The patient’s medical history of experiencing headaches accompanied by temporary visual disturbances, along with the results of their examination, suggest that they may be suffering from idiopathic intracranial hypertension (IIH). However, the fact that these symptoms began during the puerperium period, and that the patient is otherwise healthy and not at risk for IIH, raises concerns that they may actually be experiencing cerebral venous sinus thrombosis. Therefore, it is important to conduct imaging of the venous system using CT or MR to rule out a thrombus, which would require anticoagulation treatment.

While a therapeutic lumbar puncture can provide temporary relief for IIH-related headaches, it is not a long-term solution. In cases where medical management has failed, ventriculo-peritoneal shunting may be necessary.

Nerve conduction studies are not relevant to this patient’s condition. Additionally, since acetazolamide can cause paraesthesia as a side effect, increasing the dosage may not be a viable option for this patient.

Intracranial Venous Thrombosis: Causes, Symptoms, and Management

Intracranial venous thrombosis is a condition that can cause cerebral infarction, although it is less common than arterial causes. About 50% of patients have isolated sagittal sinus thromboses, while the remainder have coexistent lateral sinus thromboses and cavernous sinus thromboses. Common symptoms include sudden onset headache, nausea and vomiting, and reduced consciousness.

To diagnose intracranial venous thrombosis, MRI venography is the gold standard, although CT venography is an alternative. Non-contrast CT head is normal in around 70% of patients, and D-dimer levels may be elevated. The management of intracranial venous thrombosis involves anticoagulation, typically with low molecular weight heparin acutely, and warfarin for longer-term anticoagulation.

There are specific syndromes associated with intracranial venous thrombosis. Sagittal sinus thrombosis may present with seizures and hemiplegia, and parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen. Cavernous sinus thrombosis may cause periorbital oedema, ophthalmoplegia, trigeminal nerve involvement, and central retinal vein thrombosis. Lateral sinus thrombosis may cause 6th and 7th cranial nerve palsies.

In summary, intracranial venous thrombosis is a serious condition that requires prompt diagnosis and management. Patients with symptoms such as sudden onset headache, nausea and vomiting, and reduced consciousness should seek medical attention immediately. MRI venography is the gold standard for diagnosis, and anticoagulation is the mainstay of treatment. Specific syndromes associated with intracranial venous thrombosis may present with seizures, hemiplegia, ophthalmoplegia, and other symptoms.

The patient is most likely suffering from motor neuron disease. He is a male in his 50s and has shown signs of bulbar involvement without any peripheral or sensory issues. The chronic nature of his symptoms makes a stroke unlikely, while his age, sex, and progressive symptoms make multiple sclerosis less probable. The absence of arm weakness or sensory loss makes syringobulbia less likely, and myotonic dystrophy would present with more peripheral signs. Therefore, the most likely diagnosis is motor neuron disease with bulbar features.

Motor neuron disease is a neurological condition that is not yet fully understood. It can manifest with both upper and lower motor neuron signs and is rare before the age of 40. There are different patterns of the disease, including amyotrophic lateral sclerosis, progressive muscular atrophy, and bulbar palsy. Some of the clues that may indicate a diagnosis of motor neuron disease include fasciculations, the absence of sensory signs or symptoms, a combination of lower and upper motor neuron signs, and wasting of small hand muscles or tibialis anterior.

Other features of motor neuron disease include the fact that it does not affect external ocular muscles and there are no cerebellar signs. Abdominal reflexes are usually preserved, and sphincter dysfunction is a late feature if present. The diagnosis of motor neuron disease is made based on clinical presentation, but nerve conduction studies can help exclude a neuropathy. Electromyography may show a reduced number of action potentials with increased amplitude. MRI is often used to rule out cervical cord compression and myelopathy as differential diagnoses. It is important to note that while vague sensory symptoms may occur early in the disease, sensory signs are typically absent.

Diagnosis of Sporadic Creutzfeldt-Jakob Disease

This patient is experiencing a rapid decline in cognitive function, dysphasia, cerebellar symptoms, myoclonus, and frontal release signs. Although his thyroid-stimulating hormone is elevated, his thyroxine level is normal, ruling out hypothyroidism as the cause of his symptoms. While depression can cause pseudo-dementia, this patient’s abnormal neurology suggests a different diagnosis. Vascular dementia typically presents with a history of cerebrovascular disease and a step-wise decline in cognition, which is not the case here. Cerebral vasculitis is a possibility, but the patient’s generalized myoclonus and short progressive history are more consistent with sporadic Creutzfeldt-Jakob disease (sCJD).

To confirm a diagnosis of sCJD, a combination of tests is necessary. An EEG will show deterioration in normal background rhythms and periodic sharp wave complexes in two-thirds of patients. A positive CSF 14-3-3, a normal neuronal protein released following neuronal damage, can support a diagnosis of sCJD. MRI is also important in ruling out other potential diagnoses and can reveal a characteristic signal change in the putamen and caudate.

