CN Omar TIA_Layout 1 30/03/2015 11:43 Page 1

TIA diagnosis z Case notes

Beware of a diagnosis of TIA in a

young patient
Sarim Kamal Omar MBBS, MRCPI, Mazen Rizeq FRCP

Cavernomas are abnormal vascular lesions consisting of many small lobules, like a bunch
of small berries. They may be asymptomatic or they may present with seizures, in around
60% of cases, or symptoms similar to a transient ischaemic attack (TIA) or stroke. Here, the
authors describe a case of a 30-year-old woman who presented with TIA-like symptoms
for which treatment was started. Imaging revealed a spinal cord cavernoma, after which
the course of management was changed and the patient referred to the neurosurgeons.

Presentation
A 30-year-old
lady, an ex-
smoker, with a
background
history of anxi-
ety and asthma,
presented to A&E with a sudden
onset of headache, neck pain,
right shoulder pain which radiated
to the right arm along with numb-
ness, and heaviness of the same
side. She denied any chest pain or
shortness of breath. The symptoms
virtually resolved in about 20 min- Figure 1. Cross-sectional view of the cervical lesion
utes. Interestingly, she had a simi- Figure 2. Sagittal view of the cervical lesion
lar but more severe episode on the MRI cervical spine: There is a focal lesion at the C2/3 level, which measures approximately 9.8
left side ten years earlier and was x 8.5mm. It is in the right side of the cord but on the axial 2D MERGE sequences appears to
investigated for a TIA but no have two loculi. The largest is indenting the lateral white matter columns and the smaller is
definite diagnosis was made. within grey matter crossing the midline towards the left side. Most of the lesion is of low T1
and T2 signal. Following gadolinium enhancement, there is some peripheral signal bright up
On admission she looked well
around the lesion but the enhancement is circumferential and on all sequences. There is also
and obser vations were normal.
high signal within the cord extending from the foramen magnum to the C5 level. This appears
CNS examination showed slightly to be oedema. There is only a small swelling of the cord at the level of the lesion. Dark signal
reduced power of 4-5 / 5 in the on all sequences is consistent with chronic haematoma, possibly with some reaction around it.
right upper limb, plantars were The features favour a diagnosis of a vascular lesion and a cavernoma is a distinct possibility.
downgoing and there were no
cerebellar signs. Bloods, ECG, admitting team. The original diag- a haemorrhage in a cavity within
chest X-ray and CT brain were nor- nosis of TIA was queried given her the right side of the cervical cord
mal. She was started on aspirin young age and the previous at C2/C3, lots of surrounding high
300mg once daily by the admitting episodes; hence the possibility of T2-weighted signal in the cord with
team and was referred to the either a demyelinating disease, a mild contrast enhancement
stroke team for further assessment. posterior circulation lesion, a pos- around the edges (see Figures 1
The next day she developed sible dissection or stenosis in the and 2).
further numbness on the same vertebral artery was entertained. A diagnosis of spinal cord cav-
side and became significantly Hence an urgent MRI to include ernoma was made and the acute
ataxic and was seen by the stroke the cer vical spine was arranged. symptoms were thought to be
team at the request of the Interestingly enough, this showed most probably due to a bleed.

