We describe an unusual case of clinical amyotrophic lateral sclerosis (ALS) with initial neurophysiological studies suggesting demyelination, along with neuroimaging findings that helped to support the eventual diagnosis. An otherwise well 68-year-old man had 8 weeks of left upper limb weakness. On examination, there were widespread lower and upper motor neurone findings suggesting ALS. However, nerve conduction studies identified sensorimotor demyelinating changes suggesting chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a diagnosis further supported by cerebrospinal fluid analysis. MR scan of the brain revealed a ‘motor band’, a feature seen commonly in ALS. His condition was refractory to immunotherapy with clinical progression in-keeping with ALS, establishing the diagnosis. ALS is rarely associated with demyelinating neurophysiological changes resembling CIDP. The clinical phenotype is crucial to support the correct diagnosis and imaging findings may help.

Two patients presented with side-locked frontal head pain, involving the supraorbital nerve territory, with an associated hypopigmented macule. The clinical progress and nerve biopsy in one indicated leprosy. In endemic regions, supraorbital neuralgia may be caused by leprosy sometimes without other neurocutaneous markers.

Tubular aggregate myopathies comprise a rare group of disorders with characteristic pathological findings and heterogeneous phenotypes, including myasthenic syndrome. We describe a patient with tubular aggregate myopathy who presented with fatiguable weakness improving with pyridostigmine, respiratory involvement and possible cardiac manifestations. We highlight the utility of muscle biopsy in atypical myasthenic syndrome.

A young woman with Rogers syndrome (thiamine-responsive megaloblastic anaemia, diabetes mellitus and sensorineural deafness) presented with headache, recurrent supraventricular tachycardia and features of an upper gastrointestinal bleed, 1 month after radiofrequency cardiac ablation for supraventricular tachycardia. She deteriorated rapidly after endoscopy and subsequently died. Brain imaging during the acute deterioration showed diffuse intracranial air embolism and hypoxic–ischaemic injury. Postmortem examination showed an atrio-oesophageal fistula, a rare complication of cardiac ablation. Clinicians should suspect this condition in patients with acute neurological deterioration after cardiac ablation who have diffuse air embolism on imaging.

A 21-year-old woman with multiple sclerosis (taking regular fingolimod) developed sudden-onset severe headache with nausea and malaise. Neurological examination was normal and she was afebrile. Blood results showed lymphocytes 0.53 x 109/L and C reactive protein 19 mg/L. CT scan of head and venogram were normal. CSF showed an opening pressure of 33 cm H2O and an incidental light growth of Cryptococcus neoformans, confirmed with positive India Ink stain and a positive cryptococcal antigen (1:100). She was treated for cryptococcal meningoencephalitis with amphotericin and flucytosine. Her presenting symptoms had closely mimicked subarachnoid haemorrhage. This atypical presentation of cryptococcal CNS infection highlights the need for vigilance in immunosuppressed patients.

![Graphic][1]</img> Although writing this in August 2023, it is the morning after half a month’s rain fell in the space of a few hours overnight with gale force winds into the bargain. All par for the course in an English summer. Naturally, being in a tent on the South Coast did not help the unseasonable feeling. Although we always feel the grass is metaphorically greener elsewhere, the grass is at least literally greener here in the UK. By the time you read this, the inaugural ‘Preparing for Consultancy’ Course run by the ABN will have happened. We rightly focus so much on … [1]: /embed/inline-graphic-1.gif

Neurological involvement in Behçet’s syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. The brainstem is involved in 50% of cases, the diencephalon and other areas of the brain in 30%, and the spinal cord in 10%. Movement disorders and epilepsy may occur. Psychiatric syndromes may arise with brain and brainstem involvement, and cognitive disorders relate to the brain disease, to circulating inflammatory factors, and to fatigue and despondency. Eighty per cent of cases begin with a relapsing disease course, of whom 70% have only one attack, and 30% have a progressive disease course either from onset or following an initially relapsing course. Venous thrombosis leading to intracranial hypertension and cerebral venous infarction is less common and caused by inflammation in affected veins and a circulating prothrombotic state. Arterial involvement is rare and relates to an arteritis affecting large-sized and medium-sized vessels within the brain leading to infarction, subarachnoid and parenchymal haemorrhage, aneurysm formation and arterial dissection. There is a newly recognised disorder of cerebral cortical hypoperfusion. Cranial neuropathy, peripheral neuropathy and myositis are rare. There has been significant progress in understanding the pathophysiology and treatment of the systemic disease, leading to improved outcomes, but there has been no randomised trial of treatment in the neurological disorder.

status: Published