Overall, this patient’s symptoms and clinical picture suggest a diagnosis of sCJD, which can be confirmed through a combination of EEG, CSF analysis, and MRI findings.

Parkinson’s disease is a progressive neurodegenerative disorder that occurs due to the degeneration of dopaminergic neurons in the substantia nigra. This leads to a classic triad of symptoms, including bradykinesia, tremor, and rigidity, which are typically asymmetrical. The disease is more common in men and is usually diagnosed around the age of 65. Bradykinesia is characterized by a poverty of movement, shuffling steps, and difficulty initiating movement. Tremors are most noticeable at rest and typically occur in the thumb and index finger. Rigidity can be either lead pipe or cogwheel, and other features include mask-like facies, flexed posture, and drooling of saliva. Psychiatric features such as depression, dementia, and sleep disturbances may also occur. Diagnosis is usually clinical, but if there is difficulty differentiating between essential tremor and Parkinson’s disease, 123I‑FP‑CIT single photon emission computed tomography (SPECT) may be considered.

Medications for Headaches: Understanding Their Uses and Limitations

Cluster headaches are severe, unilateral, periorbital headaches that are often accompanied by autonomic features such as eye watering. Verapamil is the first-line option for prophylaxis of cluster headaches, as it can prevent future attacks. High-flow oxygen and triptans can be used to terminate an acute attack, but they do not prevent future attacks. Indomethacin is useful in terminating ice-pick headaches, which are brief, severe stabbing pains that primarily affect women. Sumatriptan can terminate an acute attack of cluster headache or migraine, but it does not prevent future attacks. Propranolol is a first-line option for prophylaxis of migraine, but it is not useful in the prophylaxis of cluster headache. Enalapril is not used in the treatment of any commonly occurring headache syndromes. Understanding the uses and limitations of these medications can help healthcare providers make informed decisions when treating patients with headaches.

Determining the cause of a myopathy that presents in late adolescence and progresses can be challenging. However, in this case, the absence of elevated CK and CRP levels, as well as the lack of systemic symptoms, make a diagnosis of polymyositis unlikely. Myotonic dystrophy type 1 is also improbable due to the absence of myotonia and the absence of other associated symptoms such as cardiac and respiratory involvement. MERRF can also be ruled out as it typically presents with myoclonus, peripheral neuropathy, and ataxia. The muscles affected in this case are the facial and upper limb girdle muscles, which is consistent with facioscapulohumeral muscular dystrophy. It is important to note that this type of muscular dystrophy can initially present asymmetrically before progressing to bilateral involvement.

Understanding Facioscapulohumeral Muscular Dystrophy

Facioscapulohumeral muscular dystrophy (FSHMD) is a type of muscular dystrophy that is inherited in an autosomal dominant manner. This means that a person only needs to inherit one copy of the mutated gene from one parent to develop the condition. FSHMD is characterized by the progressive weakness and wasting of muscles, particularly those in the face, scapula, and upper arms.

The first signs of FSHMD usually appear during adolescence or early adulthood, typically around the age of 20. The facial muscles are often the first to be affected, leading to difficulty with tasks such as closing the eyes, smiling, and blowing. As the condition progresses, weakness in the shoulder and upper arm muscles becomes more pronounced, and the borders of the shoulder blades may become abnormally prominent, giving the appearance of winging.

In addition to upper body weakness, FSHMD can also affect the lower limbs, causing weakness in the hip girdle and foot drop. While there is currently no cure for FSHMD, there are treatments available to help manage symptoms and improve quality of life. Physical therapy, assistive devices, and medications may all be recommended to help individuals with FSHMD maintain their mobility and independence for as long as possible.

Alcoholic Neuropathy: Diagnosis and Management

Alcoholic neuropathy is a condition that affects peripheral nerves and is caused by prolonged and excessive alcohol intake. Symptoms include distal and symmetrical sensory disturbances, mild motor manifestations, and less common autonomic disturbances. Diagnosis is based on an accurate history, clinical signs and symptoms, and electrophysiological testing. Management involves abstinence from alcohol, nutritional support, and active rehabilitation. The prognosis is generally good if alcohol intake is discontinued and other causes of neuropathy are excluded. Vitamin B supplementation is important in conjunction with rehab therapy. Magnetic resonance imaging of the thoraco-lumbar spine and sural nerve biopsy are not necessary for diagnosis. Amitriptyline may be useful in neuropathic pain, but establishing the correct diagnosis should be prioritized.

When patients with chest wall or neuromuscular disease experience type 2 respiratory failure, non-invasive ventilation (NIV) should be the preferred treatment. This is especially true for patients with diaphragmatic weakness caused by motor neurone disease, as evidenced by a respiratory acidosis and a PaO2 level below 8 kPa. If hypoxaemia persists despite NIV, additional LTOT may be necessary.

Managing Motor Neuron Disease

Motor neuron disease is a neurological condition that affects both upper and lower motor neurons. It typically presents after the age of 40 and can manifest in different patterns, such as amyotrophic lateral sclerosis, progressive muscular atrophy, and bulbar palsy. While the cause of the disease is unknown, there are several management strategies that can help improve the patient’s quality of life and prolong survival.

