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Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare primary headache syndrome first described in 1978. We report on a 43-year-old man with a 10 year history of SUNCT in whom a pituitary macroadenoma was eventually detected. His pain rapidly improved with medical treatment of the prolactinoma and we propose that this is a case of symptomatic SUNCT., (Crown Copyright © 2012. Published by Elsevier Ltd. All rights reserved.)

Intraneural perineurioma is a rare tumour that affects peripheral nerves and, based on its histological features, may be confused with hereditary motor and sensory neuropathies. Detailed neuropathology, including immunoperoxidase stains and electron microscopy, is vital to distinguish these conditions. We report two patients with intraneural perineurioma that demonstrate salient features of this tumour. The first patient is the longest reported follow-up of an intraneural perineurioma; extension of the lesion was observed over 14years. The second patient is an 11-year-old female whose treatment highlights some of the controversy surrounding the management of these lesions, and the importance of thorough macroscopic and microscopic assessment by pathologists, including the status of surgical resection margins.

We report a case of relapsing multifocal neurological disease associated with CNS echovirus 6 infection in an HIV-1-infected individual with no evidence of immunoglobulin deficiency. The illness was initially characterized by optic and cranial neuropathies and myelopathy; concurrent granulomatous hepatitis suggested disseminated viral infection. Treatment with combination nucleoside analogues led to partial remission, but a demyelinating polyneuropathy subsequently developed. There was improvement and sustained remission in the polyneuropathy following treatment with intravenous immunoglobulin. Neurotropic enterovirus infection may be involved in the pathogenesis of certain HIV-associated neurological syndromes.

Ophthalmoplegia is a rarely observed sign in carbamazepine and other anticonvulsant overdoses. We present a patient who developed transient complete external ophthalmoplegia and asterixis with relative preservation of consciousness, in association with carbamazepine toxicity. Previously reported cases and proposed mechanisms are reviewed.

Phenelzine associated sensorimotor peripheral neuropathy is reported in two patients. Symptoms were predominantly sensory, and improvement occurred after withdrawal of phenelzine. Electrophysiologic findings were consistent with an axonal process.

There is a definite relationship between the vascular type of benign sexual headache and benign exertional headache. Forty five patients with benign vascular sexual headache were reviewed. Twenty seven (60%) experienced benign vascular sexual headache alone and eighteen (40%) had experienced both benign vascular sexual headache and benign exertional headache on at least one occasion. The mean age was 34.3 years with a male:female ratio of 5.4:1. Thirty patients with a history of benign vascular sexual headache were followed for an average of 74 months. A personal history of migraine was found in 47% of cases and a family history of migraine in 30%. Forty one per cent of patients with benign vascular sexual headache alone had recurrences after diagnosis, and stress and fatigue were considered major contributing factors to the initial and recurrent headache. Nine patients had experienced benign vascular sexual headache and benign exertional headache within 72 hours of each other on at least one occasion, often with a residual headache between the two. Four patients experienced their benign vascular sexual headache and benign exertional headache separated by months to years. The prognosis of benign vascular sexual headache and the clinical and possible pathophysiological relationships between benign vascular sexual headache and benign exertional headache are discussed. Knowledge of the interrelationships of these varieties of headache is valuable in the counselling of patients.

Pudendal neuropathies are well recognised as part of more generalised peripheral neuropathies; however, focal abnormalities of the pudendal nerve due to cycling-related injuries have been infrequently reported. We describe two patients who developed pudendal neuropathies secondary to pressure effects on the perineum from racing-bicycle saddles. Both were male competitive athletes, one of whom developed recurrent numbness of the penis and scrotum after prolonged cycling; the other developed numbness of the penis, an altered sensation of ejaculation, with disturbance of micturition and reduced awareness of defecation. Both patients improved with alterations in saddle position and riding techniques. We conclude that pudendal nerve pressure neuropathy can result from prolonged cycling, particularly when using a poor riding technique.

Clinical and spinal fluid evidence of aseptic meningitis were the sole features at presentation in a patient with pituitary apoplexy. Visual impairment and bitemporal field defects developed later. Necrosis within a chromophobe adenoma was found at surgical decompression of the sella.

