die Liquorabnahme innerhalb weniger Tage nach der Attacke erfolgte. Mit zunehmender Besserung des klinischen und röntgenologischen Befundes zeigte sich bei dieser Krankheitsgruppe eine rasche Normalisierung der PGF2α-Konzentration im Liquor. Bei 27 Patienten mit transitorisch-ischämischen zerebralen Attacken fanden wir einen mittleren PGF2α-Spiegel von 170 pg/ml (Bereich: 35–355 pg/ml). Bei Patienten mit Epilepsie zeigte sich ein deutlicher Anstieg der PGF2α-Konzentration in Zusammenhang mit Krampfanfällen mit Normalisierung der Werte nach längeren, anfallsfreien Phasen.Bei 4 Patienten mit einer milden Verlaufsform einer Multiplen Sklerose ohne besondere klinische Symptomatik zum Zeitpunkt der Liquorabnahme wurden normale PGF2α-Spiegel gefunden, während der mittlere Spiegel aller 10 Patienten mit Multipler Sklerose mit 152 pg/ml doch deutlich erhöht war. Hohe PGF2α-Konzentrationen fanden wir auch im Liquor von Patienten mit Tumoren des ZNS und Meningitis bzw. Meningoenzephalitis.

Incidence and prognostic value of spindles in post-traumatic coma

One hundred and thirty-three EEGs were analysed from 80 comatose patients with signs of brain stem impairment due to head/brain injury. Seventy EEGs were taken in acute coma on day 1 or 2 after brain injury. Sixty-three EEGs were recorded in prolonged coma 3-12 days after brain injury. Brain stem involvement was divided by neurological signs and by CT scan into secondary lesions due to supratentorial mass displacement and primary lesions due do direct violence to the brain stem. Different EEG patterns were observed, but spindle activity was of special interest. Spindles were classified as typical (easily recognizable, well organized, 12-14 c/sec activity) or atypical (hardly recognizable, distorted form, 6-11 c/sec activity). Furthermore, asymmetries of spindles were noted. The spindles and their alterations were related to different stages of outcome. Spindles were seen in 91% of the EEGs in acute coma and in 30% in prolonged coma. In acute coma due to secondary brain stem involvement a good outcome was heralded by the occurrence of typical symmetrical spindles combined with early stages of secondary brain stem impairment at neurological examination. In cases of primary brain stem involvement typical spindles also suggested a good prognosis despite the observation of serious clinical signs (decerebrate posturing). The percentage of spindle activity decreased, and distortion and asymmetry of spindles increased with the worsening of outcome. Severe intracerebral lesions (confirmed by clinical and CT scan examinations) led to distortion, asymmetry and finally disappearance of spindles. In prolonged coma spindle activity was markedly reduced regardless of the final outcome. When spindles were present atypical and asymmetric forms significantly increased in patients with bad outcome. There were no significant differences in spindle activity in the different outcome categories, if primary and secondary brain stem lesions were compared.

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

SummaryFour 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.

Recurrent transient global amnesia in a case with cerebrovascular lesions and livedo reticularis (sneddon syndrome)

SummaryEight 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 paraproteinemia (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.ZusammenfassungAcht Episoden einer transitorischen globalen Amnesie wurden bei einer Patientin beobachtet, bei der eine Livedo reticularis auch mit einer Reihe anderer, meist reversibler neurologischer Ausfälle verbunden war. Diese setzten sich aus fokalen epileptischen Anfällen, einseitigem Visusverlust, Paresen der linken oberen und/oder unteren Extremität und dysarthrischen Störungen zusammen. Die erste amnestische Episode trat im Alter von 19 Jahren auf. Die Episoden hielten meist nur wenige Stunden, einmal aber auch drei Tage an. Die Zeitabstände zwischen den einzelnen Attacken lagen zwischen wenigen Tagen und 11 Jahren. Die Ausprägung der Livedo reticularis verstärkte sich in den Perioden mit neurologischen Ausfällen und nahm in den Rückbildungsphasen ab. Die Laboruntersuchungen ergaben eine benigne Form einer essentiallen Hypertonie und Paraproteinämie (Gamma-M). Hinweise für eine essentielle Thrombozytämie, eine Polyarteriitis nodosa, einen Lupus erythematodes oder für andere Immunkomplex-Erkrankungen waren nicht zu erheben. Die Ursache der Symptomenkombination blieb ungeklärt.

