• 2 Nov, 2020
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FieldsCluster Headache Acute Angle Closure Glaucoma Giant Cell Arteritis Encephalitis Extradural Haemorrhage Medication OveruseMalignant HTNMeningitis Raised Intracranial Pressure Intracranial Venous Thrombosis Tension Headache Sinusitis Subdural Haemorrhage Subarachnoid Haemorrhage 
Age Peak age 20-50yrs Increases with Age >55yrs All Ages Increases with age Usually Adults Common in Young Adults All Ages Increases with age Peak 20-35 years Most commonly Young Adults Increases with age Increases with age Peak 35-65 years 
Gender M:F 5:1 M=F M=F M=F M=F M=F M>F M=F M=F F>M F>M M=F M=F F:M 3:2 
Typical Presentation A 40 y/o male smoker visits his GP with a 1 year HX of episodic, intense, unilateral pain in his head, most often focussing around one eye, which lasts for about an hour each time. He goes through periods of having these attacks frequently, and may subsequently be without them for several months. They are often brought on by alcohol. An elderly man presents with a headache and an acutely painful red eye, associated with a reduction in visual acuity. His symptoms are worse in the dark. On examination, he has a semi-dilated, non-reacting pupil An elderly patient with a 2 week history of unilateral headache associated with pain on chewing and brushing her hair presents to her GP. A 45 year old woman brings her husband to A&E after he develops a headache and becomes increasingly confused. She says he has been talking jibberish and thinks that he may be hallucinating. On examination, his GCS is reduced and he has a fever. A middle-aged man presents with a head injury after falling down some stairs. He has a wound on his right temple. After losing consciousness, he quickly recovers, but complains of a worsening headache. Over the next few hours he becomes more confused and has one seizure. A 30 year old woman with a history of migraines develops a constant, severe headache which is not relieved with medication. She takes sumitriptan and over the counter analgesics daily with no improvement. A 20 year old African male is brought to A&E with a headache, chest pain and shortness of breath. On examination, his blood pressure is 240/210mmHg An anxious woman brings her baby into A&E with a purpuric rash and a fever. He has loud pitched cry which she says he has been doing for several hours with intermittent periods of remaining silent and stationary. An elderly female patient presents with a constant headache associated with nausea. The headache is present at night and worse when coughing. A young woman presents with an acute headache associated with unilateral periorbital oedema. Her only drug history is a prescription for the OCP. On examination, she has a lateral gaze palsy. A 20 year old woman presented to her GP with a 6 month history of feeling low and stressed. She has had several headaches which last up to a couple of days, and feel as if someone has tied a tight band around her head. A 30 year old woman presents to her GP with a two week history of a dull, constant headache which is worse when she bends over. She has recently had a cold and on examination, has some tenderness overlying her maxillary and frontal sinus. An elderly alcoholic man presents with a persistent headache after falling over the previous day. On examination, he has fluctuating levels of consciousness. A 50 year old woman presents to A&E with ‘the worst headache ever’ and thinks she may have been hit on the back of the head. When questioned further, she also complains of a stiff neck. She has a medical history of hypertension and polycystic kidneys. On examination, she has focal neurological signs and shortly afterwards, she begins vomiting and has a seizure. 
