  |
|
|
|
|
|||
| Journal of the New Zealand Medical Association,
27-October-2006, Vol 119 No 1244
|
|||
|
Coma in an alcoholic: Marchiafava-Bignami
disease
Lenneke Haas, David Tjan, Jos Van Die, Anton Vos, Arthur van
Zanten
Coma can be a challenging diagnosis for the critical care
doctor, especially in alcoholic patients.
We report a case of a 55-year-old male patient in whom the
diagnosis of the coma was initially unclear and only discovered with magnetic
resonance imaging (MRI).
Case reportA 55-year-old man with chronic alcohol abuse was found at
home with altered consciousness and dysarthria.
Initially he was considered to be “just drunk
again”. However, the emergency medical service was eventually called in
after 24 hours because the patient did not wake up.
His medical history revealed a non-insulin dependent
diabetes mellitus and hypertension. Alcohol abuse was known for 12 years. He
used to drink several litres of beer a day. He was on the following medications:
losartan 100 mg once daily (OD) and glimepiride 2 mg OD.
On admission, neurological examination showed a Glasgow Coma
Scale (GCS) of
E1M1V1,
pupil reactions were symmetrical. Slight diverging strabismus was noticed. The
oculo-cephalic reflex was normal. There was no lateralisation or pathological
reflex present and no neck stiffness was found. Vital signs were as follows:
temperature 37.4ºC, blood pressure 120/80 mmHg, pulse 100 beats per minute,
and oxygen saturation 97% on room air.
Further physical examination was unremarkable.
Laboratory results showed a Hb of 7.4 mmol/L (8.5–11.0
mmol/L), MCV 108 f/L (80–100 f/L), leucocytes 9.7/nL (4–11/nL),
platelets 41/nL (150–400/nL), γ-glutamyltransferase 562 U/L
(0–50 U/L), ASAT 113 U/L (0–45 U/L), ALAT 68 U/L (0–45 U/L),
LD 942 U/L (0–450 U/L), ammonia 40 μmol/L (0–35 μmol/L),
Ca 1.74 mmol/L (2.20–2.65 mmol/L), Mg 0.64 mmol/L (0.7–1.2 mmol/L),
glucose 11.2 mmol/L (4–10 mmol/L). Serum thiamine concentration was 43
mmol/L (70–185 mmol/L) and folic acid level 15.2 nmol/l (5–55
nmol/L). The ethanol level was <0.1 promille. Toxicological screening proved
to be negative.
A lumbar puncture yielded clear colourless cerebrospinal
fluid that contained no red cells and six leucocytes per cubic millimetre
(3–15/mm3). Glucose level was 6.0 mmol/L
and total protein level 0.55 g/L (0.29–0.67 g/L). A stain smear showed no
micro-organisms and cultures did not show any growth.
Computer tomography (CT) of the brain, which was performed
immediately on the emergency department, showed no significant abnormalities.
The patient was intubated, ventilated, and transferred to the ICU.
Electroencephalogram (EEG) revealed slow background activity
with minimal irregularities and abundance of theta-activity occipito-temporal
and no seizure activity suggesting a metabolic cause of coma.
MRI of the brain showed a high signal lesion in the corpus
callosum and internal capsule in the T2-weighted sagittal (Figure 1) and axial
view (Figure 2), as a sign of demyelinisation and oedema.
Figure 1. T2-weighted sagittal image in
Marchiafava-Bignami disease demonstrating a small, well-defined, and hyperdense
lesion in the genu of the corpus callosum (arrowed)
 Figure 2. T2-weighted axial image in
Marchiafava-Bignami disease showing a high signal lesion in the corpus callosum
and internal capsule (arrowed)
 Intravenous thiamine (100mg OD) was started and in 2 days
the neurological condition showed gradual neurological improvement
(E4M4V1).
After 72 hours, patient was successfully extubated and discharged from the ICU.
DiscussionExtrapontine myelinolysis in chronic alcoholism are typical
findings of Marchiafava-Bignami disease (MBD). It is characterised by
demyelinisation or necrosis of the corpus callosum and adjacent subcortical
white matter. Necrosis of the corpus callosum is pathognomic for MBD.
MBD is a rare, severe, and usually fatal neurological
disorder associated with chronic alcoholism. It is first described by Carducci
in 1898 in Italian red wine drinkers and by Marchiafava and Bignami in
1903.1,2 It occurs predominantly in
malnourished alcoholics and is reported more often in male than female
drinkers.3,4 About 250 cases have been reported
in the medical literature, but it is likely that its incidence is higher, since
this diagnosis might easily be missed.