One such strategy is the use of riluzole, which prevents the stimulation of glutamate receptors and is mainly used in amyotrophic lateral sclerosis. Studies have shown that it can prolong life by about three months. Respiratory care is also crucial, and non-invasive ventilation, usually BIPAP, is used at night. This has been shown to provide a survival benefit of around seven months.

Nutrition is another important aspect of managing motor neuron disease, and the preferred method of support is through a percutaneous gastrostomy tube (PEG). This has been associated with prolonged survival. However, despite these management strategies, the prognosis for motor neuron disease remains poor, with 50% of patients dying within three years.

Management of Normal Pressure Hydrocephalus

Normal pressure hydrocephalus is a condition characterized by a triad of urinary incontinence, gait disturbance, and dementia. In a patient with this condition, a high normal cerebrospinal fluid (CSF) opening pressure is observed, and symptomatic improvement can be achieved through the removal of CSF. Therefore, the patient should be referred for a ventriculo-peritoneal shunt. While low B12 levels should be replaced, the primary focus should be on managing the normal pressure hydrocephalus.

One option for managing normal pressure hydrocephalus is CSF drainage through repeated lumbar punctures. However, this method can be difficult for patients to tolerate. Another temporary method for relieving CSF pressure is through the use of an external ventricular drain. While an MRI may be performed, it is unlikely to provide any additional information or improve the patient’s symptoms. Therefore, the primary focus should be on managing the normal pressure hydrocephalus through appropriate interventions.

The correct diagnosis for the patient’s symptoms is anterior spinal artery occlusion. This is likely due to the patient’s risk factors for atherosclerosis, such as hypertension and hypercholesterolemia. An infarct in this artery can result in bilateral weakness below the level of the lesion, which in this case appears to be at the T10 level. Additionally, the patient may experience a loss of pain and temperature sensation. This type of injury affects the spinothalamic and corticospinal tracts, leading to an initial flaccid paralysis that may progress to spastic paraparesis over time.

Basilar artery occlusion is an incorrect diagnosis, as it causes a condition known as ‘locked-in syndrome’ that results in complete paralysis of voluntary muscles except for those that control eye movement. Posterior spinal artery occlusion is also an incorrect diagnosis, as it causes a dorsal cord syndrome that primarily affects proprioception and vibration.

Spinal cord lesions can affect different tracts and result in various clinical symptoms. Motor lesions, such as amyotrophic lateral sclerosis and poliomyelitis, affect either upper or lower motor neurons, resulting in spastic paresis or lower motor neuron signs. Combined motor and sensory lesions, such as Brown-Sequard syndrome, subacute combined degeneration of the spinal cord, Friedreich’s ataxia, anterior spinal artery occlusion, and syringomyelia, affect multiple tracts and result in a combination of spastic paresis, loss of proprioception and vibration sensation, limb ataxia, and loss of pain and temperature sensation. Multiple sclerosis can involve asymmetrical and varying spinal tracts and result in a combination of motor, sensory, and ataxia symptoms. Sensory lesions, such as neurosyphilis, affect the dorsal columns and result in loss of proprioception and vibration sensation.

Vertebral Artery Dissection: A Common Cause of Stroke in Young Patients

Vertebral artery dissection is a well-known cause of stroke in patients under the age of 45. It has a mortality rate of 10% during the acute phase and can lead to death due to intracranial dissection, brainstem infarction, or subarachnoid hemorrhage. The causes of vertebral artery dissection include structural defects of the arterial wall, connective tissue disease, trauma, and chiropractic manipulation of the neck.

The typical clinical presentation of vertebral artery dissection is a severe occipital headache followed by focal neurological signs that can be attributed to brainstem or cerebellum ischemia. However, other less likely differential diagnoses of stroke in this age group include focal seizure, migraine with prolonged aura and migraine variants, multiple sclerosis, and conversion disorders.

It is important to recognize vertebral artery dissection as a common cause of stroke in young patients and to consider it in the differential diagnosis of stroke. Early diagnosis and treatment can improve outcomes and prevent mortality.

The most probable diagnosis is medication overuse headache, given the duration of the headache and regular use of codeine. To address this, it is recommended to abruptly stop taking paracetamol and ibuprofen, but gradually reduce the intake of codeine to avoid the adverse effects of acute opioid withdrawal. It is important to note that not only codeine and triptans, but other types of analgesia can also contribute to this condition. Improvement can typically be observed within a few days.

Understanding Medication Overuse Headache

Medication overuse headache is a common cause of chronic daily headache that affects up to 1 in 50 people. It is characterized by headaches that occur for 15 days or more per month and are worsened by regular use of symptomatic medication. Patients who use opioids and triptans are at the highest risk of developing this condition. Additionally, there may be psychiatric co-morbidity associated with medication overuse headache.

