SAZA.GVAVA.16.11.0929

Volume 9 No 3 - Issue 31 2016

N eur o l ogy N ewsl etter

Page 3 NEURON SA Volume 9 No 3 Issue 31 2016

“What is portrayed as a science, can sometimes be scarily close to guesswork”

- Steven Hatch, Snowball in a Blizzard

Editor: Dr Jody PearlNeurologist

Sunninghill HospitalJohannesburg

This newsletter is proudly sponsored by

NeurologyDr Jody PearlDr Dominic Giampaolo

Paediatric NeurologyDr Tiziana Aduc

NeuroradiologyDr Farrell Spiro

CardiologyDr Anthony Stanley

Paediatric CardiologyDr Jeff Harrisberg

Powerdigm Health ConsultancyDr Victor Ramathesele

Special edition - Interesting case presentations

Editorial Dr Jody Pearl 3

Cerebral amyloid angiopathy Dr. Taniel TownsendDr. Diana Alagem 4

Sodium channel Myotonia Congenita - differentiating this from chloride channel myotonia Dr Lia Mavrodaris 8

Raeders Paratrigeminal Syndrome Dr Dominic Giampaolo 10

Ethics - Euthanasia and the psychiatrist Dr GC Verster 12

Editorial board

ince the beginning of modern medicine, the advances have come with ever-increasing speed in all fields of medicine. In the last decade we have mapped the human genome, we can access any topic with the most up-to-date information with the push of a

button.

Heart attacks and strokes, previously catastrophic, can be treated with clot busters in an emergency unit, diseased organs can be removed using robotic surgery and we can peer into the mind of an individual using functional MRI. Yet medicine is subject to far more uncertainty than we are willing to acknowledge and this uncertainty lies at the heart of what doctors do on a daily basis.

Misdiagnosis in neurology occurs in even the most common conditions. Take epilepsy for example, in a recent study of patients referred to an epilepsy centre nearly 25 % received a misdiagnosis. Simple disorders learnt as medical students such as syncope, migraine, sleep disorders and non-epileptic seizures are missed resulting in patients being started on toxic anti-epileptic medication and having their driver’s license revoked.

We have all encountered patients with headache disorders such as cluster headache or simple migraine that have been subjected to sinus surgery after several failed courses of antibiotics and anti-histamines or the trigeminal neuralgia patient who has had multiple teeth extracted.

Unfortunately patients don’t read like textbooks and we cannot adopt the “one fits all” approach. Every time a new patient walks through the door, even with what seems like a

straightforward problem, it is imperative that we apply our minds before making a diagnosis and starting treatment and not rely on our overconfidence. Over-diagnosis and over- treatment is more dangerous than sometimes admitting that we simply don’t know.

We still have much to learn!

“There are known, knowns; there are things we know that we know. There are known unknowns; that is to say there are things we know we know we don’t know. But there are also unknown unknowns; these are things we don’t know we don’t know.”

- Secretary of state Donald Rumsfeld 2002

NEURON SA Volume 9 No 3 Issue 31 2016 Page 4

Cerebral amyloid angiopathy

Dr Taniel Townsend, Neurologist, Linksfield HospitalDr Diana Alagem, Radiologist, Diagnostic Radiological Services Inc.

Johannesburg

HisTory

74-year-old gentleman Mr. W, was brought to see me by his wife at the request of his general practitioner. The history provided was that he had suffered two “mini strokes” in 1999 for which he had been treated by a neurologist. At that stage he was put on aspirin,

a statin and blood pressure tablets. With the second event he developed left-sided weakness and had a seizure. He was put on phenytoin due to recurrent left focal motor seizures.

According to his wife his condition was stable until 2005 when she noted personality changes. In particular, he appeared to be demotivated and lacked interest in everything apart from that that affected him. Over the next few years his memory became worse. He continued to lack interest in daily activities, and began to stop talking. He also seemed to be oblivious to what was going on around him. More recently she noticed that he didn’t “travel well”, being very confused and forgetful while they were away and returning to normal after being at home for a few days.

Past medical history • Habits - He was previously a smoker, but had stopped 30

years ago. He did not drink alcohol.• Medication – combination antihypertensives, antiplatelet

agent, statin, SSRI, phenytoin, allopurinol.• Obstructive sleep apnoea.• Prostate cancer diagnosed in 2008, in remission.

on examination• Systemic examination was unremarkable.• Higher functions: He scored 16/30 on MoCA, and was

unconcerned with his deficits.• Cranial nerves: No obvious deficits• Motor examination: Tone, power, reflexes were unremarkable• Cerebellar examination: Normal• Extrapyramidal examination: Bradykinetic

AssessmentI assessed Mr. W as most probably having a vascular dementia, and ordered an MRI as well as outstanding bloods that the GP hadn’t done in the workup of dementia.

resultsHis bloods were unremarkable.His MRI showed features that were in keeping with amyloid angiopathy reporting leucomalacia, evidence of lacunar infarcts, diffuse micro hemorrhages as well as superficial siderosis.

