Medical students and young doctors are taught many questions to ask about headache, most of these questions are not particularly helpful. Whether the headache is constant or throbbing rarely helps to differentiate the various causes of headache, the severity of pain is subjective so once again severity does not help differentiate the cause. The site of the headache is important, a focal headache or focal facial pain may indicate a specific diagnosis such as cluster headache that is always unilateral in or around the eye a as opposed to a generalised headache.

The single most important question to ask is the rapidity of onset from the moment it starts until it reaches its maximum intensity. This will help differentiate between the various causes of headache eg SAH, Meningitis, Migraine and a Hangover! All four conditions will present with headache, nausea, vomiting, photophobia, phonophobia and in some instances neck stiffness.

Correct Answers: Corticospinal Tract and Trigeminal Sensory Nucleus.

Weakness means the motor pathway is affected either in the peripheral nervous system or central nervous system somewhere between the muscle and the motor cortex.

The altered sensation in front of both ears is within the distribution of the trigeminal (5th cranial) nerve.

The altered sensation in front of both ears is the clue and it is the parallel of latitude, but you need a little piece of neuroanatomy knowledge to realise this. The 5th cranial nerve dips down into the upper cervical cord with the lower most fibres representing sensation in front of both ears. An “onion skin” sensory loss spreading forward from the ears is classical for a lesion at the level of the foramen magnum such as a syrinx

One would very much suspect that he has a lesion at the level of the foramen magnum

The appropriate diagnostic test would be an MRI of his upper cervical spine and foramen magnum region.

All Answers are correct!

There are several options when you are uncertain of the diagnosis. The most common employed is to adopt a wait and see approach, this is only ok if you are not dealing with a condition that will cause harm if not dealt with.

A 2nd approach is to obtain a second opinion.

A 3rd approach is the advice given to me by a mentor and outstanding clinical neurologist Dr Arthur Schwieger. One day I said to him “Arthur I have just seen a patient, taken a history and performed an examination and I don’t have a clue what is wrong, what do I do? His reply “Start again” Best advice ever given.

If the x-ray does not detect what you were expecting, then another option is to discuss with the radiologist tell them what you are looking for and make certain the x-ray was of good quality and that the correct area was imaged. You know the patient, sometimes this leads them seeing some thing had missed but more importantly you have confirmed that the correct imaging has been performed and the quality is good.

Each individual episode is clearly migraine because the initial symptoms are resolving before the latter symptoms are appearing or worsening. No other condition other than migraine has this characteristic mode of onset.

However this case highlights a second principle and that is if there is a change in the tempo of an illness you should always think of an alternative explanation or diagnosis.

The unusual aspect of this case is the flurry of events every second day out of the blue in a man who’s never had migraine in the past and there is no family history of migraine.

The diagnostic procedure was a lumbar puncture, it revealed 45 white cells all lymphocytes, a normal protein, a normal glucose and an opening pressure of 32 cm H2O (normal less than 20 cm H2O).

Explanation

This man has a very rare form of migraine referred to as HaNDL; this is an acronym for Headache, Neurological Deficit Lymphocytosis in the CSF. The aetiology of this condition is unknown, it is a self-limiting condition although some case reports suggest that the duration may be shortened by a course the corticosteroids. I do not want to teach you about rare diseases but I wanted to emphasise the principles involved that this case highlights.

His symptoms resolved spontaneously.

Formal Cerebral Angiography has largely been abandoned with the Advent of CT Angiography and MR Angiography. It is still used in the treatment of aneurysms.

Tests rarely help with intermittent disturbances and in this case A contrast CT brain and an MRI brain would both have been normal.

Lesion in the brainstem could produce dysarthria, dysphagia, the incompetent soft palate, possible facial weakness and double vision, however he would have to involve the pons and the medulla without producing impaired consciousness and affecting the limbs – most unlikely. Furthermore, it would not produce neck flexion and neck extension weakness. Anterior horn cell disease i.e. motor neurone disease could produce all these symptoms but the absence of fasciculations makes that diagnosis less likely. Spinal cord compression at the level of foramen magnum, this is where the spinal cord meets the brainstem, cannot produce dysarthria and dysphagia. Clearly this must be Myasthenia Gravis with the problem at the neuromuscular junction

The fluctuating symptoms and the signs clearly point to the diagnosis of myasthenia gravis

Explanation:

The challenge of this case is a situation when you see someone with symptoms that you either have never seen before or that you do not understand. There is a tendency for a number of doctors to say that the patient is psychiatrically unwell. There is also a tendency for other doctors just to give up and say they don’t know, such was the case here. She had consulted three ENT surgeons. So ringing an ENT surgeon would not be an option.

Brief stereotyped symptoms indicate an organic pathology, prolonged symptoms that fluctuate both in intensity and content are more suggestive of a non-organic problems.

When I see someone with symptoms I have never seen before I simply go to the Internet i.e consult Dr Google. There is no excuse for not looking things up. In fact, if you just type in repetitive clicking noises in the ear, you will be a miracle worker and find the explanation. There are 2 causes; one is palatial myoclonus and this was not present in this patient. The other is middle ear myoclonus, if it affects the tensor Tympani muscle and the result is a repetitive vibration noise. However, if it affects the Stapedius muscle the result is a repetitive clicking noise.

The patient’s eustachian tube was blocked. Although I was doubtful that this could be the cause, the first step I took was to get her to unblock the eustachian tube with a nasal decongestant. To do this the patient has to look down at the floor and then hold the decongestant spray vertically in order to directed towards the Ostium of the Eustachian tube. This failed to lead to any resolution of the problem.

Anticonvulsants can be used to treat this or it may require surgery.

