A 31-year-old woman with vertigo

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Problem 45 A 31-year-old woman with vertigo

Timothy Kleinig

A 31-year-old teacher presents to your general practice with a 2-day history of a sensation of the room spinning and unsteadiness while walking. The sensation is continuous and partially relieved by lying down. These symptoms have prevented her from going to work. She feels her hearing is normal and she has no tinnitus. Her only medication is the oral contraceptive pill.

Q.1

What are the principles of assessing a patient with vertigo?

Q.2

How do you differentiate central from peripheral causes of vertigo? What are some causes of each?

On examination she appears fit and well. She is afebrile and her cardiovascular examination is normal. The ear canals and tympanic membranes appear normal. There is no evidence of hearing impairment on clinical testing. Visual acuity testing reveals 6/6 vision on the right but 6/9 on the left. The pupils are symmetrical, but you think there is an afferent pupillary defect on the left. On fundoscopy the left optic disc appears pale compared to the right.

On testing the external ocular movements there is nystagmus on looking to the right and on vertical gaze. The nystagmus is independent of head position and is not fatigable. The remainder of the cranial nerve examination is intact. Limb tone, power and reflexes are normal. However, on finger-nose testing there is an intention tremor of the right arm with mildly reduced coordination.

She recalls having problems taking a photograph using her left eye while on holiday 3 months previously. This had resolved spontaneously over 1 week and she did not pursue the matter.

There is nothing else in the history and from the examination that helped elucidate the cause of the patient’s current problem.

Q.3

How do you interpret this woman’s neurological signs?

Given the probable site of the problem, the patient is referred to a neurologist. Her vertigo had improved but has not resolved completely. Various tests were performed. Figure 45.1A, B was taken from one of the tests.

Figure 45.1

Q.4

Name the investigation and describe the abnormalities.

Q.5

What is the likely diagnosis and what other tests could be done to further support the diagnosis?

The cerebrospinal fluid demonstrated a mild elevation of mononuclear cells, normal protein and glucose and an elevated IgG:albumin ratio. Oligoclonal bands, unmatched in a serum specimen, were detected.

After the diagnosis is made, the patient returns to see you. Her symptoms have largely resolved. She asks what her prognosis is and if any treatment is available.

Q.6

What is your reply?

Answers

A.1 Vertigo is a sensation of movement, typically spinning or rotating, either of the environment or the patient in relation to the environment. This spinning sensation distinguishes vertigo from pre-syncopal ‘dizziness’ which occurs in cardiac arrhythmias, postural hypotension, anaemia or hypoglycaemia. A history of loss of consciousness suggests syncope or epilepsy.

Patients with vertigo present in three main ways: spontaneous onset of persistent vertigo, recurrent vertigo associated with changes in posture, and recurrent episodes of spontaneous vertigo. The major task in the assessment of vertigo is to decide whether the cause is peripheral (related to the vestibular apparatus) or central (vestibular nuclei and connections). Central causes of vertigo are generally more sinister, and a thorough neurological examination is required in patients presenting with new-onset vertigo.

On examination attention should be paid to a full neurological examination looking for focal signs suggesting a central cause. In particular:

• Examine the eyes looking for nystagmus.

• Examine the external ear, including the tympanic membrane and hearing.

You will also need to perform a careful general physical examination.

A.2 Signs suggesting central nervous system involvement include:

• Vertical nystagmus.

• Dysarthria.

• Facial sensory loss.

• Upper motor neurone signs and limb or gait ataxia.

• Internuclear ophthalmoplegia (lateral gaze induces horizontal nystagmus in the abducting eye and incomplete or slowed adduction of the contralateral eye) – due to a lesion of the medial longitudinal fasciculus, which connects the abducens nucleus (CN VI) to the contralateral oculomotor nucleus (CN III).

Examples of central lesions causing vertigo include:

• Vertebrobasilar ischaemia.

• Demyelination (e.g. multiple sclerosis).

• Cerebellar disease.

• Temporal lobe epilepsy (rare).

• Cerebellopontine angle tumour (more commonly associated with deafness, ataxia and other neurological signs such as facial sensory loss).

Examples of peripheral causes of vertigo include:

• Benign paroxysmal positional vertigo.

• Vestibular neuronitis.

• Ménière’s disease.

• Middle ear disease.

A.3 This woman has multiple signs of CNS pathology supporting a central cause of vertigo. The vertical nystagmus suggests brainstem dysfunction, the pale left optic disc suggests optic nerve disease, and intention tremor of the right arm suggests a disorder of the right cerebellar hemisphere or its connections.

She has no tinnitus, deafness or nausea and does not find that head turning precipitates vertigo. She is otherwise well. This would be unusual for any of the common peripheral causes of vertigo.

A.4 Magnetic resonance imaging (MRI) of the brain. Both FLAIR (fluid attenuation inversion recovery) and post-gadolinium T1-weighted sequences are shown. T1 MRI scans are good at showing anatomy (i.e. grey matter is greyer than white matter). FLAIR imaging is very sensitive at demonstrating pathology. Gadolinium enhancement demonstrates areas of acute blood–brain barrier disruption.

There are multiple hyperintense periventricular FLAIR signal foci, as well as callosal hyperintensities and several juxtacortical lesions. Several lesions were also seen in the posterior fossa and spinal cord (not shown). Some, but not all lesions demonstrated gadolinium enhancement (see Figure 45.1B), suggesting that lesions are of differing stages. This picture is almost pathognomonic for multiple sclerosis (MS), particularly with this clinical presentation.

A.5 This woman has a number of features in her illness that point to central nervous system disease:

• Left optic atrophy (previous optic neuritis).

• Vertigo and nystagmus (brainstem dysfunction).