ProgressI informed Mrs. W of the probable diagnosis and outcome, and advised that the antiplatelet agent be stopped.

Figure 1. T2 weighted image - Leucoencephalopathy Figure 2. Mag images - Superficial siderosis (focal subarachnoid hemorrhages)

Mri images of Mr. W.

Page 5 NEURON SA Volume 9 No 3 Issue 31 2016

Figure 3. SWI sequence Figure 4. SWI sequence

Figure 5. SWI sequence Figure 6. GE T2*W sequence

Figure 7 Figure 8 Figure 9

Figure 5 & 6. Demonstrating that SWI sequence more sensitive than Gradient echo T2* sequence in identifying microhaemorrhages

Figures 7,8 &9 - micro haemorrhages tended to spare the cerebellum, pons and basal ganglia

Figures 3 & 4. Diffuse microhemorrhages at the grey white interface

NEURON SA Volume 9 No 3 Issue 31 2016 Page 6

DiscussioNCerebral amyloid angiography (CAA), also known as congophilic angiopathy due to the aggregations of amyloid being demonstrated after the application the stain Congo Red, involves deposition of amyloid in the cerebral blood vessels.

It is classified into various types according to the amyloid protein involved, and so far seven proteins have been reported. Of these, sporadic amyloid ß-protein (Aß) is the one most commonly found in older individuals and patients with Alzheimer’s disease.

EPiDEMiologyThe prevalence increases with age, and occurs in approximately half of elderly individuals. It is a relatively frequent incidental finding, found on screening imaging in up to 16% of asymptomatic elderly patients. Autopsy studies have found a prevalence of up to 9% in patients between 60 and 69 years, and up to 58% in patients over the age of 90. Autopsies of patients with evidence of Alzheimer’s disease have found CAA in the vast majority of cases (90%).

PATHology AND PATHoPHysiologyCAA is observed mainly in the leptomeningeal and cortical vessels of the cerebral lobes and cerebellum. The occipital lobe

is preferentially affected, whereas changes in the basal ganglia, thalamus, brainstem and white matter are uncommon. In mild cases a small proportion of the leptomeningeal and superficial cortical vessels are affected. However, in severe cases most small arteries and arterioles are also affected, with their vessel walls often being totally replaced by amyloid deposits (except for the endothelial cells). The amyloid deposits in capillaries and arterioles appear to infiltrate the surrounding parenchymal tissue, and accompany dystrophic neurites forming plaque-like structures. Recently, CAA has been divided into capillary CAA (CAA-Type 1) and non-capillary CAA (CAA-Type 2). CAA-Type 1 can be accompanied by a local robust neuroinflammatory response and perivascular phospho tau accumulation that is not commonly associated with the larger vessels in CAA-Type 2.

Severe CAA is associated with vasculopathies that include obliterative intimal changes, hyaline degeneration, microaneurysmal dilatation and fibrinoid necrosis. In particular, fibrinoid necrosis is closely associated with CAA-related hemorrhage.

The pathophysiology of Aß-type CAA is shown in Figure 10. CAA-associated vasculopathies lead to development of haemorrhagic lesions, ischemic lesions, and encephalopathies. In addition, CAA

Figure 10 - Pathophysiology of cerebral amyloid angiography related disorders. J Stroke. 2015 Jan; 17(1):17-30

Page 7 NEURON SA Volume 9 No 3 Issue 31 2016

is related to dementia through Alzheimer’s disease pathology and CAA-related vascular lesions.

cliNicAl fEATurEs of cAA

intracerebral haemorrhages (icH)

Symptomatic lobar haemorrhage• Not deep ICH as sporadic AB-type CAA is rarely found in

deep vessels.• According to two studies (Helsinki ICH study and National

Taiwan University Hospital stroke registry) CAA related ICH was the second most common cause of ICH following hypertensive angiography.

Cortical microhaemorrhages• Seen in 47% of pathologically confirmed CAA cases• Frequently lobar in distribution • Confer a considerable risk of future symptomatic lobar ICH.

cortical superficial siderosis (focal convexity sAH) Found in 60% of pathologic confirmed cases of CAA.

White matter disease and cortical infarction• CAA related occlusive small vessel disease may cause

progressive white matter lesions and lacunar infarcts. • There is an occipital lobe propensity for the lesions

compatible with the predilection of CAA pathology for posterior brain regions.