www.progressnp.com Progress in Neurology and Psychiatry March/April 2015 17

CN Omar TIA_Layout 1 30/03/2015 11:43 Page 2
Case notes z TIA diagnosis
(Please note the patient received
aspirin 300mg on two occasions.)
In light of the MRI findings,
aspirin was stopped immediately
and the patient’s case was dis-
cussed with the neurosurgeon,
who suggested managing the
patient expectantly, while plans for
possible stereotactic radiosurgery
would be considered after the
MDT meeting. At the time of
preparing this case, we understand
that the patient has had a dynamic
magnetic resonance angiogram
(MRA) followed by a focused
spinal angiogram. The latter has
excluded any arteriovenous shunt-
ing in this area.
At a follow-up clinic the patient
remained well with no recurrence
of her symptoms. She has decided
to undergo the stereotactic radio-
surgery, and has regular follow-ups
with the neurosurgical team.
Discussion
A cavernous malformation, also
known as a cavernous haeman-
gioma or cavernoma, is an abnor-
mal vascular lesion consisting of
many small lobules, like a bunch
of small berries. These contain
blood products in different stages
of evolution. The ‘sinusoidal’ com-
partments are enclosed by abnor-
mally thin and quite fragile
endothelialised walls. Unlike an
arteriovenous malformation, there
is no large feeding artery and no
large draining vein in a caver-
noma. However, there frequently
is an associated venous angioma.
The prevalence of cavernous
malformations is between 0.1%
and 0.5%; they are about as com-
mon as brain arteriovenous mal-
formations, and far less common
than brain aneurysms.
Most cavernomas are found in
the supratentorial region of the
brain hemispheres, but up to 1 in
4 or 5 are found in the posterior
fossa / infratentorial, especially in
18 Progress in Neurology and Psychiatry March/April 2015
the pons region of the brainstem.
Much less commonly, a cavernoma
may be found in the spinal cord, as
was the case for our patient, but
this may be more likely to occur in
patients with a family histor y of
cavernous malformations.
Cavernomas may be asympto-
matic, or may present with seizures
(60%) or, as in our patient, with
TIA- / stroke-like symptoms with
progressive neurological impair-
ment or ‘deficits’ (50%). Some can
present with hydrocephalus or
raised intracranial pressure
depending on their size and loca-
tion. It is uncommon for caver-
nomas to cause sudden cata-
strophic or devastating neurologi-
cal injur y, but the progressive
brain or spinal cord injury associ-
ated with cavernomas may be
severely disabling as time goes on.
The risk of haemorrhage is
between 0.5 and 1% per year. The
re-haemorrhage rate varies in the
literature, but is between 4 and
10% per year.
Cavernomas are best detected
using MRI cerebral angiography.
The treatment is surgery. There
is no effective radiation treatment.
The one exception is a possible
cavernoma variant that occurs in
the venous sinuses (intrasinus cav-
ernoma) which has been reported
to be susceptible to radiation (eg
gamma knife or stereotactic radio-
surgery). Cavernomas can develop
in regions of brain that have previ-
ously been exposed to radiation.
In general, a cavernoma that is
enlarging radiologically and / or
symptomatic should be considered
for surgery. Even those located in
the brainstem or spinal cord or in
other highly ‘eloquent’ areas
(areas of the cortex which if
removed, could lead to a loss of lin-
guistic ability or sensory process-
ing or can even lead to a degree of
paralysis or complete paralysis at
times), should be considered for
removal if symptomatic and if rel-
atively safely accessible surgically.
In a recent study published by
Kharkar, et al. 14 patients were
included from 1989 to 2002.1 The
mean age at presentation was 42
years. Four lesions (29%) were
located in the cervical region and
10 lesions (71%) were present in
the thoracolumbar spinal cord.
Seventy-one per cent were man-
aged conservatively. Four patients
(29%) were treated surgically. The
mean duration of symptoms
before presentation was 33
months. The mean duration of
follow-up from the time of presen-
tation was 42 months. In this
series, patients harbouring a
symptomatic spinal cord cav-
ernous malformation did not
have significant permanent neu-
rological decline during the fol-
low-up period when treated with
the conser vative approach of
observation. This is a similar situ-
ation to the case described above
as, at the time of writing, our
patient was still under active fol-
low-up with no neurological dete-
rioration, yet surger y was still
planned and considered.
Three cases of surgically veri-
fied intramedullar y cavernous
angiomas (cavernoma) of the
spinal cord were reported in 1995,
by Gordon, et al.2 The most com-
mon presenting symptom was
pain, which in all cases preceded
weakness. In two of the cases the
typical progression of sudden
paroxysmal worsening of symp-
toms accompanied by pain was
noted. This was thought to be
related to bleeding in the lesion.
In the case we have described, we
believe that the two doses of
aspirin given to the patient led to a
bleed in the cavernoma itself and
this was clearly reflected in her new
neurology findings the day after
and the spontaneous resolution of
symptoms within a few days.
www.progressnp.com
CN Omar TIA_Layout 1 30/03/2015 11:43 Page 3