According to the 2008 SIGN guidelines, the management of medication overuse headache involves abruptly withdrawing simple analgesics and triptans, which may initially worsen headaches. On the other hand, opioid analgesics should be gradually withdrawn. However, withdrawal symptoms such as vomiting, hypotension, tachycardia, restlessness, sleep disturbances, and anxiety may occur when medication is stopped. Therefore, it is important to seek medical advice before discontinuing any medication.

Differential Diagnosis for a Patient with Spastic Paraparesis

This patient is presenting with spastic paraparesis, which suggests a lesion in the thoracic spine or cerebral cortex. However, the MR thoracic spine is normal, indicating a probable cortical lesion. Other clues to this diagnosis include sensory neglect in the right leg, headaches, and absence of a sensory level. A parasagittal meningioma is a likely cause of these symptoms, as it primarily affects the lower limb and cortical sensory function, and can also impact bladder function and vibration and light touch sense. The CSF findings of raised protein and WCC support this diagnosis.

Multiple sclerosis is unlikely due to the patient’s age, normal MRI spine, and abnormal CSF analysis, which would typically only show intrathecal oligoclonal band production. The presence of both CSF and serum oligoclonal bands suggests a systemic cause, such as rheumatoid arthritis. Anterior spinal artery thrombosis would cause an acute anterior cord syndrome with dissociated sensory loss affecting the lower limbs. Guillain-Barré syndrome typically presents following viral or bacterial enteritis and would show lower motor neurone signs in the limbs. A central cord syndrome within the cervical spine would cause upper motor neurone weakness in the legs, lower motor neurone weakness at the level of the lesion, and dissociated sensory loss of pain and temperature in the upper limb.

In summary, this patient’s symptoms suggest a cortical lesion, possibly caused by a parasagittal meningioma. Other potential diagnoses include multiple sclerosis, anterior spinal artery thrombosis, Guillain-Barré syndrome, and central cord syndrome.

This woman in her middle age is showing concerning symptoms that indicate Creutzfeldt Jakob disease (CJD). She is experiencing rapid cognitive decline, myoclonus, extrapyramidal signs (ataxia), startle response, positive 14-3-3 on CSF, and periodic spiking on EEG. The MRI results suggest cortical ribboning, with high signal on the cortical sulci surfaces, and increased signal in putamen and caudate head, which are all indicative of CJD.

Furthermore, the patient’s age falls within the typical range for CJD, which is between 57 and 62 years old. Other factors in her history and investigations, such as a viral respiratory tract infection, are unlikely to be the cause of her symptoms. ADEM, which produces many focal areas of central demyelination within 1 to 3 weeks of a viral illness, is also not a likely diagnosis based on the MRI findings and time course.

PML, which results in central demyelination and is caused by activation of JC papovavirus in immunosuppressed patients, is not expected in this patient. The raised TSH may indicate subclinical hypothyroidism, but this is not likely to be the primary cause of her symptoms.

Understanding Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurological condition caused by prion proteins that induce the formation of amyloid folds. These folds result in tightly packed beta-pleated sheets that are resistant to proteases. The disease is characterized by dementia with a rapid onset and myoclonus.

Investigations for CJD include a normal cerebrospinal fluid (CSF), biphasic high amplitude sharp waves on EEG (only in sporadic CJD), and hyperintense signals in the basal ganglia and thalamus on MRI.

Sporadic CJD accounts for 85% of cases, while 10-15% of cases are familial. The mean age of onset is 65 years. New variant CJD affects younger patients, with an average age of onset of 25 years. Psychological symptoms such as anxiety, withdrawal, and dysphonia are the most common presenting features. The ‘prion protein’ is encoded on chromosome 20, and its role is not yet understood. Methionine homozygosity at codon 129 of the prion protein is a risk factor for developing CJD, and all patients who have died from the disease have had this. The median survival time for CJD is 13 months.

Other prion diseases include kuru, fatal familial insomnia, and Gerstmann Straussler-Scheinker disease. Understanding the features and investigations for CJD is important for early diagnosis and management of this devastating disease.

Management of a Patient with Transient Ischaemic Attacks and Atrial Fibrillation

This patient has a history of two transient ischaemic attacks affecting the left anterior circulation. Based on the ABCD Oxfordshire prognostic score, his risk of stroke in the next seven days is 12.1%. He also has controlled atrial fibrillation, hypercholesterolaemia, and elevated blood pressure despite bendroflumethiazide. Although he has a left carotid bruit, it is not significant as it is emanating from the external carotid artery.

To manage his condition, the patient should be started on warfarin for his atrial fibrillation, and a statin and echocardiography should be arranged. Additionally, an additional agent such as perindopril should be added to his medication to control his blood pressure.

It is important to note that the notch3 mutation is associated with cerebral autosomal dominant arteriopathy with subcortical infarcts (CADASIL), which presents as recurrent subcortical infarcts, pseudobulbar palsy, and dementia in mid-adulthood.