Dementia • Noted in 74% of individuals with severe CAA at autopsy. • Cognitive deficits are most notable in perceptual speed

(reaction + response time), which is separate from the effects in Alzheimer’s pathology.

cAA-related inflammation• A subset of patients who present with seizures, subacute

cognitive decline or headaches• Asymmetric hyperintensities on T2 imaging with micro

haemorrhages seen on SWI. • Responsive to immunosuppressive therapy.• Neuropathologic examination has generally revealed

angiitis of CAA-affected vessels, with inflammation appearing to represent an autoimmune response to vascular ß-amyloid deposits.

DiAgNosis

imagingCT/MRICT is an option for macrohaemorrhages. However, MRI is more sensitive for detecting microhaemorrhages and superficial siderosis. Findings include:

• Microhaemorrhages that tend to be sub cortical, and are best seen on SWI sequences (difficult to see on conventional T1 and T2 sequences).

• Cerebral haemorrhage, usually superficial lobar.• Superficial siderosis, seen in up to 60% of patients.• Leucoencephalopathy.

Differential diagnosis• Hypertensive micoangiopathy - usually seen in basal

ganglia, pons and cerebellum.• Multiple cavernoma syndrome - usually random in

distribution and size, with often characteristic cavernous malformations identified.

• Haemorrhagic metastasis - usually larger and enhancing.• Neurocystercercosis - nodular calcified stage, and random

in distribution• Prior radiotherapy - although very similar in appearance

due to similar pathophysiology, the distribution is related to the treatment field.

Functional imagingImpaired vascular reactivity in response to visual stimulation or visual tasks has been reported, consistent with greater distribution of CAA in the occipital lobe. Biochemical markersDecreases in CSF Aß and higher levels of CSG total tau and phosphosylated tau have been reported. The presence of anti-Aß autoantibodies in CSF is a marker for CAA-related inflammation.

Diagnostic criteriaThe Boston Criteria were proposed for the diagnosis of CAA ICH, with a sensitivity of 89.5% reported in a small pathologic series. When superficial siderosis was added (the modified Boston criteria) the sensitivity increased to 94.7%. Specificity remained unchanged at 81.2% for both.

TrEATMENTThere are currently no disease modifying therapies available for CAA. Subacute leucoencephalopathy associated with CAA-related information is reported to respond to immunosuppressive therapy.

references1. Cerebral Amyloid Angiopathy: Emerging Concepts. M.

Yamada. JoS 2015;17(1):17-30.2. MRI of Cerebral Microhemorrhages. AJR 2007;189:720-

725.3. Radiopedia.

NEURON SA Volume 9 No 3 Issue 31 2016 Page 8

Patient profileThe patient is a 15 year old scholar. He complains that for as long as he can remember, he feels “stiffness” when initiating certain movements. This is especially noticeable when running, specifically starting a sprint, after which he notes that, the movement becomes easier after repetition. Stiffness is often relieved by repeated contraction of the muscle. He often feels the symptoms after exercise too, especially when swimming. His symptoms are worse with cold. The stiffness is sometimes painful. He feels as though he will fall if suddenly confronted with an obstacle and has been noted to suddenly drop objects. More recently, he describes some articulation difficulties and the sensation of having a “big tongue”.

His mother reported that as a child, he would have difficulty in opening his eyes after closing them tightly. Therefore stiffness in all striated muscle groups including the extrinsic eye muscles, facial muscles and tongue are described.

His activities have always taken slightly longer to complete. In terms of extracurricular activities, he has tailored them to those with which he can cope.

He has had a muscular physique from a young child.

There is no co-morbidity apart from Osgood-Schlatters disease affecting the left knee.

He has a 19 year old sister and a 10 year old brother, neither of whom display similar symptoms. His father gives a long history of stiffness in his arms and legs, and cramping after sport, but has never been diagnosed with a specific condition. His grandfather has a muscular appearance.

On examination, all the vital signs and systemic examination were normal and there were no musculoskeletal deformities. He has a muscular build out of keeping with his exercise history; this involves all muscle groups but especially the deltoid and trapezius muscles.

On neurological examination, myotonia is clearly evident on eye closure, finger grip, and thenar percussion. Tongue myotonia is also present. Warm up phenomenon is present.

The rest of the neurological examination is normal with specifically no weakness, normal tone and reflexes.

Extramuscular manifestations such as cataracts, abnormal cardiac conduction, or endocrine dysfunction are absent.

The following investigations were performed:• Bloods: raised CK on 2 occasions (in the 700s and 200s)• Nerve conductions were normal• EMG: insertion or movement of the needle results in

bursts of various fluctuating motor unit discharges, with

a clearly audible “dive-bomber” sound, in keeping with myotonic discharges. Otherwise, motor unit appearance and recruitment pattern was normal.

