Meeting reports

Scientific meeting of the Netherlands Society of Neurology held in Utrecht, 28th March 1987.

Traumatic brachial plexus lesions

A.C.J . Slooff (Heerlen)

A review is given on the aetiology of 199 brachial plexus lesions. We analysed the cause and clinical details of 136 traumatic lesions. For this purpose we excluded the specific group of obstetrical and iatrogenic lesions.

This analysis resulted in a classification of the lesion, from which indications for treatment are derived.

The age varies from 2-67 years old; 91 patients are below the age of 25. There is a remarkable preponderance of males: 115/136. A high frequency is registrated for traffic acci- dents: 98/136; shoulder (fracture-)dislocation in domestic or sportive environments counts for 22/136. The amount of industrial lesions, stab- wounds and gunshot lesions is 12/136. In 4 pa- tients the exact cause was not described.

The number of associated lesions (cra- niocerebral, humerus, clavicula, costal and vascular) is impressive. We counted 60/136 multitraumatised patients.

The classification is based on the nature and type of the injury, the amount of neurological deficit and the assumed rate of the anatomical and histological lesion.

The indications for surgical treatment are evi- dent for open lesions and for closed injuries with

vascular insufficiency, large compressing hematoma or bony compression.

We propagate to operate the severe lesions (grade 4 and 5-Sunderland), avulsions and pa- tients with no or retarded recovery after 3-4 months. Severe and early pain is highly suspect for an avulsion and this is an indication for surg- ery. For an uncertain degree of the lesion we prefer to explore.

We performed reconstructive neurosurgery in 64 patients, consisting of 73 surgical pro- cedures: nerve transplants (free, pediculed or vascularised), neurotisations (intercostal nerves, cervical or accessory nerves), neurolysis or nerve transposition.

These are time-consuming, longlasting and demanding procedures with a difficulty in assessing the extension and degree of the lesion and evaluating the surgical possibilities.

Our experience is that these procedures are rewarding: gaining limited but useful function and treating the pain syndrome.

The neurosurgery of the plexus is only a part of the total treatment of the patients. A team of rehabilitation specialists, orthopedic and handsurgeon are working together to find the appropriate treatment including the limitations and priorities of functional recovery.

Cavernous angiomas, diagnostic and therapy

P.A.W. Verhoeven (Tilburg)

Intracranial cavernous angiomas are vascular malformations, and have to be distinguished

from capillary teleangiectasias, the venous and the arteriovenous malformations.

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Cavernous angiomas are well circumscribed and composed of thin walled sinusoidal spaces, lined with endothelium. They have, in contrast to the capillary teleangiectasias, no intervening neural tissue. Cavernous angiomas may increase in size: the most plausible mechanism of growth involves hemorrhage. Small hemorrhages followed by organization, fibrosis and calcification probably account for their slow progressive growth. Essentially all cavernous angiomas show microscopic evidence of repeated hemorrhage.

In 1985 and 1986 in the Elisabeth Hospital Tilburg, the Netherlands, eight patients with an intracerebral cavernous angioma were treated. The clinical aspects are illustrated with the case histories of these 8 patients. The diagnosis was

based on CT and angiography in three cases, and on CT and angiography and MRI scan in five cases. The treatment of choice is neurosurgical extirpation. We believe it is worthwhile to consider radiation therapy, when clinical or radiological deteriora- tion is seen. In one case with a cavernous angioma in a surgically inaccessible location, we treated the patient, with a progressive hemiparesis, with radiation therapy, with ex- cellent result. This cavernous angioma was localized in the right paraventricular parietal lobe. Three months after radiation therapy, control CT scan did not show the cavernous angioma any more and repeated MRI scan showed an old infarct. The progression of the pa- tient's hemiparesis stopped.

N e u r o m y e l i t i s opt ica

J .H. Begeer, M.C. Hoogstraten, L.M.