We investigated pupillary responses to parasympathetic (pilocarpine) and sympathetic agents (tyramine, cocaine, and phenylephrine) in a 51-year-old woman with tonic pupils, loss of muscle stretch reflexes in the limbs, and hemifacial loss of sweating and flushing (Ross' syndrome). A smaller pupillary response to tyramine and cocaine eyedrops on the symptomatic side indicated that outflow was disrupted in the postganglionic section of the ocular sympathetic pathway. A greater response to phenylephrine eyedrops on this side was consistent with denervation supersensitivity to adrenergic agents. Loss of thermoregulatory sweating and flushing and emotional blushing in the forehead, cheek, and chin indicated that sympathetic disruption was proximal to the bifurcation of the common carotid artery, probably in the superior cervical ganglion. A similar degenerative process may be responsible for loss of muscle stretch reflexes, tonic pupils, and other autonomic disturbances in Ross' syndrome.

Two definite and 2 probable cases of anogenital herpes simplex and sacral radiculitis are described. Symptoms were typical and consisted of paraesthesia and neuralgic pain in the perineum and legs, urinary retention and constipation occurring within several days to a week after an anogenital herpetic eruption. However, at presentation only 1 case had an obvious history of anogenital herpes simplex. Neurological signs were not striking and consisted of a reduced appreciation of light touch and pin prick over the sacral dermatomes and in 2 cases reduced anal sphincter tone. CSF examination in 3 patients showed a lymphocytosis. Bladder catheterisation was required for up to 2 weeks in 2 patients. The paraesthesia persisted for weeks to months. It should be more widely recognised that anogenital herpes simplex, with sacral radiculitis, is probably the commonest cause of acute retention of urine in young sexually active people.

Optic neuropathy in Graves' disease is an uncommon, but potentially treatable cause of disabling visual loss. Optic nerve damage is probably secondary to compression by swollen extraocular muscles at the apex of the orbit. The visual loss is usually bilateral and insidiously progressive, although accelerated visual loss, fluctuations in vision, and features mimicking orbital cellulitis may occur. Ocular congestive symptoms and proptosis have no direct relationship to the severity of visual loss. Early diagnosis is facilitated by orbital CT scanning. Oral corticosteroids and radiotherapy, alone or in combination, are the primary modalities of medical treatment. Surgical decompression of the orbit can be used where medical approaches have failed.

A case of poisoning caused by an excessive dose of herbal laxative tablets is reported. The patient's symptoms included coma and prolonged peripheral neuropathy. The clinical features were characteristic of podophyllin poisoning. Podophyllin was a component of the laxative tablets, but the container gave no indication of their potential toxicity. Methods of reducing the risk of inadvertent poisoning by herbal remedies are suggested.

Three patients who were admitted to hospital because of syncope were found to have malignant head and neck tumours. A patient who presented with malignant external otitis with syncope and postural hypotension is also described. It is postulated that the syncope and postural hypotension result from compressive irritation of the glossopharyngeal nerve either in the neck or at the base of the skull.

A patient with symptomatic hydrocephalus, due to an elongated and ectatic basilar artery and involving the third ventricle, is described. Characteristic CT scan features are outlined and were sufficient to confirm the diagnosis without resort to pneumoencephalography or angiography. The intraventricular CSF pressure was normal and a ventriculoperitoneal shunt resulted in significant sustained improvement of her balance and higher mental functions, coincident with CT scan evidence of return of ventricular size to normal. Other neurological presentations of an elongated and ectatic basilar artery include single or multiple cranial nerve involvement, a cerebellopontine-angle syndrome or chiasmal compression with visual failure.