The features seen were suggestive of a channelopathy like myotonia congenita.

The family history was suggestive of an autosomal dominant type like Thomsen’s disease. In addition, the presentation in infancy/childhood, with hypertrophied muscles, athletic appearance, and muscle stiffness after rest, warm-up phenomenon, and percussion myotonia was all suggestive of Thomsen’s disease.

Myotonia congenita is caused by mutations in the CLCN1 gene on chromosome 7, which codes for chloride channels on the membranes of skeletal muscles. Analysis of the gene usually identifies a mutation in 90% of the individuals with a clinical diagnosis of Thomsen’s disease. If no fault is identified, then it may mean a mutation in the SCN4 gene.

Paramyotonia congenita (PMC) and sodium channel myotonia (SCM) are caused by mutations in SCN4A which encodes the alpha subunit of the skeletal muscle voltage gated sodium channel, NaV1,4. These mutations causing myotonia are dominantly inherited and result in a gain of sodium channel function.

DNA samples were sent to the Netherlands Radboud University Medical centre, Department of Human Genetics.

Single gene testing was performed. No pathogenic mutations were detected in the CLCN1 gene by DNA sequence analysis of the coding region and splice sites. Therefore, the diagnosis of autosomal dominant myotonia congenita caused by a mutation in the CLCN1 gene could not be confirmed.

The DNA was subsequently tested for SCN4A gene mutations and a heterozygous pathogenic mutation was detected in SCN4A gene. It is a missense mutation which has been described in several families with myotonia.

DiscussionMutations of the SCN4A gene, which encode the skeletal muscle voltage gated sodium channel NaV1,4 cause various skeletal muscle disorders. Paramyotonia congenita, sodium channel myotonia, Hyperkalaemic periodic paralysis, and Hypokalaemic periodic paralysis are representative of these disorders. To date, more than 60 mutations of SCN4A have been reported. However, these disorders are not described adequately because of the heterogeneity and the complicated pathophysiology of ion channels.

A case series in Japan reported five patients that had been encountered with SCN4A mutations, exhibiting various phenotypes. Three patients had sodium channel myotonia, one had sodium channel myotonia with periodic paralysis, and one had hyperkalaemic periodic paralysis with myotonia.

Interesting case presentation Sodium channel Myotonia Congenita - differentiating this from chloride channel myotonia

Dr lia Mavrodaris Neurologist

Olivedale, Randburg

Page 9 NEURON SA Volume 9 No 3 Issue 31 2016

The overlapping forms of sodium channelopathies are not unheard of.

The clinical characteristics are skeletal chloride and sodium channel myotonia is summarised in Table 1. Specifically sodium channel myotonia is difficult to distinguish from chloride channel myotonia on clinical grounds. Some other helpful clues are that eye closure myotonia appears to be more frequent in sodium channel myotonia, and many individuals with sodium channel myotonia have painful myotonia.

Evaluation following initial diagnosisThe above patient and his family had a consultation with a medical geneticist. Consultation with neurology was to evaluate the need for treatment.

Treatment of Manifestations• Some individuals with minor complaints only need to

accommodate their activities and lifestyle to reduce symptoms.

• Myotonic stiffness may respond to sodium channel blockers or other pharmacological treatment options:

• Mexiletine, a lidocaine derivative, is the best documented treatment option. In a double blind randomised trial, mexiletine (200mg 3 times a day) significantly reduced stiffness in a group of 59 patients with myotonia, 34 of who had myotonia congenita. In clinical practice, doses generally begin at 150 mg twice a day, increasing slowly as needed. The most common potential side effects include epigastric discomfort, nausea, lightheadedness, dizziness, tremor, and ataxia. These are reversible with dose reduction.

• Other sodium channel blockers such as phenytoin and carbamazepine have been reported to have a beneficial effect.

• Compounds with other presumed modes of action such as quinine, dantrolene or acetazolamide may be beneficial in some cases.

• A trial is currently ongoing for lamotrigine.

Agents/circumstances to avoidIn general, anaesthesia should be used with caution. Particular care should be taken with depolarising muscle relaxants because they may result in anaesthesia related events. Because life threatening muscle spasms and secondary ventilation difficulties occurred following a preoperative injection of suxamethonium, it is recommended that this is avoided.

In rare cases, injections of adrenaline or selective beta-adrenergic agents in high doses may aggravate myotonia.Propranolol has likewise been reported to aggravate myotonia.Colchicine may cause a myopathy with myotonia in individuals with renal insufficiency and may thus also, in theory, aggravate myotonia of individuals with myotonia congenita.