Neurological Disorders: Characteristics and Differences

Multiple system atrophy (MSA), idiopathic intracranial hypertension (IIH), multiple infarct dementia, normal pressure hydrocephalus (NPH), and Parkinson’s disease are all neurological disorders with distinct characteristics and differences. MSA is characterized by urinary dysfunction, marked postural hypotension, cerebellar ataxia, and symptoms of parkinsonism. IIH is prevalent in overweight young women and presents with morning headaches and transient visual disturbances. Multi-infarct dementia is associated with vascular risk factors and a step-wise deterioration in cognitive function. NPH is associated with cognitive impairment, incontinence of urine, and gait disturbance, but postural hypotension is unusual. Parkinson’s disease is associated with a more marked tremor and less prevalent postural hypotension and falls in the early stages. These disorders have unique features that distinguish them from each other.

The patient is suffering from pulmonary fibrosis, which is likely caused by rheumatoid arthritis and medication side-effects. Ergot-derived dopamine-receptor agonists like cabergoline, bromocriptine, and pergolide are no longer commonly used in Parkinson’s disease due to their side-effects, including fibrotic reactions in the lungs, retroperitoneum, and pericardium. Instead, non-ergot-derived dopamine-receptor agonists such as pramipexole, ropinirole, and rotigotine are typically prescribed, with levodopa eventually becoming necessary for most patients. Monoamine-oxidase-B inhibitors like rasagiline or selegiline may also be used alone or in combination with levodopa to manage end-of-dose fluctuations. However, it’s important to be aware of the potential side effect of postural hypotension, especially when starting dopamine-receptor agonists. For patients with refractory PD, an apomorphine pump given subcutaneously or deep brain stimulation (DBS) can be highly effective.

Understanding the Mechanism of Action of Parkinson’s Drugs

Parkinson’s disease is a complex condition that requires specialized management. The first-line treatment for motor symptoms that affect a patient’s quality of life is levodopa, while dopamine agonists, levodopa, or monoamine oxidase B (MAO-B) inhibitors are recommended for those whose motor symptoms do not affect their quality of life. However, all drugs used to treat Parkinson’s can cause a wide variety of side effects, and it is important to be aware of these when making treatment decisions.

Levodopa is nearly always combined with a decarboxylase inhibitor to prevent the peripheral metabolism of levodopa to dopamine outside of the brain and reduce side effects. Dopamine receptor agonists, such as bromocriptine, ropinirole, cabergoline, and apomorphine, are more likely than levodopa to cause hallucinations in older patients. MAO-B inhibitors, such as selegiline, inhibit the breakdown of dopamine secreted by the dopaminergic neurons. Amantadine’s mechanism is not fully understood, but it probably increases dopamine release and inhibits its uptake at dopaminergic synapses. COMT inhibitors, such as entacapone and tolcapone, are used in conjunction with levodopa in patients with established PD. Antimuscarinics, such as procyclidine, benzotropine, and trihexyphenidyl (benzhexol), block cholinergic receptors and are now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson’s disease.

It is important to note that all drugs used to treat Parkinson’s can cause adverse effects, and clinicians must be aware of these when making treatment decisions. Patients should also be warned about the potential for dopamine receptor agonists to cause impulse control disorders and excessive daytime somnolence. Understanding the mechanism of action of Parkinson’s drugs is crucial in managing the condition effectively.

Ramsay Hunt Syndrome: A Herpes Zoster Infection

Ramsay Hunt syndrome is a condition characterized by a lower motor neurone facial palsy on one side of the face, often caused by herpes zoster infection of the geniculate ganglion. While a vesicular eruption in the external auditory canal, cranial integument, and oropharynx is typically present, it may not appear immediately or at all. The eighth cranial nerve may also be affected, leading to deafness and vertigo. The virus can be detected through exudates collected from the skin of the pinna and analyzed through PCR. Treatment with a combination of prednisolone and acyclovir is effective if started within seven days of symptom onset. Left-sided acoustic neuroma is unlikely due to the absence of hearing loss and tinnitus, while herpes simplex infection leads to cold sores rather than facial palsy. Atypical migraine may cause paralysis, but the absence of headache and other symptoms rules it out as a diagnosis.

The individual has exhibited classic symptoms of superficial siderosis, including anosmia, bilateral symmetrical ataxia, cognitive impairment, and sensorineural hearing loss. These symptoms can be detected through an MRI of the head, which would reveal prominent siderosis in the posterior fossa. The accumulation of iron is believed to be caused by chronic or past intracerebral hemorrhages. In this particular case, the presence of brain aneurysms associated with polycystic kidney disease is likely to be significant. Other patients may develop siderosis due to previous head trauma or neurosurgery. Unfortunately, there is currently no treatment for superficial siderosis, although some case studies have suggested that lipid-soluble iron chelators may offer some improvement.

Understanding Superficial Siderosis

Superficial siderosis is a condition characterized by the accumulation of iron in the neurons of the central nervous system. This chronic deposition of iron is often caused by chronic bleeding, which can result from a subarachnoid or subdural hemorrhage. The condition is associated with several symptoms, including sensorineural hearing loss, ataxia, dementia, anosmia, and anisocoria.