Explanation:

Carpal tunnel syndrome is one of the most common neurological problems and yet it seems to be poorly understood by many. The pathognomonic symptom is being woken at night with numbness and/or in the hand(s) that is relieved by hanging the hand out of the side of the bed or moving the hand. It is not uncommon for the condition to be present in both hands. Similar numbness and/or pins and needles can occur when the patient is holding objects, for example – a book, riding a bicycle, driving the car, knitting or mowing the lawns.

Patients do not read textbooks and sometimes their symptoms appear to be atypical. It is not uncommon for patients to feel that the whole hand is affected. It is not uncommon for patients to complain of pain. However, a word of warning “if pain occurs when there are no sensory symptoms present, that pain is very unlikely to be related to carpal tunnel syndrome and therefore very unlikely to resolve with treatment to the carpal tunnel syndrome.” This is the most common reason for failed carpal tunnel surgery, symptoms that were never related to carpal tunnel persist following the operation. Some patients even complain of pain going up the arm.

Interpreting nerve conduction studies in carpal tunnel syndrome

The symptoms of carpal tunnel syndrome are intermittent and, like any intermittent problem, often tests for that problem can be normal. I explain to the patient that the symptoms of carpal tunnel syndrome are due to irritation of the nerve whereas the nerve conduction studies can only detect pressure and damage to the nerve.

Therefore a normal nerve conduction study does not exclude very mild or recent onset carpal tunnel syndrome. It is also not uncommon for patients without symptoms have evidence of median nerve compression at the wrist on nerve conduction studies. In older patients, it is not uncommon for them to present with few symptoms and severe changes on the nerve conduction studies.

The earliest change seen is a mild prolongation of the sensory potential except in the very rare instance of someone with a pure motor carpal tunnel syndrome. As the degree of compression worsens, the sensory latency will be further delayed and the patient will develop a prolongation of the distal motor latency which also increases with worsening severity. Initially, it may only be prolonged compared to the corresponding distal latency of the ulnar nerve but subsequently it is prolonged in absolute terms.

Permanent numbness and weakness only occurs with more severe compression and therefore more severe findings on the nerve conduction studies. In this patient, the findings were only mild and therefore could not be the explanation for permanent numbness. Once again, one needs to define the exact nature and distribution of the symptoms. This man had tingling on the back of the hand between the first and second digits radiating up the lateral border of the right forearm. The distribution was in that of the C6 nerve root. His Brachioradialis reflex was absent, strength was normal. He clearly had a painless sensory C6 Radiculopathy.

Thus the answer is question the diagnosis of carpal tunnel as the cause for his symptoms.

Explanation:

The principle here is that seizures are brief, stereotyped, with positive phenomena (no loss of function), with or without a fall and or LOC. His episodes do not fit this definition.

Fluctuating symptoms, particularly if associated with symptoms outside the nervous system such as difficulty breathing or chest discomfort, are not primarily neurological in origin. The trembling in the Emergency Department and ambulance was hyperventilation syndrome with reproduction of his symptoms he was made to hyperventilate.

It was assumed that the if accident had occurred as the result of a seizure, that the seizures were caused by his extensive brain damage from previous head injury. The questions to ask are: has the patient had anything to suggest minor seizures? Such as if the patient has ever had any unexplained motor vehicle accidents. Ask if they miss parts of television programmes or conversations suggesting they switched off. Ask if they have been called a daydreamer and then ask family members if they have seen any episodes in which “the lights are on but there is no one home”. These would all point to clinically unrecognised absence or complex partial seizures. Remember simple partial seizures refer to those that do not involve loss of awareness whereas complex partial seizures (which used to be called temporal lobe epilepsy) are where the patient has impaired awareness of what is happening around them and is unresponsive for a short period of time often with a short period of postictal confusion.

The cause of the accident?

An eyewitness was spoken to and they saw him slump at the wheel, there was no evidence of a convulsion.  He had been very sleep deprived and recalled being very drowsy, his car was witnessed to slowly veer across to the left side of the road before it crashed. He had been up most of the night before and had very little sleep, clearly he fell asleep at the wheel. Although another possibility is that he had an arrhythmia causing him to fall asleep at the wheel. He was monitored for 48 hours in the Emergency Department and no arrhythmia was detected, but this doesn’t exclude an arrhythmia.

Explanation:

If you thought he might have epilepsy you would have agreed with the doctors who have seen him for the previous 46 years. However, you were wrong. Although a rising sensation is often a typical Aura of a seizure, if this was the aura of a tonic-clonic seizure, then he should have actually had convulsions and he didn’t. If this was a complex partial seizure he would have blackouts, but he wouldn’t have fallen to the ground or slumped in the chair. If it were a seizure, he wouldn’t wake up immediately and be perfectly normal even after a few brief jerks. If it were a simple partial seizure, he would not blackout and he would not fall. The brief loss of consciousness with rapid return to normal indicates almost certainly a Cardiac Arrhythmia. An implantable loop recorder was inserted and within one month 3 episodes of complete heart block were documented. His “seizures” resolved following insertion of a pacemaker.

Explanation:

Take a proper history and perform a detailed and directed examination! Ask the patient exactly where the pins and needles are, in this case they were affecting the palm and dorsal aspect of the fifth finger. That could be an ulnar nerve lesion but it could also be a C8 radiculopathy. Both can cause pain although pain is much more common with a radiculopathy. Palpation of the ulnar nerve in the groove at the elbow reproduced the tingling and pain. This man was resting his elbow on hard surfaces and irritating his ulnar nerve. The principal here is that symptoms are due to irritation of the nerve whilst the nerve conduction studies can only detect problems when there is pressure and damage to the nerve. This is particularly common in carpal tunnel syndrome and ulnar nerve lesions. The positive Tinel’s sign at the elbow clearly indicates that this is an ulnar nerve lesion at that site, he was advised to avoid resting his arm on the other surfaces.