Seven patients with neuromyelitis optica (Devic's Disease) are reported. Their signs and symptoms differed only slightly from what is mentioned in 24 patients collected from recent literature.

With the exception of one patient, all our patients were treated with corticosteroids. Compared with the recent literature our group

Vencken, and A.E .J . de Jager (Groningen)

of patients differed in two aspects: - none of our patients died - two of our patients became corticosteroid

dependent. Magnetic Resonance Imaging was performed

in 5 out of 7 patients. No abnormalities were found in myelum and brain. In one patient, however, a lesion was found in the optic nerve.

T h e k n e e j e r k in l ow l u m b a r disc h e r n i a

J.N. H a m b u r g e r , D. Moffie, H.L. Hamburger (Amsterdam)

During the years 1976-1985, 604 patients with signs of low lumbar prolapsed disc where ad- mitted to the neurologic department of the Municipal Hospital Slotervaart in Amsterdam. A large number of these patients with a low lumbar prolapsed disc displayed a diminished knee jerk on the side of the lesion. This symptom is scarcely mentioned in current neurological text books. In these patients spe- cial attention was paid to the knee and ankle

jerks, in relation to clinical signs, EMG, lumbar myelography and pre-operative findings. The number of patients was calculated with a com- bination of a diminished knee jerk and

1. herniated disc L4-L5 and/or L5-$1, and/or 2. congenital or degenerative stenosis of the

lower lumbar canal. A comparison has been made with the find-

ings in recent neurological studies, with surpris- ing results. Etiological factors are discussed.

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Topographical location of cerebral damage in AIDS patients

D. Troost , M.E.I . Schipper, J. de Gans, and H.J. Houthof f (Amsterdam)

The brains of 33 patients with acquired immuno deficiency syndrome (AIDS) were examined. Brain pathology was present in almost every patient, also in those which had no clearcut neurologic dysfunction. Opportunistic infect- ions included Toxoplasma gondii abcesses, cryptococcal meningitis, progressive multifocal leukoencephalopathy, cytomegalovirus infect- ion and a case of possible coccidioidomycosis infection. The opportunistic infections, present in 39% (13/33) of the patients, often masked the more sophisticated lesions in the cerebral white matter and certain brain stem nuclei. These le- sions are probably due to direct effect to the HIV virus upon the CNS. Compared to normal controls, the inferior olive, dentate nucleus and less severely so the red nucleus and the substan- tia nigra showed neuronal loss and reactive gliosis. Other lesions of the brain stem were seen only sporadically. White matter lesions were found in all patients except one. The white

matter lesions ranged from mild vacuolation of myelin with some macrophages to severe vacuolation and complete demyelination. The patients without neurologic dysfunction (19/33, 58%) showed histologically mild and diffuse brain lesions, and the previously mentioned brain stem lesions. Demented AIDS patients without opportunistic infections in the brain showed mainly white matter lesions and brain stem lesions. In 9 patients destruction of certain tracti was noted in addition to the damage in the brain stem nuclei and other lesions.

Neural transport of HIV virus is possible and can probably be held responsible for the destruction and demyelination within the anatomical boundaries of certain tracti. Trans- port of the HIV virus along the bloodstream in combination with axonal transport of the virus is probably responsible for the slow dementing process which strikes many AIDS patients.

Pre-evaluation for surgical treatment of medical refractory seizures

R.M.Chr . Debets , C.W.M. vanVee len , W. van E m d e Boas, A.C. van Huffelen (Heemstede , Utrecht)

Surgery for epilepsy has been performed since 1936 in the Netherlands. In 1972 De Vet de- scribed the results of temporal lobectomy in 45 patients. These patients, operated between 1949 and 1969, benefited up to 77,5% and became seizurefree or had only sporadic seizures. The site and the size of a cortical resection was derived from presurgical EEG recordings, seizure semiology and acute corticography pre- ceding the resection performed under local anaesthesia.