Other Symptoms ● Bloodshot eye ● Lid swelling ●Lacrimation ● Facial flushing ● Rhinorrhoea ● Unilateral congestion of sinuses ● Constant aching pain around one eye ,radiating to forehead ● N&V ● Generalised headaches ● Scalp tenderness ● Jaw claudication associated with jaw and mouth pain ● Visual loss (temporary/ permanent) ● Weight loss ● Malaise ● Morning stiffness ● Headache ● Photophobia ● Fits ● Malaise ● Vomiting● Stiff neck ● Fatigue ● Hallucinations ● Convulsions ● Lucid interval following trauma ● Increasingly severe headache ● Vomiting ● Fits ● Constant headache ● Headache ● Blurred vision ● Change in mental status – anxiety, confusion ● Chest pain ● Cough ● N&V ● Seizure ● SOB ● Headache ● Photophobia ● Stiff neck ● Nausea● Vomiting ● Fits ● Transient loss of vision Note: presentation varies according to vein affected. ● Generalised headache ● Vomiting ● Seizures ● Pressure or tightness all around the head ● Pressure behind eyes ● Bilateral, non-pulsatile headache ● Scalp muscle tenderness ● Dull, constant aching pain over the frontal/maxillary sinus ● Postnasal drip ● Coryza Single episode, usually subacute onset over 2-48h following trauma. May be very gradual onset over days or even weeks. Initial traumatic event may be trivial and may not be remembered ● ‘Worst ever’ headache, often occipital ● Stiff neck ● Vomiting ● Collapse ● May be a sentinel headache 
Pain Acute onset, recurrent Acute single episode Acute onset Acute single episode Single episode, subacute onset over period of a few hours following trauma Chronic Acute, medical emergency Acute single episode Chronic Usually acute, but can be chronic (depends on vein affected!) Chronic and episodic; diffuse, dull ache Acute single episode ● Fluctuating levels of consciousness ● Insidious physical or intellectual slowing ● Raised ICP ● Localising neurological symptoms (e.g. unequal pupils, hemiparesis) long after injury Sudden onset, single episode 
Signs ● Miosis ● Ptosis ● ipsilateral Horner’s Syndrome ● Reduced visual acuity ● Visual haloes● Red, congested eye ● Cloudy cornea ● Dilated, non-responsive pupil ● Superficial temporal artery may become tender, firm and pulseless ● Blindness ● Low grade fever ● Amaurosis fugax ● Unequal weak pulses ● Dyspbiea ● Fever ● Kernig’s and Brudzinksi’s signs ● Odd behaviour ● LOC or reduced GCS ● Cranial nerve palsies ● Hemiparesis ● Memory problems ● Tremor Infants: ● Irritability ● Poor appetite ● Fever ● Gradual reduction in GCS ● Coma ● Confusion ● Hemiparesis ● Brisk reflexes ● Upgoing plantar ● Ipsilateral pupil dilation ● Bilateral limb weakness ● Respiratory arrest ● Bradycardia ● Hypertension ● No neurological signs ● Extreme HTN ● Oedema of lower limbs/feet ● Abnormal heart sounds ● Pleural effusion ● Abnormal reflexes ● Reduction in GCS ● Reduced U/O ● Weakness of limbs/face ● Ophthalmoscopy: flame-shaped haemorrhages, hard exudates, cotton wool spots, papilloedema ● Kernig’s and Brudzinski’s signs ● Fever ● Purpuric rash ● Signs of shock ● Lymphadenopathy Infants: ● Bulging fontanelle ● High pitched cry● Paradpxical irritability ● Hypotonia ● Focal neurological signs ● Worse when walking/ lying down/ coughing ● Papilloedema ● False localising signs ● Behavioural change ● Impairment of consciousness Note: presentation varies according to vein affected. ● Papilloedema ● Visual disturbances ● Focal CNS signs ● Cranial nerve palsies ● Cerebellar signs ● Encephalopathy● Worse on touching scalp ● Aggravated by noise ● No neurological signs ● Tender overlying skin ● Pain worse on bending forwards ● Sleepiness ● Headache ● Personality change ● Unsteadiness ● Seizures ● Focal neurological signs ● Reduced GCS/LOC ● Kernig’s sign after 6h ● Retinal or subhyaloid haemorrhage ● Hemiplegia ● Papilloedema ● Coma 