A clinical diagnosis of trochanteric bursitis was made and treatment was recommended with rest and ice packs. The first ice-pack was applied on day two, it lead to increased pain and stiffness and increased difficulty walking. The patient was encouraged to persevere with that ice-pack, reluctantly a second ice-pack was applied and the result was exactly the same.

Continue to the next question.

The principle is that if a treatment does not work then it is either:

Wrong Treatment

Wrong Dose

Wrong Diagnosis

The diagnostic test was an ultrasound of the thigh that demonstrated calcific myositis, a very rare condition which usually follows an injury to the muscle and the formation of a haematoma. The patient develops infiltration of calcium within the haematoma in the muscle causing severe pain. Steroids and local anaesthetic were injected into the site of calcium formation with significant but temporary relief of pain.

The diagnosis was correct but there was a possibility of an osteosarcoma and therefore a plain x-ray of the femur was performed to ensure that the calcium was in fact confined to the muscle. This revealed isolated fluffy calcium confined to the Vastus Lateralis muscle.

A second injection of a higher dose of steroids and local anaesthetic was given with complete relief of symptoms.

I was able to return to my golf and bike riding. Yes, this was me!

The first 3 of these diagnoses are possible with the history that you were given, each can cause pain, weakness and sensory loss. A pronator syndrome can cause weakness, rarely it can be painful but it does not cause sensory symptoms.

However the pattern of weakness affecting the 3^rd muscle abductor pollicis brevis and 4^th muscle the long flexors of the lateral two digits (flexor digitorum profundus innervated by the median not the ulnar nerve). The pattern of weakness pointed to a proximal median nerve lesion. This is the sort of weakness you would see with a pronator syndrome, but as we have said it shouldn’t produce sensory loss. The sensory loss is due to involvement of the medial cutaneous nerve of the forearm and this is close to the median nerve in the upper arm at the insertion of coracobrachialis muscle several centimetres above the elbow. Initial nerve conduction studies revealed a marked reduction in the amplitude of the median response of abductor pollicis brevis with normal sensory findings in the hands and normal conduction velocity in the median nerve. All other nerves were normal, although the medial cutaneous nerve of the forearm was not tested in the first study. Electromyography was also not performed during the first study because the symptoms had only been present for five days and denervation take somewhere between 10 days and three weeks to appear.

The distribution of his pain was beyond that of a single nerve root and followed the line of the right trapezius muscle. In fact this muscle was very tender to palpation. Thus, his neck pain was due to spasm of the trapezius muscle, this cannot explain the numbness in his hand, however, so there must be two problems.

He did not have pain radiating down the lateral aspect of the forearm that one would expect to see with a C6 radiculopathy. There is no way that a C6 nerve root compression could produce the sensory symptoms that this man had in the fourth and fifth digits of the right hand, that is either the ulnar nerve or a C8-21 nerve root problem.

A diagnostic procedure was performed, it was nerve conduction studies that demonstrated a straightforward ulnar nerve lesion at the elbow and, using Kimura’s inching technique, the site of compression was just at the medial epicondyle. The patient had a positive Tinel’s sign with palpation of the ulnar nerve at the elbow.

If you chose an S1 radiculopathy that could explain the onset of severe pain whilst in bed but once again this is very unusual.The preserved ankle reflex would be atypical for an S1 radiculopathy.

I was uncertain so if you chose uncertain that is a reasonable answer.

The patient should probably not have been sent home from the emergency department without diagnosis when the pain was so severe. The treating doctors were told the ultrasound of his calf was completely normal so they hadn’t found the explanation for his pain.

When I saw him, I thought he must’ve had a ruptured Achilles tendon, but why would this happen in the middle of the night when he is not weight-bearing?

The first thing that I did was to check that the original ultrasound did not reveal deep vein thrombosis by ringing the radiology department, they checked and were absolutely convinced that the scan was of excellent quality, had examined the correct area and was normal. However they only scanned the calf. I then checked the sensitivity and specificity of ultrasonography in the detection of DVT. Ultrasonography is considered to be the best non-invasive diagnostic method for DVT showing an average sensitivity and specificity of 97% for proximal deep vein thrombosis. However, ultrasonography cannot be relied on to diagnose calf vein thrombosis, the sensitivity for symptomatic calf vein thrombosis has been reported to be as low as 75%. Thus, it is conceivable that the first scan did not detect a deep vein thrombosis in the calf. However, I was still uneasy because such severe pain with a DVT is very unusual.

An inability to stand on the toes suggests a problem with the Achilles tendon or the gastrocnemius and soleus muscles. The two diagnostic tests performed at the bedside were tests that I had never heard of before. I simply looked them up on the Internet. As I suspected he may have ruptured his Achilles tendon, I looked for bedside tests. The first was the calf squeeze or Thompson’s test. In a normal person when you squeeze the calf the foot plantar flexes, if there is a rupture of the Achilles tendon the foot does not plantar flex. His did not plantar flex. The 2nd test, the knee flexion test or Matles test, is where the patient lies prone on the bedside and bends their knees 90° from the horizontal, in a normal person the sole of the foot flexes at the ankle and the toes are above the heel. In this man his right foot remained horizontal. Thus the two bedside tests suggested the probability of torn Achilles tendon. I discussed the appropriate radiological procedure with a radiologist and they recommend initially an ultrasound of the Achilles tendon and if that was unhelpful then an MRI scan.

The ultrasound was completely normal! There was no evidence of an Achilles tendon tear!

A further diagnostic procedure was requested. Continue to the next question.

The correct answer is Start Again!

A reasonable answer would have been refer for another opinion. However, that is not the point we are trying to make here.