With stringent criteria only a small proportion of patients with farmacoresistant partial epilepsy are suitable candidates for surgical therapy. In order to improve the effectiveness of the presurgical strategy, to detect a well localised epileptic focus or region, a stepwise procedure is developed. After a standard neurological investigation all patients undergo

neuropsychological examination, magnetic resonance imaging and positron emission tomography before implantation with subdural and intracerebral electrodes for ictal EEG-vid6o monitoring.

Since 14 years we have used a specially designed method of subdural and depth multielectrode recording. The electrodes are in- serted through central burrholes bilaterally over the convexity of the frontal, parietal and tem- poral lobes, and can be manipulated:to reach basal parts of the frontal and temporal lobs. With these subdural electrodes only a restricted number of depth electrodes is necessary, After pre-evaluation 12 patients underwent a temporal or frontal resection. Seven patients are seizure free, 2 have markedly reduced seizures and three patients continued to have epileptic fits. No patient suffered functional im- pairment after operation.

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Lissencephaly type I

J.F. de Rijk-van Andel (The Hague) , J.P. Slooff (Nijmegen), M.C.B. Loonen (Rot- terdam), P.H. Hoogland (The Hague) , W.F.M. Arts (The Hague)

Lissencephaly type I is a major manifestation of Miller-Dieker syndrome (MDS) but also occurs as an isolated developmental defect (Isolated Lissencephaly Sequence = ILS). Only 35 well- documented patients have been reported in literature, of which 25 were MDS patients and 10 ILS patients. All patients are severely retarded, and most of them die early.

We present here a series of 17 patients with lissencephaly type I. The correct diagnosis was

obtained by computed tomography (CT) in 15 patients and by autopsy in 6.

Lissencephaly type I is often missed by CT. Optimal image quality is required for adequate visualisation of the sulci. But even then lissen- cephaly is frequently overlooked and dif- ferentiation from polymicrogyria and schizen- cephaly may be difficult. Magnetic resonance imaging seems to provide the most reliable results.

Sixteen of our 17 patients had an ILS indicat- ing that the incidence of this disorder has been underestimated in the past. An important find- ing was that ILS is not always associated with epilepsy.

Application of non-autoradiographic (in situ) hybridization for the diagnosis of human viral neurological disease

R.H. Boerman, A.K. Raap , and A.C.B. Peters (Leiden)

With respect to diagnosis, the most challenging viral infection of the human CNS is H ~ s Simplex type-1 (HSV-1) encephalitis. So far, no reliable non-invasive radiological or virological technique has been developed to diagnose HSV-1 encephalitis during its early clinical stages. At present the only conclusive way to diagnose HSV-1 encephalitis is by isolation of the virus from a biopsy. Detection of viral nucleic acids in CSF by filter hybridization may provide a non-invasive alternative to operative procedures. In addition, detection of viral genomes in morphologically intact biopsy or autopsy tissue sections by in situ hybridization will also contribute to diagnosis and insight into pathogenesis.

We have applied both radioactive and non- radioactive hybridization techniques for this purpose. With the newly developed non- radioactive acetyl aminofluorene (AAF) in situ hybridization technique positive results were

obtained on routinely processed paraffin sec- tions of brain tissues from three patients with proven HSV-1 encephalitis using cloned HSV-1 DNA.

Furthermore, in two samples of CSF from a patient with a proven HSV-1 encephalitis, taken 2 and 11 days after hospitalization, HSV-1 DNA was detected by autoradiographic filter hybrid- ization using cloned HSV-1 DNA labelled with 32P.

Also in another neurological disease with a viral etiology, progressive multifocal leucoen- cephalopathy, the application of non- autoradiographic in situ hybridization with AAF-labelled probes proved to be of value. JC virus DNA was readily detected in glia cells present in brain biopsy material from one and autopsy material from a second patient.

These results show the potential of these hybridization techniques for rapid viral diagnosis.

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