Sensorineural hearing loss refers to a type of hearing loss that occurs when there is damage to the inner ear or the nerve pathways that transmit sound from the inner ear to the brain. Ataxia is a condition that affects coordination and balance, while dementia is a term used to describe a decline in cognitive function. Anosmia refers to the loss of the sense of smell, while anisocoria is a condition characterized by unequal pupil size.

Cluster Headache: Symptoms, Diagnosis, and Treatment

Cluster headache, also known as migrainous neuralgia, is a recurring condition characterized by severe, unilateral periorbital pain that lasts between 15-180 minutes. This condition is most common in individuals aged 20-50 years, with a strong male preponderance. The pain is often described as boring and can radiate to the frontotemporal region, jaw, neck, or shoulder. Accompanying symptoms include miosis, redness and watering of the eye, and rhinorrhea or a blocked nostril on the same side as the pain.

Oxygen therapy at 100% concentration (6-8 L/min) can provide relief within 10 minutes, while sumatriptan is also effective in treating acute attacks. Prophylactic treatment options include ergotamine, methysergide, verapamil, and prednisolone. Vasodilators such as alcohol, nitrates, and calcium channel blockers may trigger attacks during the acute period but not during remission.

Chronic paroxysmal hemicrania (CPH) is a differential diagnosis that can be distinguished from cluster headache by its shorter attack duration (2-45 minutes), increased frequency of attacks, female preponderance, and selective response to treatment with indomethacin.

In summary, cluster headache is a debilitating condition that can be effectively managed with a combination of acute and prophylactic treatments. Early diagnosis and appropriate treatment can significantly improve the quality of life for individuals suffering from this condition.

Inclusion Body Myositis: A Rare Myopathy with Distal Weakness

This patient is experiencing progressive weakness in both proximal and distal muscles, with associated wasting but no reflex changes or sensory disturbance. The weakness is more pronounced in finger and wrist flexion than in extension, and is not fatigable. These symptoms are consistent with inclusion body myositis, a rare myopathy that can be either sporadic or inherited and is characterized by the presence of inclusion bodies on muscle biopsy.

Inclusion body myositis is unique in that it typically presents with more distal weakness, particularly affecting the finger and wrist flexors and knee extensors. Dysphagia may also occur in some patients. This condition can be distinguished from other myopathies, such as cervical myelopathy or myasthenia gravis, which would not present with these specific symptoms.

While a lower motor neurone variant of motor neurone disease may be considered as a differential diagnosis, fasciculations would be expected in this case. Multifocal motor neuropathy associated with anti-ganglioside antibodies may also be considered, but this would typically present with isolated limb neurology and depressed reflexes. Overall, inclusion body myositis should be considered in patients presenting with distal weakness and wasting, particularly in the absence of fasciculations and upper motor neurone signs.

Optimizing Secondary Stroke Prevention: Medication and Surgical Recommendations

To optimize secondary stroke prevention, the following medication and surgical recommendations should be considered:

– Change aspirin to clopidogrel 75 mg OD: NICE guidance recommends clopidogrel for secondary prevention of stroke and in patients who suffer TIAs whilst on aspirin.
– Add dipyridamole MR 100 mg BD: Aspirin and dipyridamole in combination became the treatment of choice for secondary prevention after stroke or TIA following the results of the ESPS-2 study. Original guidance has been superseded by NICE guidance supporting use of clopidogrel.
– Avoid increasing aspirin to 150 mg OD: Increasing aspirin does not offer a reduction in rates of secondary stroke over a 75 mg daily dose, but does increase the risk of GI haemorrhage.
– Avoid left carotid endarterectomy: There is no indication for carotid endarterectomy with internal carotid artery (ICA) stenosis under 50%.
– Avoid commencing rivaroxaban 10 mg OD: This patient is not in atrial fibrillation, and therefore there is no indication for anticoagulation.

By following these recommendations, healthcare providers can help optimize secondary stroke prevention for their patients.

Autonomic dysreflexia can be diagnosed by palpating a distended bladder. Pulmonary murmurs may be present in the carcinoid syndrome, while a relative afferent pupillary defect is indicative of optic nerve pathology like optic neuritis. Flushing of the skin above the cord lesion is common due to parasympathetic feedback causing vasodilation, but anything below the cord lesion remains vasoconstricted.

Autonomic dysreflexia is a condition that occurs in patients who have suffered a spinal cord injury at or above the T6 spinal level. It is caused by a reflex response triggered by various stimuli, such as faecal impaction or urinary retention, which sends signals through the thoracolumbar outflow. However, due to the spinal cord lesion, the usual parasympathetic response is prevented, leading to an unbalanced physiological response. This response is characterized by extreme hypertension, flushing, and sweating above the level of the cord lesion, as well as agitation. If left untreated, severe consequences such as haemorrhagic stroke can occur. The management of autonomic dysreflexia involves removing or controlling the stimulus and treating any life-threatening hypertension and/or bradycardia.