Shortly after starting my own private practice I saw a patient where, at the end of the history and examination, I did not have any idea what was wrong or what to do next. I asked my mentor Dr Arthur Schwieger who was in his rooms on the other side of the corridor. He said two words that have echoed in my ears ever since “start again”

I asked the patient to return, repeated the examination, confirmed the findings. I had allowed my presumptive diagnosis to influence my interpretation of the tests. It is clear that these two bedside tests are not specific for a ruptured Achilles tendon. I then performed electromyography and H reflex. There was evidence of denervation in the gastrocnemius muscles and the H reflex was delayed on the symptomatic side confirming an S1 radiculopathy. This diagnosis should have been obvious to me from the start, I allowed myself to be influenced by two bedside tests that I found on the Internet to diagnose my suspected diagnosis of a ruptured Achilles tendon. I am unable to find any papers that document the sensitivity and specificity of Thompson’s test, but like all tests it will have a less than 100% sensitivity and specificity.

Why would an Achilles tendon rupture in the middle of the night when someone is lying in bed, it just wouldn’t happen. I jumped to conclusions without considering the facts carefully enough.

Ultrasound is the investigation of choice for a suspected Achilles tendon, if it is normal an MRI scan will not help. I have appended to the end of this case a detailed discussion of MRI versus ultrasound for musculoskeletal medicine that is an excellent reference source.

One should never hesitate to refer the patient for a second opinion, particularly if they request one, never refuse.

If you tell the patient you can’t find anything wrong they will simply go to another doctor.

Explanation:

The distribution of this man’s pain was in the area supplied by the C6 nerve root and the associated sensory symptoms clearly indicate that his pain was neurological in origin. A bursa of his shoulder cannot produce sensory symptoms. If the pain is related to shoulder pathology then the pain will be exacerbated by movement of the shoulder and will not be associated with any sensory symptoms. If the pain is related to nerve root compression then the pain is often but not always exacerbated by movement of the neck. Carpal tunnel syndrome can produce numbness in the second finger, but it would not produce pain radiating down from the neck to the index finger. A cervical rib can produce pain and numbness, but it compresses the lower cord of the brachial plexus and therefore the numbness would be in the fourth and fifth digits, within the distribution of the C8-T1 nerve roots.

Bursitis of the shoulder is very common in middle-aged to older people and is not always the explanation for their symptoms.

Unilateral headache initially confined to the region of the temple would be most unlikely with idiopathic intracranial hypertension. Furthermore, there was no convincing papilloedema (there have been rare cases described of IIH without papilloedema) and the blind spot was not enlarged – it is enlarged if there is swelling of the optic disc.

Migraine rarely, if ever, causes daily headache all day every day unless the patient develops a very rare entity described as “status migraine”. We are all taught that throbbing headache occurs with migraine but it also occurs with other causes of headache and migraine is not necessarily a throbbing pain. The presence of a family history of migraine is circumstantial evidence and should not be used to make a diagnosis. Photophobia and phonophobia are also very non-specific as anyone who has ever had a hangover would be aware of. Remember the pain of migraine comes on rapidly, and a characteristic feature of common migraine, or in the new nomenclature, migraine without aura (migraine without any visual, gastrointestinal or neurological symptoms) is that patients retire to bed without a headache and wake in the middle of the night or awaken at their normal time the next morning with a severe headache.

Chronic daily headache by definition is a headache present all day every day 6 months, therefore she does not fulfil this criteria as the headaches had only been present for 4 months.

The most likely diagnosis is tension headache and there was considerable stress in her life. She was very unhappy in her work, this latter fact was elicited after one came to the conclusion that this was most likely tension headache. Tension headaches can be very difficult to treat, I usually advise people to change their lifestyle and find more time for themselves in addition to time spent at their work and with their family. The patient was advised to return if the headaches didn’t settle.

The correct diagnosis proved to be myasthenia gravis.

All tests are used in the diagnosis of Myasthenia Gravis. Repetitive exercise of his limbs resulted in severe weakness that recovered with 30 seconds, only to recur with repeat testing.

Repetitive stimulation studies may demonstrate a decremental and it is usually done on a proximal muscle such as the trapezius.

A Tensilon test looks for the reversal of weakness (initially a 2mg dose and then after a pause 8mg as some are sensitive to small doses others need a bigger dose) looks for resolution of weakness with onset within 30 seconds of injection and reappearance of weakness 2-3 minutes after the injection. Atropine is given first to avoid vomiting induced by then Tensilon. This test in not particularly easy if there is no fixed deficit, eg permanent ptosis.

SFEMG (single fibre EMG) will detect increase jitter in myasthenia but it is not specific for myasthenia, it can occur in motor neuron disease and Lambert-Eaton Syndrome.

Thyroid function tests are indicated as it is not uncommon to have co-existent thyroid disease.

The Ice Test is very useful if th4ere is ptosis. A block of ice is applied to the drooping eyelid and it magically opens!

The film ET has a classic line “Phone home”. I have often been able to make many a diagnosis by obtaining a blow by blow description from an eye-witness.

The first “diagnostic procedure” was to call an eyewitness. The episode had occurred whilst the patient was doing yoga, at the stage where she was told to take deep breaths for relaxation. The patient stated that she needed to go to the toilet but when she stood up she actually sat in a chair and her limbs started trembling and jerking while she was fully conscious. This episode lasted 15-20 seconds and she seemed to pass out for 20 or 30 seconds with her eyes closed and being motionless. There was incontinence of urine. On coming to, she was fully oriented, not confused, although did not understand what had happened.

The correct answer is hyperventilation syndrome. Only tonic-clonic and atonic seizures make the patient fall. An tonic-clonic seizure has little or no warning, the patient is “out to it” and they are confused after the episode. AN atonic seizure has no warning the patient drops but remains fully conscious and is not confused afterwards.

