Consultant Neurology oral assessment prep

Knowledge: This is metastatic spinal cord compression until proven otherwise, and the single strongest predictor of walking at six months is ambulatory status at the time treatment starts, so every hour of delay costs function permanently. Bladder involvement is a late feature and signals that the cord is already decompensating.

Management: I would see him immediately, document a precise neurological baseline including power by myotome, the sensory level, tone, reflexes, plantar responses, perianal sensation and anal tone, catheterise for the palpable bladder, and give dexamethasone 16 mg intravenously with gastric protection and glucose monitoring, unless lymphoma were a serious possibility in which case I would discuss with haematology first.

Ethics and communication: Accepting the delay would be the path of least conflict and the wrong decision, so my duty of advocacy for this patient outranks my wish for a smooth relationship with the on-call rota; I would put the request and the clinical urgency in writing as well as by telephone.

Reference: NICE Guideline NG234, Spinal Metastases and Metastatic Spinal Cord Compression (2023) and Patchell et al., Direct Decompressive Surgical Resection in Metastatic Spinal Cord Compression (Lancet, 2005)

Knowledge: The combination of a subacute dementia over eight weeks, myoclonus, ataxia, cortical ribboning with basal ganglia restricted diffusion and periodic sharp wave complexes is highly suggestive of sporadic Creutzfeldt-Jakob disease, and MRI in expert hands is the most sensitive single investigation. Even so, my obligation is to exclude the treatable mimics rigorously, because prion disease has no treatment and misdiagnosis costs a recoverable patient their recovery.

Management: I would admit or expedite a coordinated workup: MRI reviewed personally with a neuroradiologist including DWI and ADC sequences, EEG, and a lumbar puncture sending cell count, protein, glucose, oligoclonal bands, RT-QuIC, 14-3-3, total tau, viral PCR, cytology and flow cytometry, together with serum and CSF autoimmune and paraneoplastic antibody panels, thyroid antibodies, B12, thiamine, HIV, syphilis serology, ANA and ANCA, and a CT of chest, abdomen and pelvis for occult malignancy.

Ethics and communication: His wife has asked a direct question and she deserves a direct, honest answer: I would say that the scan and EEG appearances make prion disease the leading possibility, that I am completing tests to exclude conditions we can treat, and that I will tell them the moment I know more, rather than either evading the word or asserting a diagnosis before confirmation.

Reference: Zerr et al., Updated Clinical Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease (Brain, 2009) and CDC Infection Control Guidance for Creutzfeldt-Jakob Disease (2020)

Knowledge: Every element of this examination points centrally rather than peripherally: a normal head impulse test in a patient with a genuine acute vestibular syndrome, direction-changing nystagmus and skew deviation together form a dangerous HINTS pattern, which in experienced hands is more sensitive than early diffusion-weighted MRI for posterior circulation stroke in the first 24 to 48 hours.

Management: At the first assessment I would not accept the vestibular neuritis label; I would arrange urgent MRI with diffusion-weighted imaging and MR angiography of the posterior circulation, admit him to a stroke unit with hourly neurological observations, keep him nil by mouth until swallow assessment, and start secondary prevention appropriate to the mechanism once haemorrhage is excluded.

Ethics and communication: There has been a diagnostic error by the emergency team, and my responsibility is candour with the family about the change in diagnosis, explained without disparaging colleagues: I would say clearly that the original assessment was of an inner ear problem, that the picture has proved to be a stroke in the back of the brain, and what we are doing now.

Reference: Kattah et al., HINTS to Diagnose Stroke in the Acute Vestibular Syndrome (Stroke, 2009) and AHA/ASA Scientific Statement on the Recommendations for the Management of Cerebral and Cerebellar Infarction with Swelling (Stroke, 2014)

Knowledge: Clinically this is amyotrophic lateral sclerosis with bulbar and limb involvement meeting the Gold Coast criteria, and before accepting that label I would satisfy myself that the mimics have been excluded, particularly cervical myeloradiculopathy, multifocal motor neuropathy with conduction block, inclusion body myositis, Kennedy disease, myasthenia with bulbar onset, and treatable causes such as thyrotoxicosis or lead exposure, alongside HIV and paraneoplastic considerations.

Management: I would take the son to a private room, thank him, explore his fears and what he has been told, and explain that I will not lie to his father but that I will let his father set the pace, which usually reassures families more than any argument about rights.

Ethics and communication: I would frame my answer to the son around a shared goal rather than a confrontation, saying that I share his wish not to harm his father and that experience shows patients suffer more from a conspiracy they can sense than from the truth delivered gently.

Reference: NICE Guideline NG42, Motor Neurone Disease: Assessment and Management (2016, updated 2019) and Baile et al., SPIKES: A Six-Step Protocol for Delivering Bad News (The Oncologist, 2000)