The internal spinal cord blood flow was measured in dogs at the site of local cooling using hydrogen polarography. Blood flow decreased to 50% of the normothermic values during cooling of the cord to a central temperature of 16 degrees Celsius. Upon cessation of cooling internal blood flow rapidly returned to normal values. Implications of this finding for the treatment of spinal cord injury are discussed.

Since Binswanger’s description of subcortical arteriosclerotic encephalopathy in 1894, numerous cases have been reported. Several authors doubt the validity of this malady, although the majority consider it to be a disease entity. We report seven cases with this type of pallor of myelin, only two of which are accompanied by a history of dementia. Among the seven cases, two had arteriosclerosis of penetrating arteries and arterioles in cerebral white matter. Electron microscopy showed splitting of myelin sheaths, probably the result of edema. In reviewing the blood supply of the cerebral white matter, we conclude that no pathological alterations of medullary branches of the cerebral arteries, the same vessels supplying the white matter, can give rise to such diffuse pallor of white matter and spare the arcuate fibres. This pallor can only be due to cerebral edema, most likely of hypoxic-ischemic, hypotensive, or acidotic origin. We also contend that arteriosclerosis can only cause dementia through multiple infarcts or lacunae, if it indeed leads to dementia.

A retrospective study of neonatal seizures in a tertiary care neonatal intensive care unit determined a 3.2% incidence, and confirmed the relatively poor efficacy of the traditional anticonvulsants phenobarbital and phenytoin. Only 33% responded to an initial adequate loading dose of phenobarbital, while 56% responded to either or both anticonvulsants. Although multifocal clonic seizures were most common (42%), tonic seizures were next in freqency (30%). Tonic seizures which did not respond to phenobarbital responded quite poorly to the addition of phenytoin compared to other seizure types. Tonic seizures may be the result of brainstem release phenomena and require a different strategy for management. Among nonresponders in this study, there was a 56% mortality rate but only 33% of responders died. There is a critical need for studies to find more efficacious agents than phenobarbital and phenytoin to treat seizures in the newborn.

We studied multimodal evoked potentials (EPs) longitudinally in a series of children with Friedreich’s ataxia and ataxia telangiectasia to determine both their diagnostic utility and their correlation with clinical regression.

The auditory brainstem responses (ABRs) were abnormal only in the children with Friedreich’s ataxia. The abnormality seen in these patients was a rostral-caudal loss of the ABR waves. The visual EPs (VEPs) were abnormal in many of the patients; those with ataxia telangiectasia had unusually low amplitude or absent VEPs, occasionally with increased latencies, whereas those with Friedreich’s ataxia had normal amplitude VEPs, often at increased latencies. The somatosensory EPs were usually of increased latency or absent in these patients. Unlike the ABR and VEPs, they did not serve to differentiate the groups.

Changes in the EPs appeared to reflect clinical deterioration; patients with little change in their EPs over several years were regressing very slowly, whereas others had rapid deterioration in both EPs and clinical status. We suggest that the EPs are diagnostically of value in degenerative ataxias and may be of value in monitoring these patients and their response to therapy.

We have examined the distribution of HLA antigens in 70 multiple sclerosis (MS) patients divided in three groups defined according to clinical criteria: benign MS, severe MS, cerebellar MS. We have found a significant association between severe MS and HLA-DR2, and between benign MS of more than 15 years of evolution and HLA-DR3. We review previous work along the same line and conclude that the association of HLA antigens with “clinical subgroups of MS” could indicate a genetically based heterogeneity of the disease and offer help in establishing a prognosis.

Does the human fetal brain influence muscle development? The importance of suprasegmental cerebral influence on developing human fetal muscle is less well understood than the control of histochemical differentiation of muscle by the motor neuron. Muscle biopsies of 21 hypotonic infants and children with nonprogressive congenital lesions of the cerebellum and/or brainstem were studied by histochemical methods. Two neonates who died with severely dysplastic brains and no descending motor tracts had normal muscle. The others, particularly those with cerebellar hypoplasia, had delayed muscle maturation, selective predominance of type I or II muscle fibres or disproportion in fibre sizes. It is concluded that the motor unit is capable of developing normally without suprasegmental influence, but that an abnormal balance of descending impulses may alter histochemical differentiation of fetal muscle. The small ‘subcorticospinal’ pathways arising in the brainstem probably are more influential than the larger corticospinal tract because of later myelination in the latter. The muscle biopsy thus serves to provide evidence of suprasegmental disease in infantile hypotonia.