A Stokes-Adams attack is complete heart-block. There is no warning, a fall with loss of conscious and no confusion after the episode unless they hit their head in the fall

When symptoms vary in nature and duration between episodes this is usually a clue that the problem is of psychological origin. Thus an non-epileptic seizure is a possibility

It is the long warning and the dryness of the mouth, difficulty getting her breath and tingling in the right arm that are the clues to the correct diagnosis of hyperventilation syndrome. Her incontinence probably reflected a full bladder. It has to be said that it is unusual for the patient to fall with hyperventilation syndrome usually they remain conscious and can describe the entire episode. Her symptoms including “out to it” were reproduced by vigorous hyperventilatoin

Osteoarthritis can produce pain affecting the metatarso-phalangeal joints but will not be associated with sensory loss.

The distribution of the sensory findings is that of the deep peroneal not the superficial peroneal nerve but this cannot explain the pain on palpation of the 2^nd, 3^rd and 4^th metatarso-phalangeal joints

Morten’s neuroma specifically refers to a neuroma on the nerve between the third and fourth digits.

Thus, the information given to you so far means you cannot be certain about the diagnosis.

Beneath the site that elicited the shooting pain was a palpable lump and this patient almost certainly has a neuroma on the deep peroneal nerve, but this cannot explain the pain involving the metatarso-phalangeal joints.

The MRI scan of her foot showed inter-metatarsal bursal fluid at the 1st, 2nd and 3rd interspaces, this usually occurs in mechanical stress. This is almost certain an explanation for the pain elicited by her daughter and the pain elicited when we examine those joints.

Although we are taught Occam’s razor, it is not always correct and one needs to carefully analyse every aspect of the patient’s symptoms and signs to come to the correct diagnosis in this patient.

Please continue to the next question for the diagnosis.

Most 56-year-old patients will have degenerative disease of the cervical spine. The distribution of the sensory symptoms is not reflective of that of the C6 nerve root and therefore the findings on the CT scan of the cervical spine are irrelevant or an incidental finding. Disc disease compresses the nerve below the vertebrae ie C6 nerve below C5 vertebra, thus it cannot be a C5 radiculopathy.

An MRI scan is more sensitive at detecting nerve root compression in the cervical and thoracic spine while a CT scan is adequate in most cases with problems in the lumbosacral spine.

He was booked for cervical surgery after an MRI scan of the spine.

The patient himself requested another opinion for another diagnosis. The examination at that time demonstrated weakness of both APB muscles and also sensory loss affecting the tips of the digits of the lateral 4 1/2 fingers of both hands on the palm up to the wrist but not the dorsal aspect.

Two provocative tests were performed at the bedside, Tinel’s sign where the median nerve is precast using attendant hammer commencing 6 cm above the wrist and coming downwards and into the proximal palm, a positive test is when the patient gets tingling or numbness in the hand and fingers, not pain at the site of percussion. Phalen’s sign is where the wrists are either forcibly flexed or extended and once again a positive test is where the patient experiences tingling or numbness in the fingers, pain is not a positive sign. These tests don’t have a high sensitivity and specificity.

The diagnostic procedure of course was nerve conduction studies that confirmed the patients diagnosis i.e. bilateral carpal tunnel syndrome.

The lateral aspect of this case highlights the importance of establishing the exact nature and, more importantly, the distribution of the symptoms. If he had C6 nerve root compression he would more likely have pain radiating down his arm, although you can have painless nerve root compression. If the compression affected the dorsal root then you would expect to see sensory loss in the C6 dermatome which is the lateral aspect of the forearm and the lateral two digits, not the third and fourth digits. If this were an ulnar nerve lesion you would not expect the third digit to be affected.

If the motor nerve root was affected one would expect to see weakness affecting the C6 nerve root which would give you partial weakness of biceps, brachioradialis and to a lesser extent triceps, there may also be some weakness of supraspinatus and infraspinatus and deltoid, together with a reduced biceps reflexes possibly an absent brachioradialis reflex.

Bilateral symptoms of nerve root compression is very rare indeed, if a patient has bilateral numbness in the hands it is far more likely to be related to carpal tunnel syndrome because this is the most common condition affecting the nerves in the upper limbs.

A diagnosis of alcoholic hepatitis was made (Dr jump to conclusions) based on his excessive alcohol consumption (circumstantial evidence) and he was treated appropriately, however he lost his ability to communicate due to severe hoarseness.  His tests for haemochromatosis showed he was heterozygote, making this diagnosis an unlikely.casue for his cirrhosis His autoantibody tests for autoimmune cirrhosis. Anti-mitochondrial antibodies (AMA) are present in sera of approximately 90-95% of patients with primary biliary cirrhosis (PBC).  Smooth muscle antibodies (SMA) of IgG class are found in 92% of sera from patients with chronic active hepatitis but in only 20% of sera from primary biliary cirrhosis,  whereas the incidence of IgM-SMA is higher in PBC (67%) than in chronic active hepatitis  (38%).

Myasthenia can give a hoarse voice extremely rare isolated laryngeal myasthenia cases have been described but it would be very unlikely to occur . Myasthenia does not cause cirrhosis.

The low ceruloplasmin raised the possibility of Wilson’s disease but it was borderline low and this can occur with liver disease . A normal serum copper does not exclude this entity.

A search for hoarseness with chronic liver disease did not find any cases but a search for hoarseness with Wilson’s disease found several case reports. The dysphonia may be related to dystonic vocal cords.

A slit lamp examination detected Kayser-Fleischer rings and his 24 hour urinary copper was markedly elevated confirming the diagnosis of Wilson’s disease.

The dysphonia was treated with Botox injections.