Classical beliefs about the functions of the dorsal columns of the spinal cord have been attacked following recent evidence that position and vibration sensations may be carried in the dorsal spinocerebellar tracts. There is evidence that the one specific function of the dorsal columns is for the transmission of information concerning the direction of tactile cutaneous movement. Thirty normal controls, 43 patients with spinal cord disorders and 10 patients with functional disorders were examined prospectively using an easily administered "direction of scratch" protocol. Interpretation of the direction of a 2 cm vertical tactile cutaneous movement over the lower limbs was found to be accurate in normal controls and grossly inaccurate in patients with functional disorders, exceeding the error rate of guessing. Detection of direction of 2 cm scratch was moderately impaired in 11 of 13 patients with spastic paraparesis and preserved sensation to all other modalities and 23 of 24 patients with spastic paraparesis and impaired proprioception and/or vibration sensations. Direction of 2 cm scratch, proprioception and vibration sensations were preserved in the three cases with anterior spinal cord syndromes. It is proposed that tactile perception of direction of 2 cm scratch over the lower limbs is a sensitive sign of posterior column function which can be usefully incorporated into the clinical sensory examination in the evaluation of spinal cord disorders.

Primary position upbeating nystagmus has been associated clinically with intra-axial brainstem and cerebellar lesions but evidence for more precise localisation to the ponto-mesencephalic and ponto-medullary junctions is accumulating. We report the occurrence of primary position upbeating nystagmus in three patients who had clinical signs of pontine lesions at the ponto-mesencephalic and ponto-medullary junctions. Radiological confirmation was possible in two cases.

Whole tibialis anterior muscles were removed from a number of dogs and were then reimplanted in the original sites. Microsurgical anastomoses of the major nerve, artery, and vein were performed. Biopsy revealed some minor regenerative changes in the muscle a few weeks after the operation. Electromyographic recordings 6-9 months after implantation showed near-complete functional recovery of the muscles. This was confirmed histologically. The study demonstrates not only that whole-muscle reimplantation is technically feasible but that a functionally satisfactory result may be expected.

Three parkinsonian patients are described who independently discovered that their gait was facilitated by inverting a walking stick and using the handle, carried a few inches from the ground, as a visual cue or target to step over and initiate walking. It is suggested that the "inverted" walking stick have wider application in patients with Parkinson's disease as an aid to walking, particularly if they have difficulty with step initiation and maintenance of stride length.

Orbital myositis implies orbital inflammation confined to one or more of the extraocular muscles. Orbital computerised tomography (CT) demonstrates irregular extraocular muscle enlargement which extends anteriorly to involve the tendon (muscle insertion). Six cases of presumed orbital myositis are reported, in each of whom the diagnosis was suspected clinically and confirmed by the orbital CT scan appearances. The mean age of the patients was 33 years (range 8-45 years). All presented with painful ophthalmoplegia and the majority manifested proptosis (five cases), conjunctival congestion (five cases) and periorbital and eyelid edema (two cases). Systemic corticosteroid therapy was used in two patients initially and also in another patient who relapsed, with rapid and dramatic responses. Extraocular muscle biopsy was performed in one case, disclosing features of non-specific muscle inflammation and no evidence of vasculitis. It is considered that orbital myositis is a discrete, identifiable subgroup within the spectrum of the nonspecific idiopathic orbital inflammatory syndromes; termed previously orbital 'pseudotumours'. Although the clinical features are frequently suggestive, they are nonspecific, and non-invasive investigations such as orbital ultra-sonography and CT scanning are required for precise anatomical tissue localisation and diagnosis. The role of ocular muscle biopsy is probably limited to atypical cases, or those unresponsive to steroid therapy, particularly to exclude neoplasia. Orbital myositis may be acute, subacute or recurrent. The acute form responds well to high doses of oral corticosteroids tapered gradually, but it may recur or become chronic. The subacute form of the disease responds less well.

Six hundred and sixty-one patients with stroke, confirmed by CT scan or at autopsy, were reviewed in order to evaluate the frequency of presentation with altered mental state. Nineteen patients (3%) had presented with delirium, an organic delusional state, the acute onset of dementia, or mania, mimicking psychiatric illness. All had focal cerebrovascular lesions which were usually, but not invariably, right sided. None had a previous history of cognitive impairment, psychiatric disease, drug abuse, or alcohol excess. Neurological signs were absent or mild and transient, and therefore easily missed. Post-mortem examinations in four patients showed localised cerebral infarctions with no evidence of multiple lesions, Alzheimer's disease, or metabolic encephalopathies. The possible causative factors are discussed and the evidence of asymmetrical cerebral representation of emotion, and for a relationship with epilepsy, is reviewed.