This case and discussion demonstrates the complexity, variability and overlapping nature of clinical features of the skeletal muscle sodium and chloride channelopathies. These are basically treatable disorders, so as in this patient, it is essential to reconsider genetic testing.

References1. NCBI bookshelf. A service of the National library of medicine,

national institutes of health. Pagon RA, Adam MP, Ardinger HH, et al, editors. Gene Reviews. Seattle (WA; UNIVERSITY OF WASHINGTON Seattle; 1993 to 2015.

2. Phenotypic variability in childhood of skeletal muscle sodium channelopathies Harumi; Yoshinaga et al. Okayama, Japan. Pediatric Neurology 52 (2015) 504-508.

3. Muscle channelopathies: recent advances in genetics, pathophysiology and therapy. Karen Suettwrlin, Roope Manniko, and Michael Hanna. Current opinion inn Neurology (2014).

Table 1. Summary of clinical characteristics

Disorder

Recessive myotonia congenita

(Beckers disease)

Dominant myotonia congenita (Thomsen disease)

Sodium channel myotonia

(SCM)

Paramyotonia congenital

(PMC)

Hyperkalaemic periodic

paralysis/ PMC

Hyperkalaemic periodic paralysis

Gene mutation CLCN1 CLCN1 SCN4A SCN4A SCN4A SCN4A

Age at onset <10 yrs or later <10 yrs or later <10 yrs <10, early <10 <10

Clinical myotonia + + + + + +-

Severity Moderate to severe Mild to moderate Mild to severe Mild to moderate Mild to severe Mild

Warm up + + +- - - +

Paradoxical myotonia - - - + + +-

Cold sensitivity +- +- +- + + +-

Delayed myotonia - - +- - - -

Painful myotonia +- - +- - - -

Episodic weakness +- - - + + +

Persistent weakness +- - - - +- +-

Muscle hypertrophy + +- +- +- +- -

Muscle atrophy +- +- - - - -

Potassium sensitivity - - + +- + +

NEURON SA Volume 9 No 3 Issue 31 2016 Page 10

Raeder’s Paratrigeminal Syndrome

Dr Dominic giampaoloNeurologist

Rosebank

year old physiotherapist presented with flushing of her right cheek and pain behind her right eye. This was followed after 2 days by ptosis of the right eyelid and some miosis and pain in the distribution of her right ophthalmic division of the right trigeminal nerve.

No significant past medical or surgical history.

MRI scan and MRI angio were normal. All laboratory work normal.

The patient was started on Prednisone 40mg with good relief and some improvement of the ptosis. After weaning off the cortisone after a month, the pain and ptosis returned and the prednisone was restarted with good effect. Patient still has some pain and minimal ptosis after 2.5 months but is improving with help of analgesics.

Raeder’s SyndromeRaeder’s syndrome is characterised by severe, unilateral facial pain and headache in the distribution of the ophthalmic division of the trigeminal nerve in combination with ipsilateral oculosympathetic palsy and usually preservation of facial sweating. This was first described by Raeder in 1918.

Subtypes1962 Boniak described 2 subtypes:

1. Group I had neuralgia, oculosympathetic paralysis and parasellar nerve involvement with lesions usually in the middle cranial fossa

2. Group II had no parasellar involvement but all the other features.

Some causative conditions in group II included migraine, cluster headache, hypertension, trauma, inflammatory disease, sinusitis, syphilitic osteitis, herpes zoster, otitis and pneumonitis.

In 1980 Grimson and Thompson described 3 major groups:

• Group I - includes patients with multiple parasellar cranial involvement or involvement of any or all divisions of the trigeminal nerve

• Group II - includes patients with cluster headache with an isolated oculosympathetic paresis

• Group III - includes patients with pain atypical for cluster headache, with involvement of the ophthalmic division of the trigeminal nerve

Groups II and III are associated with favourable prognosis. A further subtype has been described with a pericarotid syndrome rather than paratrigeminal and present with oculosympathetic paralysis, ipsilateral head pain and anhidrosis with otherwise intact facial sweating.

Aetiology and PathophysiologyThe pathophysiologic site of the painful oculosympathetic palsy involves the location at which oculosympathetic fibres exit the internal carotid artery to join the ophthalmic division of the trigeminal nerve. Various combinations of cranial deficiencies (nerves II-VI) also may be involved. Raeder syndrome localises lesions of the middle cranial fossa involving oculopupillary sympathetic fibers originating from the internal carotid artery plexus and travelling with the trigeminal and oculomotor nerves.

Sweating is preserved in Raeder paratrigeminal syndrome, in contrast to Horner’s syndrome, since some third-order sympathetic fibres are spared. These particular fibres travel with the external carotid artery and its branches and are involved in the production of facial sweating.