He has a classic Brown-Sequard syndrome indicating a lesion in one side of the spinal cord. The motor pathways cross at the level of the foramen magnum, thus if he has in the left leg the problem must be on the left side of the spinal. This fists with the altered sensation affecting the right side of the body as the spinothalamic pathway crosses the moment it enters the spinal cord, thus the spinothalamic pathway is also affected on the left side of the spinal cord. The upper level of the sensory signs is the armpit about T2. The upper motor neuron signs in the left arm indicate the lesion was above C5 on the left.

No question just a good review

The correct answer is D). I made an incorrect diagnosis when I first saw this man, I allowed his past history to influence my thinking. I thought he might have had something giving him intermittent ventricular obstruction such as a colloid cyst or a lesion in the fourth ventricle causing the intermittent headaches and then subsequently causing what appeared to be a pontine syndrome with involvement of the left sixth nerve in all four limbs causing weakness. I ordered an MRI scan of the brain that was completely normal! The error was assuming that the headaches must be involved in the current illness, but he didn’t have the headache at the time he developed the weakness and if the diagnosis that I had originally made was correct he should have had the headache. In fact over the next 24 hours he developed increasing weakness of his limbs  that had spread to involve all muscle groups with loss of reflexes except for the ankle reflexes! There was neck flexion weakness. The correct diagnosis was acute inflammatory demyelinating peripheral neuropathy (AIDP). Botulinum poisoning s can resemble AIDP, the clue “pentad” of symptoms signs with nausea and vomiting, dysphagia, diplopia, dilated and fixed pupils,  dry mouth and throat. I would have caution, sometimes these patients have been reported as responding to phoneme the drug used to test for myasthenia gravis. Myasthenia gravis can cause diplopia and for limb weakness, the reflexes would be normal. Other conditions to consider would be Lambert-Eaton syndrome and tickborne paralysis.

The altered sensation to pain and temperature indicates that the spinothalamic tract is affected on the left side, using the sensory signs alone all you can say is that there is a lesion on the left side of the brain above the mid-pons where the 5th cranial for trigeminal nerve is located. This is your “Meridian of longitude”. The “parallel of latitude” is the presence of the pain in the same distribution. This is the very rare central pain syndrome seen in thalamic infarct. In 99.9 % of cases when pain is of neurological origin it clearly points to a problem in the peripheral nervous system.

Weakness in both legs in the right arm almost certainly reflects involvement of the central nervous system, it would be very atypical although not entirely impossible to have a peripheral nervous system problem where three limbs are affected. The increased tone points to a central nervous system problem, flaccid paraplegia can occur in central nervous system problems. The presence of seizures and impaired cognitive function points to involvement of the cerebral hemispheres and the bilateral grasp reflexes and palmomental reflex point to the frontal lobe. This was a case that I saw in Rwanda, they do not have access to neuropsychology, although all this would confirm is that the problems was in the frontal lobe. They also do not have access to neuro-physiology nor do they have access to MRI scans except for two private scanners in the capital Kigali, this case was not seen in Kigali.

The correct answer was a CT scan of the brain. This demonstrated a massive meningioma affecting both frontal lobes.

It is sometimes difficult to tell the difference between abnormal movements due to structural pathology and abnormal movements reflecting underlying psychological problems.

Fasciculations without weakness are usually benign, this is often referred to as medical students motor neurone disease because they notice fasciculations and worry that they have that diagnosis. He has no sensory symptoms, thus anyone of the listed diagnoses are possible once weakness develops however fasciculations are more sinister. Fasciculations and weakness in upper and lower limbs can be caused by motor neurone disease, multifocal motor neuropathy or Isaac’s syndrome (Neuromyotonia). Fasciculations would indicate a lower motor neurone problem. Painful cramps with painful bulging muscles can occur with neuromyotonia and less commonly with multifocal motor neuropathy. In the latter condition the pattern of weakness should reflect involvement of multiple motor nerves.

On the information that you have been given it is not possible to differentiate between multifocal motor neuropathy, motor neurone disease for neuromyotonia.

A diagnostic procedure was performed, please proceed to the next question

If you said migraine, that was the explanation for the headaches that had been occurring over the preceding 18 years. However the classic feature of cluster headache is that one can set ones watch to them, they occur at exactly the same time every day, although she is female and cluster headache is unusual in females, she had the other characteristic features which include ptosis, ipsilateral headache that was severe, watering of the eye and she almost certainly had a small pupil but hadn’t noticed it. The presence of a Horner’s Syndrome with recurrent unilateral headache is pathognomonic for cluster headache. Cluster headache can occur once per day or several times per day, it often occurs at the same time each year, bouts may last as long as six weeks although in some patients such as this one chronic cluster headache can develop.

The presence of a Horner’s Syndrome in someone presenting with a severe acute ipsilateral headache could also suggest the possibility of other pathology, in particular a carotid artery dissection.

The pain is beyond the distribution of the trigeminal nerve and therefore it cannot be trigeminal neuralgia, tension headache tends to come on towards the end of the day but it is not so stereotyped, often varying in intensity between one episode and another, fluctuating in intensity when present and not associated with ptosis or watering of the eye. Atypical facial pain presents with a more constant boring, burning discomfort in the face without such periodicity.

Headaches that are precipitated upon standing and relieved by lying flat occur following a lumbar puncture and these are referred to as post-LP headache, they are due to a CSF leak. A spontaneous CSF leak can occur, often following very trivial injury to the neck or back. Patients present with the identical headache to those seen after lumbar puncture, severe on standing and abolished by lying flat with the feet slightly elevated, if the head is slightly elevated it would be greatly relieved that may not be abolished. It is generally bilateral and worse in the occipital region and frontal region.
Chronic daily headache is a headache that is essentially present all day every day, fluctuating in severity from hour to hour day to day. It can get worse with exertion but it is not abolished by lying flat. It is usually bilateral frontal and occiptial and non throbbing.
Occioital neuralgia is a unilateral stabbing or shooting pain that last seconds at a time and can recur many times per day

Benign exertional headache can be of sudden onset although often there a mild headache that increases in severity as the exertion continues. The past and family history of migraine was a red herring totally irrelevant and put here as a warning not to use the past or family history to make a diagnosis.

