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.

Among 38 children with pseudotumor cerebri only 3 cases were due to ear disease or its complications. The commonest cause was a refeeding syndrome either due to nutritional deprivation or as an early finding in the treatment of cystic fibrosis. It is uncommon to find the cause of pseudotumor in older children but in those under 6 years the cause was found in 85%. Because of the adverse effects of steroids we use this treatment in the more resistant cases.

Thorough pathological examination disclosed considerable diversity of abnormal cell types and correspondingly different surgical correction rates among patients with apparently similar syndromes of pituitary hypersecretion.The surgical correction of acromegaly in patients with densely granulated growth hormone tumours was threefold that in patients whose tumours showed sparse granulation. Two non-prolactinoma tumour types associated with hyperprolactinemia have aggressive growth patterns; their special therapeutic management is discussed. Of 33 patients with Cushing's disease solitary adenomas were found in only 14, while six patients had proven corticotroph cell hyperplasia. Elective hypophysectomy should replace selective adenomectomy in selected cases of Cushing's disease.

A retrospective study of 100 patients with spontaneous intracerebral haemorrhage was carried out, to identify clinical factors which have a predictive value for outcome. Numerical equivalents for the admission level of consciousness (the Glasgow Coma Scale), ventricular rupture, partial pressure of oxygen in the blood, the electrocardiogram, clot location, and clot size were combined into equations predicting outcome. The best single parameter for prediction was the Glasgow Coma Scale.

The author reviews the Princess Margaret Hospital (Ontario Cancer Institute) experience with radiation therapy in certain tumours involving the skull base including meningiomas, chordomas, craniopharyngiomas and other less common tumours. Radiation therapy offers an effective supplementary or alternative treatment for such lesions.

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.

Advances in fetal diagnostic techniques have opened many areas to prenatal anatomical scrutiny. Intrauterine hydrocephalus and ventriculomegaly are conditions which are readily diagnosed. Fetal intervention has been undertaken in humans in order to minimize the craniofacial disfigurement and to maximize the growth potential of the brain. To justify such an approach, the significance of all anomalies should be recognized prior to treatment. The authors have reviewed 41 cases of hydrocephalus diagnosed in utero in order to define associated anomalies and patient outcome.

75% of our personal series and 72% of the reviewed literature cases had other anomalies of the central nervous system. Other system malformations, some of which proved fatal, were seen commonly. Prenatal diagnostic techniques did not always reveal these additional problems.

The outcome of these pregnancies is not good. Approximately one third of these fetuses have survived to be treated postnatally and to be followed up clinically. Only 7.5% of this series were felt to have attained normal developmental milestones. The remainder of the survivors have various focal and/or global cerebral deficits.

The association of idiopathic Parkinson’s disease and Creutzfeldt-Jakob disease has not been reported previously. We describe the clinical findings in a patient with both disorders, confirmed at autopsy. The differential diagnosis of parkinsonism with cognitive and psychiatric disturbances is reviewed with emphasis on conditions which may complicate the course of idiopathic Parkinson’s disease. The etiological implications of this combination are considered.

The demonstration of low arylsulfatase-A (ASA) activity in leucocytes or fibroblasts is used often to establish the diagnosis of metachromatic leucodystrophy (MLD). However, low ASA activity is observed also in pseudo-ASA deficiency which may be as common as MLD. We report two patients with pseudo ASA deficiency who had abnormal neurological findings consistent with atypical MLD. Because the measurement of ASA activity is neither a sensitive nor specific method with which to establish a diagnosis of MLD, this diagnosis should be confirmed by nerve biopsy, measurement of urinary sulfatide or a cerebroside sulfate loading test, using cultured fibroblasts.

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.

Mu- and delta-opioid subtype receptor antagonists were tested in the mouse for their effects on vertical climbing activity, an index of striatal dopaminergic activity. The selective delta-opioid antagonist ICI 154 129 (1/1) by itself enhanced vertical climbing activity in a dose-related manner, whereas the mu-opioid antagonist naloxone by itself was inactive on climbing behavior. Naloxone increased the climbing-stimulant effect of I/I. Unstimulated vertical climbing activity was reduced, and all opioid-antagonist enhancement of climbing behavior was antagonized, by the competitive dopamine antagonist haloperidol in dose-related fashion. The observed motor-enhancement effect of a selective delta-opioid receptor antagonist is the first demonstration of physiologically-significant tonic activity on a central opioid receptor. Our observations suggest that 1/1 may be useful clinically in striatal dopamine-deficient disease conditions such as parkinsonism.

The rationale for interstitial irradiation of tumours in and around the skull base is reviewed, and the experience accumulated with pituitary adenomas, meningiomas, and chordomas is summarized. Intracystic irradiation for craniopharyngiomas is also reviewed.

Interest in photodynamic therapy of malignant brain tumours has been growing in recent years as intra-operative laser applications become more available.

We have developed an inflatable balloon which can be coupled to an argon dye pump laser in order to deliver light to a brain tumour cavity created by the subtotal resection of tumour. Eight patients with primary malignant brain tumours have been treated with photodynamic therapy (PDT) using this device. The 8 patients tolerated the treatment well; morbidity attributable to the PDT was acceptably low.

A review of the literature on primary diffuse meningeal gliomatosis (DMG) yielded three cases and we report a fourth. DMG is a syndrome characterized by extensive basal and spinal chronic meningitis with mental confusion, headaches, diplopia, papilledema and cranial nerve palsies. The cerebrospinal fluid (CSF) has a markedly elevated protein content, moderate mononuclear pleocytosis and a normal or low glucose. This picture invariably leads to the diagnosis and treatment of tuberculous or fungal meningitis despite persistently negative cerebrospinal fluid (CSF) cultures. Reaction of exfoliated CSF cells with glial fibrillary acidic protein (GFAP) immunoperoxidase labelled antibody is suggested as a diagnostic tool. A basal meningeal biopsy appears to be the only alternative diagnostic approach.

Adenoid cystic carcinoma, or cylindroma, usually presents in the salivary glands or in the upper respiratory passages. We report an unusual case of adenoid cystic carcinoma in a 45-year-old woman who presented with a three-year history of progressive right trigeminal sensory palsy, most pronounced in the second division. There was, in addition, a right trigeminal motor palsy and a partial right 6th nerve palsy. CT scanning showed only a small soft tissue mass spanning an enlarged right foramen ovale. Solid adenoid cystic carcinoma was resected from Meckel’s cave via a middle cranial fossa approach. A subsequent biopsy of the right maxillary antral mucosa found tumor tissue. We concluded that the tumor originated in the maxillary antrum and spread posteriorly along the infraorbital nerve to enlarge in the foramen ovale. Radiation to a total of 5,000 cGy was given. At 22 months there was neither radiographic nor clinical evidence of recurrence. At 30 months, the development of unsteady gait signalled the presence of recurrent tumor extending backwards from Meckel’s cave into the right cerebello-pontine angle.

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 72-year-old woman presented with painful proptosis of the right eye and a large destructive tumour of the middle cranial fossa. A diagnosis of IgDK multiple myeloma was made, based on histopathologic and immunologic studies of the biopsy. Biochemistry and bone marrow examination further confirmed the myeloma as IgDK type. The clinical, radiological, and pathological findings are presented. The patient was treated with radiotherapy with satisfactory results.

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.