Raeder Syndrome has been associated with several conditions, including the following:

• Head trauma• Hypertension • Vasculitis• Migraine headaches• Parasellar mass lesions• Internal carotid artery dissection or aneurysm

Epidemiology• This is a rare syndrome with an unknown incidence. It is

less common than Horner’s Syndrome.• Patients are usually between the ages of 18-65• Men are predominantly affected.

Clinical PresentationPatients usually present with trigeminal neuralgic type pain mostly of the ophthalmic division and this can be preceeded by flushing of the ipsilateral cheek.

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Oculosympathetic involvement can resemble Horner’s with ptosis and miosis but unlike Horner’s ipsilateral facial sweating and trigeminal sensation are usually preserved.

The pain associated with Raeder’s syndrome is deep and boring and is localised in or around the eye. Intermittent, lancinating pain also may occur. Typically self-limited, the pain usually remits in 2-3 months.

The pain occasionally follows a recurrent pattern. It can be associated with conjunctival tearing, erythema, enophthalmos, and decreased intraocular pressure. The pain is less well defined than the pain of trigeminal neuralgia.

Differential DiagnosisThis is very broad and can include:

• Carotid body tumour• Cerebral vascular dissection• Cluster headache• Craniopharyngioma• Dissection syndromes• Fibromuscular dysplasia• Head Injury• Human immunodeficiency virus 1 (HIV-1)-associated

cerebrovascular complications• Lyme disease• Meningioma• Middle cranial fossa tumours• Migraine headache• Neurosarcoidosis• Neurosyphilis• Osteitis• Persistent idiopathic facial pain• Pituitary tumors• Sinusitis• Syphilis• Trauma• Trigeminal neuralgia• Tuberculous meningitis• Brainstem syndromes• Granulomatous angiitis of the central nervous system• Carotid disease and stroke

InvestigationsDiagnosis is usually made taking into account clinical presentation and examination.

Secondary causes need to be excluded and an MRI with fine cuts through the middle cranial fossa is paramount. MRI angiogram of intra and extracranial vessels is also advised. Formal CT angiogram is warranted if any abnormality is suspected on the MRI angiogram.

Full blood count and erythrocyte sedimentation and C reactive protein are important to exclude temporal arteritis and infective causes. Autoimmune screen is also advised.

TreatmentAvoid alcohol and any vasodilator.

When parasellar involvement is absent corticosteroids can be initiated usually with good success. Oral prednisone is usually adequate for several weeks but induction with methylprednisilone can be used when there is severe intractable pain.

As the condition can take 3-4 months to abate pain control can be achieved with the use of:

• Anticonvulsants: topiramate, gabapentin, pregabalin and carbamazepine

• Analgesics• NSAIDs: naproxen, ibuprofen, indomethacin and diclofenac

are preferred• Opiates can be used short term for mild pain.• Other agents with mixed outcomes include:

‒ Tricyclic antidepressants: amytriptylline, clomimpramine, desipramine

‒ muscle relaxants: baclofen, cyclobenzaprine

Palpebral MuscleInternal Carotid Artery

Medulla

(Sweating of face)

3rd Neuron

1st Neuron

Spinal Cord

2nd Neuron

Common Carotid Artery

Inferior Cervical

Ganglion

ExternalCarotid Artery

Ganglion

Superior Cervical

Ganglion

Dilator Muscle of PupilCiliary MuscleBlood Vessels of Eyeball

Ciliary

V n

B

A

C7

C8T1

T2

III n

NEURON SA Volume 9 No 3 Issue 31 2016 Page 12

EthicsEuthanasia and the psychiatrist

Dr gc VersterDistrict Psychiatrist

Khayelitsha and Eastern substructure, Cape Town Metro Dept Psychiatry, University of Stellenbosch

“I don’t fear death so much as I fear its prologues: loneliness, decrepitude, pain, debilitation, depression, senility.”

- Mary Roach

ssisted dying is currently being actively debated in South Africa. The widely publicised cases of Prof. Sean Davison and Advocate Robin Stransham-Ford have ignited fierce debates on whether physician assisted suicide (PAS) and voluntary active

euthanasia (VAE) should be allowed or not. Although the court seems to be moving for change, the policies of the Health Professions Council of South Africa remain in place and such practices may lead to severe sanctioning by the council. Nevertheless, the issue is on the table and the discussions need to continue.

Although psychiatric conditions may not yet receive much prominence in the debate on VAE/PAS, it is clearly a growing issue, especially in the Netherlands and Belgium.

The Dutch experienceIn 1991, Dr Boudewijn Chabot, a Dutch psychiatrist, assisted a 50-year old woman in dying. She had lost both her children in their early twenties and as a single parent she could not foresee a life without them. Her only wish was ‘to lie in between the graves of her two sons’ and she requested PAS.