• Poor control of right arm movement (cerebellar disturbance).

• Multiple white plaques on MRI.

• A pleocytosis and oligoclonal bands in cerebrospinal fluid.

The presence of multiple symptoms, occurring over time, related to different sites within the central nervous system, and the finding of multiple demyelinating lesions within the central nervous system make multiple sclerosis (MS) the most likely diagnosis.

Vertigo is an unusual presenting symptom in MS, although one-third of patients will experience this symptom during the course of the illness.

Cerebrospinal fluid (CSF) obtained at lumbar puncture can be helpful. The most characteristic finding in MS is an increase in the CSF IgG:albumin ratio (suggestive of intrathecal immunoglobulin synthesis), which fractionates into oligoclonal bands on electrophoresis, seen in around 90% of patients. Evoked visual response testing would not be relevant in this case because the patient has optic atrophy. Therefore we know already that this is a site of disease.

A.6 Multiple sclerosis commonly presents in a relapsing and remitting pattern, with complete or near complete recovery after each relapse. Approximately half will eventually develop progressive disease. A smaller proportion are left with permanent deficits after each relapse. In a further group, predominantly aged over 40, the disease is slowly progressive from the outset with spinal cord dysfunction being the major feature. Currently, the average survival is at least 30 years from the diagnosis, which represents an average shortening of life expectancy of around 5–10 years.

There is no curative treatment for the disease. Acute episodes can be treated with high-dose intravenous methylprednisolone. This hastens recovery of function but does not alter the degree of recovery from acute relapses or the longer-term prognosis.

In established relapsing-remitting MS, immunomodulation with interferon beta has been shown to reduce the rate of clinical relapse by 30%. Glatiramer acetate produces an equivalent effect. Natalizumab, which specifically interferes with leucocyte transmigration into the central nervous system, is a new and potent therapy for multiple sclerosis; however, there is an associated increased risk of progressive multifocal leucoencephalopathy, caused by the JC virus. The long-term effect of these agents on secondary disease progression remains uncertain. Mitoxantrone and cyclophosphamide are both effective in the setting of aggressive relapsing disease uncontrolled by first-line therapies. Numerous other injectible and oral agents are in development.

All current preventative therapies are administered by self-injection or intravenous infusion. There are no proven therapies for primary progressive multiple sclerosis. Supportive treatments for pain, spasticity (e.g. baclofen and physiotherapy), fatigue, depression constipation and urinary dysfunction are important.

Low vitamin D levels have a strong epidemiological association with both the risk of MS development and relapse. Although still controversial, many doctors recommend maintaining vitamin D serum levels above 100 nmol/L. Psychological support is vital. Patients should be offered counselling, and involvement with national MS societies and support groups.

Revision Points

Multiple Sclerosis

Definition

A demyelinating disease of the central nervous system ‘disseminated in time and space’.

Aetiology

The aetiology is uncertain. Genetic and environmental factors (e.g. sun exposure, infectious mononucleosis) both contribute. As the disease progress (‘secondary’ or ‘primary’ progression) it assumes a neurodegenerative pattern; immunotherapy at this stage is less effective or ineffective.

Presentation

MS often begins in early adult life and is more common in women. Symptoms and signs correspond to involvement of cerebral hemispheres, brainstem, cerebellum or spinal cord. Associated fatigue and depression are common.

Diagnosis

Dissemination in time and space needs to be demonstrated. If the symptoms and signs can be accounted for by a single lesion then the diagnosis cannot be made. The presence of oligoclonal bands in CSF can help establish the diagnosis in primary progressive disease. MRI scanning can reveal additional lesions that enable the diagnosis to be made.

The differential diagnosis includes:

• Neuromyelitis optica, a severe demyelinating illness characterised by predominant spinal cord and optic nerve involvement, and association with antibodies to aquaporin-4, a brain water channel. Acute disseminated encephalomyelitis (ADEM), a monophasic demyelinating illness, commonly post-infective.

• Vasculitis affecting the central nervous system.

• Multiple ischaemic events (usually easily distinguishable on neuroimaging).

• Inherited or acquired neurodegenerative disorders (if primary progressive presentation).

Optic Neuritis

• Presents as unilateral partial or complete central visual loss associated with pain on eye movement.

• In the acute phase the optic disc will appear normal. Subsequently optic atrophy will occur as evidenced by disc pallor, as in this case.

• It is reversible in two-thirds.

• However, in women, 75% of those with optic neuritis will go on to develop MS.

Vertigo

Clinical symptoms to assist in deciding whether vertigo is of central or peripheral origin are shown in Table 45.1.

Table 45.1 Clinical symptoms of vertigo

	Peripheral	Central
Characteristics	Severe, episodic	Mild to severe, semicontinuous
Precipitants	Head movement, typically turning, lying down in bed	May be exacerbated by movement
Exacerbating factors	Head movement	Head movement
Interval after movement	Few seconds	Immediate
Duration	Seconds to continuous	Continuous
Nystagmus	Mixed horizontal/torsional, suppressed by fixation	Horizontal, vertical, gaze provoked, not suppressed by fixation
Associated features	Deafness, tinnitus	Brainstem signs, e.g. facial numbness, diplopia, ataxia, weakness
Causes	Ménière’s disease, vestibular neuronitis, benign paroxysmal positional vertigo, middle ear disease	Ischaemia, demyelination

Issues to Consider

• What other conditions may cause disc pallor and optic atrophy?

• Sudden unilateral loss or diminution of vision has a number of causes – what are they?

Further Information

Compston A., Coles A. Multiple sclerosis. Lancet. 2008;372(9648):1502-1517.

www www.msfacts.org. Multiple sclerosis website with information for patients and health professionals. Many links

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