Diagnosing a patient can be challenging, especially when they are unable to fully participate in the examination. In this case, the patient is experiencing a fever and a clouded consciousness, which could indicate encephalitis. However, other potential diagnoses such as cerebral abscess or Parkinson’s dementia can be ruled out based on the lack of focal neurology or gradual onset. Additionally, a CT scan would typically reveal an old subdural in the case of subdural empyema.

Encephalitis: Symptoms, Causes, Diagnosis, and Treatment

Encephalitis is a condition characterized by inflammation of the brain. It can cause a range of symptoms, including fever, headache, psychiatric symptoms, seizures, and vomiting. In some cases, patients may also experience focal features such as aphasia. While peripheral lesions like cold sores are not related to the presence of HSV encephalitis, HSV-1 is responsible for 95% of cases in adults. The condition typically affects the temporal and inferior frontal lobes.

To diagnose encephalitis, doctors may perform a cerebrospinal fluid test to look for lymphocytosis and elevated protein levels. They may also use PCR to test for HSV, VZV, and enteroviruses. Neuroimaging can reveal medial temporal and inferior frontal changes, such as petechial hemorrhages, although it may be normal in one-third of patients. MRI is a better option, and EEG can show lateralized periodic discharges at 2 Hz.

The treatment for encephalitis involves starting intravenous aciclovir in all cases of suspected encephalitis. This antiviral medication can help reduce inflammation and prevent further damage to the brain. With prompt treatment, many patients can recover from encephalitis without any long-term complications.

Guillain-Barré Syndrome and Peripheral Neuropathy Diagnosis

A history of progressive weakness and loss of tendon reflexes, especially after a recent infection, may indicate Guillain-Barré syndrome, also known as post-infectious polyradiculopathy. It is important to monitor respiratory function regularly, and the best way to do this is by measuring the vital capacity. When diagnosing peripheral neuropathy, a focused clinical assessment that addresses several key issues can significantly narrow down the differential diagnosis.

When a patient is suspected to have experienced a transient ischemic attack (TIA), the preferred imaging modality is a diffusion-weighted MRI of the brain. This type of MRI is highly sensitive to small ischemic lesions and can accurately detect any abnormalities.

In cases of an ongoing ischemic stroke, a CT angiogram is commonly used to quickly detect any occlusions that may require treatment. It can also be used to identify intracranial aneurysms in cases of suspected subarachnoid hemorrhage.

For detecting white matter brain lesions and assessing for leptomeningeal diseases, a FLAIR MRI of the head is a superior method.

T1-weighted MRI is best for demonstrating anatomy and providing contrast for gadolinium-containing compounds, but it is not the most effective imaging sequence for investigating a TIA.

A transient ischaemic attack (TIA) is a brief period of neurological deficit caused by a vascular issue, lasting less than an hour. The original definition of a TIA was based on time, but it is now recognized that even short periods of ischaemia can result in pathological changes to the brain. Therefore, a new ’tissue-based’ definition is now used. The clinical features of a TIA are similar to those of a stroke, but the symptoms resolve within an hour. Possible features include unilateral weakness or sensory loss, aphasia or dysarthria, ataxia, vertigo, or loss of balance, visual problems, sudden transient loss of vision in one eye (amaurosis fugax), diplopia, and homonymous hemianopia.

NICE recommends immediate antithrombotic therapy, giving aspirin 300 mg immediately unless the patient has a bleeding disorder or is taking an anticoagulant. If aspirin is contraindicated, management should be discussed urgently with the specialist team. Specialist review is necessary if the patient has had more than one TIA or has a suspected cardioembolic source or severe carotid stenosis. Urgent assessment within 24 hours by a specialist stroke physician is required if the patient has had a suspected TIA in the last 7 days. Referral for specialist assessment should be made as soon as possible within 7 days if the patient has had a suspected TIA more than a week previously. The person should be advised not to drive until they have been seen by a specialist.

Neuroimaging should be done on the same day as specialist assessment if possible. MRI is preferred to determine the territory of ischaemia or to detect haemorrhage or alternative pathologies. Carotid imaging is necessary as atherosclerosis in the carotid artery may be a source of emboli in some patients. All patients should have an urgent carotid doppler unless they are not a candidate for carotid endarterectomy.

Antithrombotic therapy is recommended, with clopidogrel being the first-line treatment. Aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel. Carotid artery endarterectomy should only be considered if the patient has suffered a stroke or TIA in the carotid territory and is not severely disabled. It should only be recommended if carotid stenosis is greater

The patient has suffered from a subdural haematoma, which is more likely to occur in elderly individuals who are taking anticoagulants or consuming excessive alcohol. The CT scan confirms the diagnosis by revealing a concave-shaped bleed at the back of the head. This is different from an extradural haematoma, which typically presents as a convex-shaped bleed due to the dura mater being detached from the skull with greater force. It is also not indicative of an intracerebral haematoma, which refers to a bleed within the brain tissue itself. The CT scan does not show normal appearances and is not indicative of a subarachnoid haemorrhage, which would display blood within the sulci of the brain parenchyma rather than being confined to the meninges.