This paper describes four patients with thoracic spinal lesions in whom the initial clinical presentation was highlighted by complaints in the lower back and lower extremities, in the absence of thoracic spinal or radicular symptoms. Initial myelography, confined to the lumbar region, failed to reveal a cause for the patients’ symptoms. Subsequently, diagnostic consideration of a thoracic spinal lesion prompted repeat myelography of the thoracic region which demonstrated a relevant lesion in each case. It is important to visualize the thoracic cord when myelography is performed for the investigation of pain or neurological symptoms in the lower back or lower extremities.

Two patients are reported with hemiballismus due to lesions in the striatum as documented on CT scan. The involuntary movements persisted for one and five years respectively. A third patient with more typical self-limited hemiballismus had no evidence of a causative lesion on CT scan. It is proposed that lesions outside the subthalamic nucleus of Luys may interfere with the compensatory mechanisms which can result in the spontaneous resolution of hemiballistic movements. None of the patients benefited from the use of sodium valproate therapy, however, pimozide reduced the severity of the dyskinesia in the two with longstanding hemiballismus.

The literature contains a single description of sensory perineuritis (Asbury et al 1972). These patients demonstrated a painful, distal, sensory neuropathy, and examination of peripheral nerve biopsies revealed focal thickening and inflammatory infiltrates of the perineurium. We report a patient with sensorimotor peripheral nerve dysfunction, accompanied by progressive slowing of nerve conduction velocity. Examination of a sural nerve biopsy demonstrated focal thickening of the perineurium, inflammatory infiltrates, and necrosis of perineurial cells. Immunohistology revealed a patchy precipitation of IgG and IgM on perineurial cells. Ultrastructurally, mononuclear cells were found adjacent to perineurial cells undergoing necrosis. The patient showed gradual improvement partially coinciding with a course of steroid therapy. We suggest that this neuropathy is caused by damage to the perineurial barrier possibly by an immune-mediated destruction of perineurial cells and subsequent compression of the endoneurial content by perineurial scarring.

Pupils in congenital neurosyphilis differ from the classic Argyll-Robertson pupil in acquired cases, and often tend to be large and unreactive. Constriction to pilocarpine in the reported patient would suggest that peripheral parasympathetic damage is responsible for such pupillary findings. Congenital neurosyphilis should be included among the causes of “tonic” pupil.

An Arab woman developed recurrent chorea gravidarum in all four pregnancies that went to term, and none in the three pregnancies that ended in spontaneous abortion. She also developed an acute psychosis in one of these four pregnancies. A discussion of similar cases in the literature is undertaken.

This review is an attempt to relate the types of angiomas and other cutaneous vascular lesions appearing in the face with abnormalities in the formation of cerebral vessels including arteries at their origin, veins and venous sinuses, and with other less constant alterations that affect other organs, particularly the heart and cerebellum. After angiographic study of a series of patients exhibiting diverse types of vascular nevi (capillary and cavernous hemangiomas, nevus flammeus, and lymphangiomas), a correlation was established for each type of dermal nevus with a corresponding group of intracranial vascular anomalies. An explanation of these malformations based on the embryological development of the principal cerebral vessels during the 7th and 8th weeks of gestation is offered. Involvement of the facial and scalp dermatome innervated by the first sensory trigeminal branch correlates most consistently with the presence of abnormal cerebral vessels. The capillary hemangioma occurs preferentially in girls and frequently is associated with developmental anomalies of major cerebral arteries characterised by the absence of some adult vessels and the persistence of others that should have disappeared during embryogenesis such as the fetal trigeminal artery. The complete angiographic exploration of the cerebral vessels from their origin in the aortic arch to the phase of intracranial venous evacuation may reveal unexpected important findings, and is recommended for children with majorfacial vascular nevi, even if they are neurologically asymptomatic.

A 2-year-old previously healthy girl presented to hospital because of irritability, fatigue, pallor and lower extremity weakness. Acute lymphoblastic leukemia (non-T non-B type) was diagnosed by peripheral blood smear and bone marrow aspirate. Chemotherapy was given and included vincristine, prednisone, L-asparaginase and intrathecal methotrexate. In addition, blood and platelet transfusions were given as appropriate. A lumbar puncture showed no cells, glucose 2.7 mmol/L (normal 2.2 – 4.4 mmol/L), protein of 0.40 (normal 0.150 – 0.450 g/L).

A routine chest x-ray had shown probable spinal column anomalies subsequently confirmed on thoraco-lumbar views as splitting of the T10 and T11 vertebra with anterior fusion. Other examiners failed to demonstrate leg weakness and at discharge (5 weeks later) the child was walking normally.