Prescribing Information
Matoride XL 18mg, 36mg & 54mg Case notes z TIA diagnosis
Prolonged Release Tablets
(methylphenidate hydrochloride)
Please consult the Summary of
Product Characteristics before prescribing
Indication: As part of a comprehensive treatment programme for Attention
Deficit Hyperactivity Disorder (ADHD) in children aged 6 years of age and over A case was described in the in the cavernoma itself. 6
when remedial measures alone prove insufficient. Must be under the supervision
of a specialist in childhood behavioural disorders. Dosage & Administration:
British Journal of Neurosurgery where Interestingly, this case was also
Tablet for oral administration, taken once daily in the morning with or without a 31-year-old male presented with a diagnosed as a TIA and aspirin was
food. Must be swallowed whole with the aid of liquids, and must not be chewed,
divided, or crushed. Pre-treatment screening: baseline evaluation of patient’s one year history of left leg weak- given at the time of admission, in a
cardiovascular status (including blood pressure and heart rate), concomitant
medication, past and present co-morbid medical and psychiatric disorders ness and right leg sensor y similar way to our patient.
or symptoms, family history of sudden cardiac/unexplained death and pre-
treatment height and weight on a growth chart. Dose titration: Start at the lowest
changes.3 Myelography revealed a In summary, we have described
possible dose. Dose adjustment in 18mg increments at approximately weekly probable intramedullary lesion at a patient who presented with TIA-
intervals. Patients new to methylphenidate: Consider lower doses of short-
acting methylphenidate formulations. Patients Currently Using Methylphenidate: T4 and CT confirmed its like symptoms and was started
Recommended dose conversion from 5, 10, 15 mg methylphenidate three times
a day is 18, 36, 54mg Matoride XL daily, respectively. Discontinue if improvement
intramedullary nature. At opera- treatment in-line with recom-
not observed after dosage adjustment over one month. Contraindications: tion, an almost black, firm mass mended guidelines, but imaging
Hypersensitivity to the active substance or to any of the excipients; glaucoma;
phaeochromocytoma; during, or within 14 days of discontinuing treatment was found projecting from the dor- revealed a spinal cord cavernoma,
with MAOIs, hyperthyroidism or thyrotoxicosis; diagnosis/ history of severe
depression, anorexia nervosa/ anorexic disorders, suicidal tendencies, psychotic sal aspect of the cord and was after which the course of manage-
symptoms, severe mood disorders, mania, schizophrenia, psychopathic/ totally excised. Histological exam- ment changed and patient was also
borderline personality disorder; diagnosis / history of severe and episodic (Type
I) Bipolar (affective) Disorder (if not well-controlled); pre-existing cardiovascular ination revealed it to be a referred to the neurosurgeons for
disorders; pre- existing cerebrovascular disorders, cerebral aneurysm, vascular
abnormalities including vasculitis or stroke. Precautions: Long-term use (more cavernous angioma. further management, in addition
than 12 months): ongoing monitoring (at each dose adjustment and then at
least every 6 months) for: cardiovascular status (blood pressure and pulse);
In a recent paper published by to being followed up by us.
neurological signs and symptoms (for those with cerebrovascular disorders Papageorgiou, et al. from Greece, a
and additional risk factors); psychiatric/neurological conditions (including
suicidal ideation, possible precipitation of a mixed/manic episode in patients 67-year-old woman presented with Dr Omar is a Consultant Physician
with comorbid bipolar disorder, epilepsy as may lower convulsive threshold);
growth (height, weight and appetite). De- challenge recommended at least once walking difficulties with acute in General and Elderly Care Medicine
yearly. Caution in underlying medical conditions compromised by increases in onset of pure right leg monopare- at Darent Valley Hospital, Dartford,
blood pressure / heart rate. Not recommended in known structural cardiac
abnormalities, cardiomyopathy, serious heart rhythm abnormalities, or other sis with moderate spasticity.4 The Kent and Dr Rizeq is a Consultant
serious cardiac problems due to sympathomimetic effects. Potential for abuse,
misuse or diversion in drug or alcohol dependency. Not to be taken by patients initial diagnosis was lacunar stroke, Physician in General Medicine,
with rare hereditary problems of galactose intolerance, the Lapp lactase
deficiency or glucose-galactose malabsorption. Should not be used in patients
but the brain MRI revealed a right Elderly Care Medicine and Stroke at
with pre-existing severe GI narrowing (pathologic or iatrogenic) or in dysphagia temporal cavernoma, not associ- East Kent Hospitals University NHS
or significant swallowing difficulties. The tablet shell may be noticed in stools.
No experience in renal or hepatic insufficiency. If leukopenia, thrombocytopenia, ated with her monoparesis. The Foundation Trust, Kent.
anaemia or other alterations, including those indicative of serious renal or hepatic