The underlying mechanism
of the disease is still not understood. It is probably caused by the combination
of alcohol abuse and malnutrition, leading to metabolic, toxic and vascular
disturbances.3
Brion observed that the disease occurred in patients who
consumed at least 2 litres of red wine for more than 20
years.5 Cases of MBD in non-alcoholic, but
malnourished patients have also been reported but are extremely rare, thus
suggesting a causative relation with alcohol
toxicity.6
Patients with severe
alcoholism who have this syndrome frequently have other problems such as
alcoholic intoxication and hallucinosis, Wernicke encephalopathy, alcoholic
liver disease, and sometimes subdural haemorrhage. Therefore, the diagnosis is
often unclear.
Until recently, the definite diagnosis was confirmed at
autopsy. However, in the era of modern imaging technology, diagnosis could be
based on clinical profiles, history of alcoholism, and specific localisations of
pathological lesions in the corpus callosum demonstrated by CT and
MRI.7
Findings on CT scan may confirm the diagnosis. However, if
callosal damage is mild or the lesion is small, it may not be obvious and easily
missed on CT, as in our patient. MRI is currently the most sensitive diagnostic
tool. It also has the advantage of sagittal imaging. Lesions appear as hypodense
areas in portions of the corpus callosum on CT and as discrete or confluent
areas of decreased T1 signal and increased T2 signal on MRI.
In a review of acute and chronic cases, Heinrich observed
that the worst case had the most significant MRI lesions suggesting a prognostic
role for MRI. CT and MRI lesions seemed to regress in patients who
improved.
There are no characteristic clinical presentations of MBD.
However, involvement of the corpus callosum may lead to various clinical
symptoms, such as: altered mentation, depression, mania, paranoia, or dementia.
It may progress into seizures, hemiparesis, aphasia, ataxia, tremor, dysarthria,
or dyskinesia and spasticity.
The course of the disease may be acute, subacute or chronic
and may lead to death within weeks to months.8
Death usually results from cardiorespiratory failure or from the complications
of alcohol abuse. Patients typically have severe neuropsychological deficits
before they die. Some patients survive for many years in a demented condition or
occasionally even show some
recovery.9 An
interhemispheric disconnection syndrome has been reported in
survivors.10
Because the aetiology of the disease is uncertain, a
specific therapy is not available. Cessation of alcohol intake is mandatory and
early supplementation of thiamine and folic acid might favourably improve the
outcome. Seizures and coma are treated symptomatically.
Patients who survive should
receive rehabilitation and, if appropriate, alcohol and nutritional counselling.
A favourable response has been reported after the use of corticosteroids in some
cases.
ConclusionWe presented a case of MBD in a comatose alcoholic.
Neurological examination and radiological imaging did not
reveal an initial diagnosis, nor did laboratory and toxicological screening.
Only MRI revealed the high signal lesion in the corpus callosum and internal
capsule, which are typical for MBD.
Our patient improved after thiamine administration and
supportive care.
Although rare, this case suggests that MBD should be
considered in patients with chronic alcoholism and unexplained neurological
deterioration, and MRI might be warranted in the absence of other causes for
coma. The diagnosis might otherwise easily be missed if not considered.
In patients with chronic alcoholism and mental confusion,
this uncommon diagnosis should be considered.
Author information: Lenneke EM Haas, Senior
Registrar, Department of Intensive Care; David HT Tjan,
Anesthesiologist-Intensivist, Department of Intensive Care; Jos Van Die,
Radiologist, Department of Radiology; Anton Vos, Neurologist, Department of
Neurology; Arthur RH van Zanten, Internist-Intensivist, Department of Intensive
Care; Gelderse Vallei Hospital, Ede, The Netherlands
Correspondence: D.H.T. Tjan,
Anesthesiologist-Intensivist, Department of Intensive Care, Gelderse Vallei
Hospital, PO Box 9025, 6710 HN Ede, The Netherlands. Fax: +31 318 434116;
email: tjand@zgv.nl
References:
|
|
|
| | Current
issue | Search journal |
Archived issues | Classifieds
| Hotline (free ads) Subscribe | Contribute | Advertise | Contact Us | Copyright | Other Journals |
|