Pharmacotherapy for depression as well as entry into a therapeutic community was suggested, but she refused. She had no terminal physical illness and after thorough assessment – he also obtained opinions from six other colleagues – she was found to be of sound mind and that her request was found to be well-considered. Dr Chabot subsequently went ahead and assisted her in dying.

The case was taken to court and caused significant debate. The main concern from the Court was that Dr Chabot’s patient was not physically, terminally ill. The Court also felt it was not possible to judge whether someone was ‘suffering unacceptably’ when the source of the suffering is mental rather than physical.1

Nevertheless, the laws in the Netherlands pertaining to VAE and PAS were subsequently changed and the Royal Dutch Medical Association published guidance which accepted that it could be acceptable in cases where there were serious psychiatric problems with no prospects of improvement.

Although the case was widely publicised, Dr Chabot experienced very little condemnation from the public and his colleagues. But as could be expected, there were some who found his actions unacceptable. Concerns were raised about the short time that had elapsed before the final conclusions

were drawn (less than two months) and one colleague raised the issue that it could not be a doctor’s job to kill people and also that the idea of a well-considered suicide is a myth.

in south AfricaAlthough it would be premature to predict future policy changes by the HPCSA, it may be prudent for psychiatrists in South Africa to consider a number of questions regarding their profession’s role in VAE/PAS.

How does one define mental anguish?Any practising psychiatrist would have experience with patients suffering mental anguish. To quantify this or compare it to the pain of a patient with metastatic cancer is probably a futile exercise, but the following description by a physician writing on depression and suicide, may give some insight:

“If one tries to get such a patient to titrate other pains against the pain of his depression one tends to end up with a description that would raise eyebrows even in a mediaeval torture chamber. Naturally, many of these patients commit suicide. They may not hope to get to heaven but they know they are leaving hell.”2

When is depression really treatment resistant?There are accepted treatment guidelines regarding duration of treatment, medication dosages and possible switches, but this does not necessarily always take into account other factors such as treatment adherence, ongoing stressors as well as availability of funding for said treatments. For example, a patient in the state sector or many of the less lenient medical aid funds clearly does not have the same options available as a patient able to afford all available options. Does this imply that the definition of treatment resistance is also funding-dependent? If a diagnosis of treatment resistance is required, does it mean that the option of PAS/VAE would be limited to the well-funded only?

These questions would need to be answered and it also emphasises the need for any decisions in this regard to be made by a panel of mental health professionals and not by a single psychiatrist only.

is the idea of a well-considered (or rational) suicide really a myth?A 1991 survey of almost 400 senior doctors in the UK found that 72% agreed with the possibility of rational suicide. The findings were independent of speciality and the survey did not investigate the presence of mental illness.3 In another study it was found that especially palliative care physicians were against PAS/VAE, but again no specific mention was made of psychiatric reasons for consideration.4

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From a bioethical perspective, it would be argued that such important decisions require a very high level of autonomy. Unfortunately, the presence of a chronic, treatment resistant mental illness (or any other illness, for that matter) is highly likely to influence truly autonomous decision-making.

should psychiatry at all be involved in the decision-making process?In a study that surveyed the matter of euthanasia in clinical psychiatry practice in Holland, it was found that the percentage of permission of euthanasia for psychiatric conditions was 2% compared to that of a 37% average amongst general medical conditions.5

One argument against psychiatry’s involvement in the process is that psychiatric practice is aimed at preventing suicide, not assisting in it. A counterargument may be that psychiatrists should be even more attuned to the pain their patients experience, and that if VAE/PAS is accepted, then psychiatry should certainly be part of the process.

Another study in 998 terminally ill patients found that 10,6% considered PAS/VAE an option, but that depression and hopelessness, rather than pain, are primary determining factors.

Added to this, 50% of these patients changed their minds over following months. Furthermore, depression is particularly difficult to diagnose in terminal patients because of ongoing pain and feelings of nihilism.6

Taking all of these factors into account, it does appear as if the role of the psychiatrist should be integral in the evaluation process – especially in order to rule out potentially treatable depression.

suggestionsIf the process of considering changes in the legal and statutory status of VAE/PAS is to go ahead, then the following aspects regarding the involvement of psychiatrists should be considered:

• Psychiatrists should be Involved in the competency assessment of patients requesting PAS/VAE

• Where appropriate, they should Identify and treat psychiatric illness

• Because of a special skill-set, psychiatrists could also be involved in a deeper role - evaluating psychodynamic issues that may be playing out as well as an understanding of other subconscious issues at play.

• Support to staff and family should also not be neglected and psychiatry should play a role.