disorders present, discontinuation should be considered. Pregnancy/ Lactation: consequent spinal MRI revealed
Not recommended in pregnancy. Discontinue in breast-feeding. Driving: Caution
is advised. Side effects: (See SPC in relation to other side effects). Very Common:
an intramedullary lesion at the T1 Declaration of interests
Insomnia, nervousness, headache. Common: Nasopharyngitis, upper respiratory level, consistent with a cavernoma. No conflicts of interest were
tract infection, sinusitis, anorexia, decreased appetite, moderately reduced
weight and height gain during prolonged use in children, affect lability, aggression, In a French study of a group of declared.
agitation, anxiety, depression, irritability, abnormal behaviour, mood swings,
tics, initial insomnia, depressed mood, decreased libido, tension, bruxism,
spinal cord cavernomas, published
panic attack, dizziness, dyskinesia, psychomotor hyperactivity, somnolence, by Labauge, et al. initial symptoms Acknowledgments
paraesthesia, tension headache, accommodation disorder, vertigo, arrhythmia,
tachycardia, palpitations, hypertension, cough, oropharyngeal pain, upper were progressive (60.37%) or of The authors would like to thank Dr
abdominal pain, diarrhoea, nausea, abdominal discomfort, vomiting, dry mouth,
dyspepsia, alopecia, pruritis, rash, urticaria, arthralgia, muscle tightness / spasms, acute myelopathy (37.73%). 5 P. McMillan for his expert report-
erectile dysfunction, pyrexia, fatigue, irritability, feeling jittery, asthenia, thirst, Clinical symptoms were related to ing of the MRI scans and to Dr R.
changes in blood pressure and heart rate (usually an increase), weight decreased,
alanine aminotransferase level increased. Other serious adverse events reported spinal cord compression (50.94%) Nahas for his help in formulating
include: Hypersensitivity reactions (such as angioneurotic oedema, anaphylactic
reactions, auricular swelling, bullous conditions), exfoliative conditions, and haemorrhage (41.50%). the MRI scans into this paper.
psychotic disorders, auditory, visual and tactile hallucination, suicidal ideation,
worsening of pre-existing tics of Tourette's syndrome, logorrhoea, sleep disorder,
Using McCormick classification,
sedation, tremor, lethargy, blurred vision, dyspnoea, hepatic enzyme elevations, 22 patients were autonomous References
angioneurotic oedema, myalgia, haematuria, pollakiuria, mania, disorientation, 1. Kharkar S, Shuck J, Conway J. The natural
confusional state, visual accommodation difficulties / impairment, diplopia, (grades 1-2), 12 handicapped history of conservatively managed sympto-
cardiovascular disorders (including angina pectoris, cardiac arrest and myocardial
infarction), blood disorders (including anaemia, leucopenia, thrombocytopenia, (grade 3), and 3 bedridden matic intramedullary spinal cord cavernomas.
thrombocytopenic purpura), suicidal attempt, convulsions, choreoathetoid Neurosurgery 2007;60(5):865–72.
movements, neuroleptic malignant syndrome, abnormal liver function including
(grade 4) at the end of the follow- 2. Gordon CR, Crockard HA, Symon L. Surgical
hepatic coma, delusions, cerebrovascular disorders (including vasculitis, cerebral up. This study defined clinical and management of spinal cord cavernoma. Br J
haemorrhages, cerebrovascular accidents, cerebral arteritis, cerebral occlusion). Neurosurg 1995;9(4):459–64.
Pack sizes/cost (30 tabs) (excl. VAT): 18mg = £24.95, 36mg = £33.96; MR patterns of spinal cavernomas. 3. Sabin HI, Daniel S, Wild AM, et al. Cavernous
54mg = £60.48
Legal Category: POM
Surgery lastingly improved more angioma of the thoracic spinal cord with intra-
and extramedullary components. Br J
MA Holder: Sandoz Ltd, Frimley Business Park, Frimley, Camberley, Surrey, than half of the patients. Neurosurg 1989;3(1):123–6.
GU16 7SR. United Kingdom. Further information is available on request.
MA No: PL 04416/1348-1349 In a recent paper from Finland, 4. Papageorgiou SG, Kontaxis T, Samara C, et
Last revision of text: February 2015 UK/MKT/RET/14-0024a(1) al. Spinal cavernoma: an unusual cause of
published by Kivelev, et al. from the acute monoparesis. Neurologist 2009;
Department of Neurosurger y at 15(5):291–2.
Adverse events should be reported. Reporting forms and information 5. Labauge P, Bouly S, Parker F. Outcome in 53
can be found at www.mhra.gov.uk/ yellowcard Helsinki University Central patients with spinal cord cavernomas.
Adverse events should also be reported to Sandoz Ltd, Hospital, a unique case of an Surg Neurol 2008;70(2):176–81.
01276 698020 or uk.drugsafety@sandoz.com. 6. Kivelev J, Ramsey CN, Dashti R. Cervical
intradural extramedullary spinal
intradural extramedullary cavernoma present-
cavernoma had a successful surgi- ing with isolated intramedullary hemorrhage.
1. Elisabeth Schapperer et al. Bioequivalence of Sandoz methylphenidate osmotic cal removal following a bleed J Neurosurg Spine 2008;8(1):88–91.
controlled release tablet with Concerta® (Janssen-Cilag) Pharma Res Per, 2(5),
online Jan 2015
2. Matoride XL SPC

UK/MKT/RET/15-0001, January 2015

20 Progress in Neurology and Psychiatry March/April 2015