This patient had a past history of migraine aura without headache consisting of spreading photopsia. The distortion of body image is referred to as metamorphopsia. It is characteristic of the “Alice in Wonderland Syndrome. First described in 1955, Alice in Wonderland syndrome (AIWS) is a perceptual disorder characterised by distortions of visual perception (metamorphopsia), the body schema, and the experience of time. The name refers to Lewis Carroll’s well-known children’s book Alice’s Adventures in Wonderland,¹ in which Alice feels (among other things) her body growing both larger and smaller. A 2016 review found 170 cases in the literature (Blom, J.D.  Alice in Wonderland syndrome. A systematic review. Neurol Clin Pract. 2016 Jun; 6(3): 259–270).

The following table is taken from Bloms article

It is said that more mistakes are made by not looking and not knowing, the clue was that she couldn’t sit on the buttock because it was so tender. Localised tenderness detected on examination points to local pathology it is not seen with referred pain. The radiological findings in the lumbosacral spine need to be ignored because such degenerative changes are very common and often asymptomatic. An ultrasound of the buttock did not reveal an explanation, I since learned that for deep tissue ultrasound is not useful. An MRI scan demonstrated a low-grade partial-thickness tear of the anti-margin of gluteus medius, the rectus femoris and mild tendinosis at the origin of the hamstring tendons and focal chondrosis near the chondrolabral junction

Elite female cyclists frequently suffer from secondary amenorrhoea and osteoporosis and thus a person this age could have suffered and impacted fractured neck of femur with a fall. Exacerbation of pain by external rotation is the clue to hip joint pathology.

The muscles of the buttock consists of the gluteus medius, gluteus maximus and gluteus minimus. These are supplied by the inferior gluteal nerve. These muscles are supplied by the superior and inferior gluteal nerve on the nerve root origin is L5-S1. In theory the possible nerve root compression at that level could cause pain in this region but it cannot explain localised tenderness.

Despite the absence of reddening and watering of the eye, blockage and ipsilateral nostril, miosis and ptosis the onset at exactly the same time of the day is typical for cluster headache. A dilated temporal artery has been described with cluster headache.
Temporal arteritis affects people over the age of 60, although the temporal artery may be dilated it is not pulsatile, it is thrombosis and tender. Patients with temporal arteritis often have associated scalp tenderness and jaw claudication (pain on chewing). Occipital neuralgia by definition must be confined to the distribution of fixable nerve, his pain affected the temple and occipital region supplied by the first division of the trigeminal nerve and the acceptable nerve. Occipital neuralgia Giselle antedating brief pain and does not last 30 minutes. Unilateral throbbing headache could be migraine but the onset at exactly the same time each day is not a feature of migraine. All though this could be tension headache whilst driving home in heavy traffic, tension headache is rarely throbbing in nature and when asked if he experienced this headache at the same time of the day when he was not driving he answered yes.

The correct and is nonepileptic seizures. There are many reasons for this diagnosis. First of all they are predictable, the warning symptom is far too long for an aura, the episodes last too long for absence or complex partial seizures and although confusion can occur after a complex partial seizure, the description of unresponsiveness with fluttering the eyelids is more in keeping with an absent seizure in which case the patient should return to normal immediately the seizure has finished and these occur without any warning whatsoever. Another clue is the eyes darting chaotically in all directions.

Many of the female patients that I have seen with nonepileptic seizures have been sexually assaulted and that was the case here. This poor lass has suffered sexual assault before the age of 13 from both her father and family friend. The treatment is not to give anticonvulsants to recommend psychological counselling.

This is a very typical case of migraine aura without headache. The sensory symptoms commencing one part of the body and spread very slowly over several minutes to affect other parts of the body. Although focal century seizures can spread from one part of the body the other the spread would be far more rapid. One can see paroxysmal symptoms in multiple sclerosis but they don’t spread in this manner. The classic paroxysmal symptoms with multiple sclerosis include trigeminal neuralgia and paroxysmal tonic seizures. They are often very brief on the matter of seconds which is too short for seizures and migraine without headache. In transient ischaemic attacks the symptoms usually are maximum at onset, only very, very rarely do they spread and there is usually a loss of function with a transient ischaemic attack.

This is a tricky case, the right-sided weakness point to a problem on the right side of the spinal cord below the level of the foramen magnum or on the left side of the brain above the level of the foramen magnum. The numbness suggests involvement of the spinothalamic tract this pathway crosses immediately after it enters to spinal cord. This means the problem has to be on the left side of the spinal cord or left side of the brain. Pain when it is due to a neurological problem is always a parallel of latitude. The neck pain with pain radiating into the occipital region indicates pain at the level of C2 on the left side. Thus the symptoms would suggest lesion at the upper end of the spinal cord lower end of the medulla i.e. at the level of the foramen magnum. An MRI scan demonstrated a haemangioma blessed over at the level of the foramen magnum extending down into the upper aspects of the cervical spine and also extending into the posterior fossa above the level the foramen magnum.