There are different types of traumatic brain injury, including focal (contusion/haematoma) or diffuse (diffuse axonal injury). Diffuse axonal injury occurs due to mechanical shearing following deceleration, causing disruption and tearing of axons. Intracranial haematomas can be extradural, subdural or intracerebral, while contusions may occur adjacent to (coup) or contralateral (contre-coup) to the side of impact. Secondary brain injury occurs when cerebral oedema, ischaemia, infection, tonsillar or tentorial herniation exacerbates the original injury.

The question of whether rituximab has caused opportunistic infections and neurological side effects is complex. It is difficult to interpret the neurological signs, as there are no clear clinical indicators that the patient’s MS has worsened. Additionally, her baseline plantar reflexes are likely to be upgoing due to previous transverse myelitis, and a mild reduction in power is expected due to decompensation during acute illness. Therefore, further aggressive immunosuppression would not be appropriate.

Rituximab works by depleting B cells, but the exact mechanisms are not fully understood. If a patient experiences acute deterioration in cognition and agitation while immunosuppressed, it could be a sign of encephalitis or meningitis. The patient may not mount a strong immune response, resulting in a relatively low number of white cells in the cerebrospinal fluid (CSF) and C-reactive protein (CRP) levels. However, elevated CSF protein levels would be consistent with viral encephalitis. A CSF:serum glucose ratio of around 1:2 reduces the risk of bacterial CNS infection. To cover for possible herpes simplex or varicella-zoster encephalitis, the patient should receive intravenous aciclovir while awaiting CSF PCR.

Multiple sclerosis (MS) is a condition that has no cure, but its treatment aims to reduce the frequency and duration of relapses. High-dose steroids are given for five days to shorten the length of an acute relapse. However, it should be noted that steroids only shorten the duration of a relapse and do not alter the degree of recovery. Disease-modifying drugs have been shown to reduce the risk of relapse in patients with MS. Natalizumab, ocrelizumab, fingolimod, beta-interferon, and glatiramer acetate are some of the drugs used to reduce the risk of relapse in MS.

Fatigue is a common problem in MS patients, and amantadine is recommended as a trial treatment after excluding other problems like anaemia, thyroid, or depression. Mindfulness training and cognitive-behavioral therapy are other options for fatigue. Spasticity is another problem in MS patients, and baclofen and gabapentin are first-line treatments. Diazepam, dantrolene, and tizanidine are other options, and physiotherapy is important. Cannabis and botox are undergoing evaluation for spasticity.

Bladder dysfunction is also common in MS patients and may take the form of urgency, incontinence, overflow, etc. Ultrasound is important to assess bladder emptying before prescribing anticholinergics, which may worsen symptoms in some patients. Intermittent self-catheterisation is recommended if there is significant residual volume, while anticholinergics may improve urinary frequency if there is no significant residual volume. Oscillopsia, where visual fields appear to oscillate, is treated with gabapentin as a first-line treatment.

The Royal College of Physicians (RCP) and NICE have published guidelines on the diagnosis and management of patients following a stroke. The guidelines provide recommendations for the management of acute stroke, including maintaining normal levels of blood glucose, hydration, oxygen saturation, and temperature. Blood pressure should not be lowered in the acute phase unless there are complications. Aspirin should be given as soon as possible if a haemorrhagic stroke has been excluded. Anticoagulants should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke. If the cholesterol is > 3.5 mmol/l, patients should be commenced on a statin.

Thrombolysis with alteplase should only be given if it is administered within 4.5 hours of onset of stroke symptoms and haemorrhage has been definitively excluded. There are absolute and relative contraindications to thrombolysis, including previous intracranial haemorrhage, intracranial neoplasm, and active bleeding. Mechanical thrombectomy is a new treatment option for patients with an acute ischaemic stroke. NICE recommends considering thrombectomy together with intravenous thrombolysis for people last known to be well up to 24 hours previously.

Secondary prevention recommendations from NICE include the use of clopidogrel and dipyridamole. Clopidogrel is recommended ahead of combination use of aspirin plus modified-release dipyridamole in people who have had an ischaemic stroke. Aspirin plus MR dipyridamole is recommended after an ischaemic stroke only if clopidogrel is contraindicated or not tolerated. MR dipyridamole alone is recommended after an ischaemic stroke only if aspirin or clopidogrel are contraindicated or not tolerated. Carotid artery endarterectomy should only be considered if carotid stenosis is greater than 70% according to ECST criteria or greater than 50% according to NASCET criteria.

Wilson disease is characterized by copper accumulation in the liver, brain, and kidney due to defective ATPases. This leads to hepatitis, liver cirrhosis, and movement disorders. Diagnosis is confirmed by low serum copper and ceruloplasmin levels and increased urinary copper levels. Kayser-Fleischer rings may be present. Other features include osteomalacia, chronic liver disease, and sunflower cataracts. Homocysteinuria can cause neurological and psychiatric complications. A liver biopsy is the gold standard diagnostic test, but it may be hazardous in patients with deranged clotting.