Psychiatrists who may become involved in these assessments should be made aware of the potential conflicts that may arise

and dedicated support should be made available to address the following:• Role confusion is inevitable when psychiatrists take over a

role of assisting – rather than preventing – suicide• It may also appear as if the psychiatrist is a gatekeeper in

the process, due to involvement in the screening process. This may cause further distress because of the perceived pressures from patients, families, the community as well as colleagues.

• Lastly, what would the impact of repeated contact with PAS/VAE evaluation be on the personhood of a psychiatrist? Is there not a risk of losing perspective on the uniqueness of human life and the particular moral status thereof?

It may also be argued that it is essentially unfair for psychiatrists (and by implication, all doctors) to play such a central role in PAS/VAE. It may even be considered that since the technical aspects of PAS/VAE are not that difficult, health practitioners could be removed from the final act of ending a life.

It is thus clear that many questions still need to be answered and a number of safeguards be put in place in very clear protocols if the PAS/VAE were to be accepted in the South African context.

references1. Sheldon T. The Doctor Who Prescribed Suicide.

Independent. [Online] 1994, June 30 [access 2016, February 20]; Available: http://www.independent.co.uk/life-style/the-doctor-who-prescribed-suicide-was-the-dutch-psychiatrist-dr-boudewijn-chabot-right-to-help-a-1425973.html

2. Price JS. Chronic depressive illness. Brit Med J 1978; 1:1200-1201.

3. Ginn S, et al. Senior doctors’ opinions of rational suicide. J Med Ethics 2011; 37:723-726

4. Seale C. Legalisation of euthanasia or physician-assisted suicide: survey of doctors’ attitudes. Palliat Med 2009 Apr; 23(3):205-12

5. Groenewoud JH et al. Physician-Assisted death in Psychiatric Practice in the Netherlands. New Eng J Med 1997; 336: 1795–1801

6. Emanuel EJ, Fairclough DL, Emanuel LL. Attitudes and desires related to euthanasia and physician-assisted suicide among terminally ill patients and their caregivers. JAMA. 2000;284(19):2460.

NEURON SA Volume 9 No 3 Issue 31 2016 Page 14

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12thEPNS CONGRESSLyon France 2017

Lyon Cité Internationale

XXIII World Congress of Neurology

September 16-21 2017 Kyoto,Japan www.wcn-neurology.com

Expert Speakers Cutting-edge Program Networking Opportunities Latest Research

Abstract Submission Deadline: April 6 2017 Early Registration Deadline: June 14 2017

SCHEDULING STATUS: S5 PROPRIETARY NAME (AND DOSAGE FORM): Dormonoct® 2 mg Tablets. COMPOSITION: Each tablet contains 2,49 mg loprazolam mesylate, equivalent to 2 mg loprazolam. Reg. No.: Q/2.2/355. NAME AND BUSINESS ADDRESS OF THE APPLICANT: sanofi -aventis south africa (pty) ltd., Reg. No.: 1996/010381/07, 2 Bond Street, Grand Central Ext.1, Midrand. Telephone: (011) 256 3700. Facsimile: (011) 256 3707. www. sanofi -aventis.com.For full prescribing information refer to package insert approved by the medicines regulatory authority.SAZA.LOME.16.05.0459

References: 1. Clark BG, Jue SG, Dawson GW, Ward A. Loprazolam – A Preliminary Review of its Pharmacodynamic and Pharmacokinetic Properties and Therapeutic Effi cacy in Insomnia. Drugs. 1986;31(6):500-516. 2. Dormonoct® 2 mg Package Insert. 04.10.1983. 3. Salkind MR, Silverstone T. The clinical and psychometric evaluation of a new hypnotic drug, loprazolam, in general practice. Curr Med Res Opin. 1983;8(5):368-374. 4. McInnes GT, Bunting EA, Ings RMJ, Robinson J, Ankier SI. Pharmacokinetics and pharmacodynamics following single and repeated nightly administrations of loprazolam, a new benzodiazepine hypnotic. Br J Clin Pharmac. 1985;19:649-656. 5. Botter PA. A Comparative Double-blind Study of Loprazolam, 1 mg and 2 mg, Versus Placebo in Anxiety-induced Insomnia. Curr Med Res Opin. 1983;8(9):626-630.

The Definition:• An effective hypnotic 1

• ‘Intermediate’ half-life (6 - 8 hours) 1,2

• Unaltered REM sleep 1,3

• Rapid sleep onset and maintenance of sleep 1

• Refreshed morning awakening 1

• Helps reduce anxiety symptoms associated with insomnia 1,4,5

- Caution should be exercised in patients suffering from anxiety accompanied by an underlying depressive disorder

ety

Restored sleep

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904067_Dormonoct Journal Ad rF.indd 1 2016/06/01 3:01 PM