Cluster headache is classically unilateral and around the eye. A characteristic feature is that patients can almost set the clock to them, they can occur at the same time every day for weeks on end or occasionally in patients with chronic cluster headache for months or years. The pain is of rapid onset and excruciatingly severe, often leading to patients banging their head on the wall or walking around. They can be precipitated by alcohol and many patients stopped drinking alcohol during a bout of cluster headache. The other characteristic features include an ipsilateral blocknose, ipsilateral watering of the eye and a pathognomonic symptom is ptosis with miosis i.e. a temporary Horner’s syndrome. Temporal arteritis is not usually paroxysmal, A normal ESR does not exclude temporal arteritis particularly in the early stages, temporal arteritis usually occurs in patients over the age of 65-70 years of age. Many patients have radiological findings of sinusitis but unless there is a fluid level the sinusitis is unlikely to be a cause of headache. If you chose paroxysmal hemicrania that would not have been an unreasonable choice. Some regard paroxysmal hemicrania as a variant of cluster headache but the headaches of paroxysmal hemicrania are usually much shorter in duration (1-20 minutes, up to 240 minutes) and more frequent (one every second day up to 25 per day), they respond very well to indomethacin where as cluster headache does not. The dose of indomethacin may have to be fairly high.

The pattern of weakness does not reflect what one would see with bilateral ulnar nerve lesions of the elbow. Patient would have weakness of finger abduction and of the long flexors of the media two digits. A C8-T1 radiculopathy is also incorrect because these patients have weakness of finger abduction and weakness of flexion of the distal phalanges is all four digits, they do not have wrist extension weakness. Although the ANF was 1:160 this is borderline in a patient of this age and does not indicate a diagnosis of SLE. Her anti-Ro antibodies would be more in keeping with mild scleroderma and Crest syndrome. Patients with myotonic dystrophy can present with distal wasting and weakness but there were no symptoms to suggest myotonia, there was no frontal baldness, no cataracts and no diabetes. There is an extremely rare entity called distal myopathy and a very rare form called Welander distal myopathy has been reported to have associated sensory involvement. (reference Udd B. Distal myopathies. Curr Neurol Neurosci Rep. 2014;14:434).

These two cases reiterate the importance of establishing the exact distribution of pain in the limb. One needs to ascertain whether the pain is in a joint or whether it represents soft tissue injury such as lateral epicondylitis. Most pain is not of neurological origin, however if it is it must be in the distribution of a nerve root or single nerve. Pain can occur in more than one nerve root in patients with brachial neuritis and lumbosacral neuritis.

Well done, a sensation of ants crawling on the skin is referred to as formication. The clue was that the episodes occured at exactly the same time every day, 2 hours after his dose of Levetiracetam (Keppra). I simply googled Levetiracetam and formication and found it was a recognised cause. His episodes ceased shortly after ceasoing the Levetiracetam. It was safe to stop the drug as he had never had seizures.

This case highlights severasl points. First of all when sympotmss are stereotyped ie the same each time they are very likely to be of organic origin.
Secondly seeing patients with intermittent symptoms at the time of their symptoms is more likely to establish the cause. If the ENT Surgeons had told her to play the trumpet until the symptoms developed they woould have seen she developed palatal incompetence with nasal escape.
Thirdly this is something I had nevere seen before, a not uncommon scenario so I searched the internet and found the diagnosis immediately.It is velopharyngeal incompetence a career ending move in 30% of musicians who generate high pressures inside their mouths is trumpet, clairent and oboe players.
The patient herself discovered a solution. She applied as swimmers nose peg to her nostrils!

It is crucial to ascertain the exact distribution of sensory symptoms sa this provides the clue to the diagnosis. I consult the anatomy textbook firmly imprinted on my medial temporal lobe. Anyone can look up an anatomy textbook to see what nerve or nerve root supplies the area affected. Sensory symptoms would have to be in the distribution of a single nerve, multiple nerves (eg peripheral neuropathy), a nerve root or reflect involvement of the spinothalamic tract. In this case the distribution of the sensory symptoms pointed to the deep peroneal (fibular) nerve.
It is not sciatica as the pain and sensory symptoms should affect the same area relecting compression of a lumbar or sacral nerve root. The pain is not in the same area and is clealry unrelated. it wa almost certainly a Morton’s neuroma , although one cannot be entirely sure as the pain is usually between two adjacent digits and is oftne burning in nature. A Morton’s neuroma is thickening of the tissue around one of the nerves leading to the toes . It is not a peripheral neuropathy as the sensory symptoms are focal and not in a circumferential pattern affecting the tips of the toes upwards. It is not a common peroneal (fibular) nerve lesion at the neck of the fibula,as this casues altered sensation on a greater area on the top of the foot and often the lateral aspect of the shin,with or without a foot drop.
The sensory symptoms are in the distribution of the deep peroneal (fibular) nerve. A most unusual problem. Palpation along the course of the nerve detected tenderness and a lump 14 cm proiximally indicating a neuroma on the nerve.

These episodes are brief, unpredictable, stereotyped and involve positive phenomena. This is the classic feature of seizures. The fact that he remains alert during these episodes means that they are simple-partial seizures. A normal EEG can be seen in patients with simple partial seizures. He had previously undergone a craniotomy for a meningioma. The goosebumps and piloereection(hairs standing on end) indicate involvement of the autonomic nervous system.

“The distribution of his burning pain was down the anterior aspect of his right thigh, clearly NOT in the distributon of the L4 nerve root and not in the pattern of an individual nerve or nerve root.Careful examination showed patchy alteration to pain and temperatuse senstation down the right side up about T10 and subtle weakness with increased reflexes and an upgoing plantar reflex on the left. He had absent right biceps and brachioralialsi reflexes but that is on the qworng side and these were also asymptomatic. He had a hemicord lesion on the left, because the motor pathway crosses at the level of the foramewn magnum, the so called Brown-Sequard syndrome. The absent reflexes were due to nerve root compression at the level of C5 and C6 on the right. His MRI revealed a thoraci cavenous malformation on the left side of his spinal cord at T9.