Treatment of Guillain-Barré syndrome by plasma exchange

SummaryPlasma 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.ZusammenfassungDie Wirksamkeit einer Plasmaaustausch-Behandlung wurde bei 8 Patienten mit einem Guillain-Barré-Syndrom geprüft. Alle Patienten hatten einen schweren Krankheitsverlauf gezeigt, mit schlaffen Tetraparesen und multiplem Hirnnervenbefall. Fünf Patienten mußten assistiert beatmet werden. Auch bei den anderen Patienten zeigten sich Hinweise für eine drohende respiratorische Insuffizienz. Nach den ersten Plasmapheresebehandlungen konnte eine abrupt einsetzende und deutliche Besserung der Ausfälle beobachtet werden. Die klinische Besserung verlief besonders eindrücklich, wenn der Plasmaaustausch an drei aufeinanderfolgenden Tagen mit einer Austauschmenge von jeweils 2,0–3,01 und in der initialen progressiven Phase der Erkrankung erfolgte. Ein großer Vorteil dieser Therapie dürfte in der Vermeidung langzeitiger künstlicher Beatmung und Ernährung und den damit verbundenen Komplikationen liegen.

Unusual ‘Spike-wave stupor’ in a patient with manic-depressive psychosis treated with amitriptyline

Summary‘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.ZusammenfassungWährend eines Stupors mit stereotypen Bewegungsabläufen und Reihensprechen, der im Rahmen einer depressiven Phase eines an einer manisch-depressiven Psychose erkrankten 58jährigen Patienten auftrat, wurde eine nahezu kontinuierliche unregelmäßige spike-wave- und sharp-wave-Aktivität im EEG abgeleitet. Der „spike-wave-Stupor“ konnte durch eine intravenöse Applikation von Diazepam in seiner klinischen und elektroenzephalographischen Ausprägung rasch unterbrochen werden. Vor und während der stuporösen Attacke wurde der Patient mit Amitriptylin als Monotherapie in üblicher Dosierung behandelt. Die Eigen- und Familienanamnese ergab keinen Hinweis auf das Vorliegen einer Epilepsie. Epileptische Anfälle hatte der Patient nur aus therapeutischen Gründen im Rahmen einer Elektroschocktherapie. Die Analyse des Falles läßt in der analeptischen Wirkung von Amitriptylin die Ursache für das Auftreten des „spike-wave-Stupors“ erkennen.

Phospholipids in human cerebrospinal fluid

SummaryPhospholipid fractions in cerebrospinal fluid of normal persons were examined by separation with thin layer chromatography. 100 ml of cerebrospinal fluid were found to contain 22±4 μg phospholipid phosphorus, on the average consisting of 12% lysolecithin, 20% sphingomyelin, 42% lecithin, 2% lysocephalin, 3% phosphatidylinositol, 2% phosphatidylserine, 9% phosphatidylethanolamine. 4% of the total phospholipids remained at the origin of the chromatograms, the rest was recovered in yet unidentified fractions.ZusammenfassungPhospholipidfraktionen wurden im Liquor cerebrospinalis von Normalpersonen durch Auftrennen mit Dünnschichtchromatographie bestimmt. In 100 ml Liquor wurden 22±4 μg Phospholipidphosphor gefunden, davon 12% Lysolecithin, 20% Sphingomyelin, 42% Lecithin, 2% Lysokephalin, 3% Phosphatidylinositol, 2% Phosphatidylserin und 9% Phosphatidyläthanolamin. 4% wurden an der Auftragsstelle gefunden, der Rest in nicht identifizierten Fraktionen. Mit der angewandten Methode war es also möglich, mehr Phospholipidfraktionen als bisher angegeben aufzutrennen.

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.