Past Medical History ● Smoker ● Alcohol precipitates attacks ● FH (autosomal dominant) ● Attacks may be precipitated by emotional upset or sitting in the dark, e.g. at cinema ● Polymyalgia rheumatica ● FH ● Immunocompromised ● HIV ● Cytotoxic drug therapy ● HX travel or animal bite ● Osteoporosis ● Hypertension ● History of falls/trauma ● DH of regular/ excessive medication use, esp mixed analgesics, e.g. containing paracetamol, codeine, opiates, ergotamine and triptans ● PMH headaches ● HX HTN ● Black African ● SLE ● Systemic sclerosis ● Periarteritis nodosa ● Renal artery stenosis ● Renal hypertension ● Immunocompromised ● HIV ● Cytotoxic drug therapy ● Pain may wake sufferer from sleep ● ● DH: oral contraceptive pill, corticosteroids, heparin ● Hx head trauma ● Neurosurgical procedures: dural taps/infusions into internal jugular vein ● Hx hypercoagulable state, e.g. antiphospholipid syndrome, protein S and C deficiency ● Pregnancy ● Hyperhomocysteinaemia ● Nephrotic syndrome ● Sarcoidosis ●Associated with stress, noise, concentrated visual effort, fumes/smells ● Hx depression ● URTI ● Diabetes ● Immunocompromised ● HIV ● Smoker ● Tooth infection ● Anticoagulants ● Alcoholic ● Elderly ● History of frequent falls/trauma, e.g. alcohol/epipepsy ● Hypertension ● Atherosclerosis (causes brain shrinkage) ● Hypertension ● Smoker ● Known aneurysm ● Ehlers-Danlos syndrome ● Polycystic kidneys ● FH ● Post-menoausal in women ● Coarctation of the aorta 
Bloods ● Serum histamine levels rise during attacks: ‘histamine cephalgia’ ● None specific ● ESR >40-50mm/h ● CRP very high ● FBC: normocytic, normochromic anaemia ● High platelets ● LFT: Low albumin, High ALP, High GGT ● Viral serology of blood ● PCR of blood ● None specific ● None specific ● ABG ● Creatinine ● Blood urea nitrogen ● WCC raised ● Blood glucose ● Blood cultures ● None specific ● None specific ● None specific ● Often normal, but may show: ● High WCC ● Raised inflammatory markers ● None specific ● None specific 
Imaging ● No investigations necessary if typical presentation ● None specific ● Head CT: often shows cerebral oedema in temporal lobes ● MRI: inflammation ● CT/MRI: Lentiform-shaped lesion which is well circumscribed with strong adherence to dura. ● X-ray: skull fracture ● No investigations necessary if typical presentation ● CXR: May show congestion in lung and enlarged heart ● Head CT scan to rule out DDX: intracranial ass lesion etc. ● CXR ● CT/MRI to exclude space-occupying lesion ● CT/MRI venography: may show absence of a sinus, though an absent transverse sinus may be a normal variant ● MRI T2-weighted gradient echo can visualise thrombus directly ● CT – may be normal until ~1 week after event. ● No investigations necessary if typical presentation ● CT if symptoms exceed 12 weeks ● Nasal endoscopy in chronic cases ● CT/MRI: typical crescent of blood around outer edge of brain in one hemisphere and midline shift of brain structures. ● CT: star-shaped lesion; blood fills gyral patterns around the brain ventricles 
Additional Investigations ● Temporal artery biopsy: intimal hypertrophy, inflammation of the intima, degradation of the internal elastic lamina, giant cells, lymphocytes and plasma cells in the internal elastic lamina. ● Lumbar puncture: only if imaging excluded intracranial mass. High CSF protein and lymphocytes and low glucose ● CSF analysis: excess lymphocytes and protein ● EEG: slow wave changes and periodic complexes in temporal lobes (if HSV encephalitis) ● Throat swab ● MSU ● LP CONTRAINDICATED AS MAY PRECIPITATE CONING ● Urinalysis ● Lumbar puncture: urgent CSF microscopy – high WCC and protein ● Auramine stain (tuberculosis) ● Indian ink stain (Cryptococcus) ● Lumbar puncture contraindicated until after imaging due to risk of precipitating coning. ● Lumbar puncture: CSF may show RBCs and xanthochromia ● Lumbar puncture; CSF is uniformly bloody early on and then becomes xanthochromic after several hours. 
Full Article Cluster HeadacheGlaucomaGiant Cell ArteritisEncephalitisExtradural HaemorrhageMedication Overuse HeadacheMalignant HypertensionMeningitisNot Available(Sorry!) - You could always write oneTension HeadacheNot Available (Sorry!) - You could always write oneSubdural HaemorrhageSubarachnoid Haemorhage