Mesial temporal sclerosis

Dr Ayush Goel and Dr Frank Gaillard et al.

Mesial temporals sclerosis (MTS) also commonly referred to as hippocampal sclerosis, is the most common association with intractable temporal lobe epilepsy (TLE) 2-3,5. It is seen in up to 65% of autopsy studies, although significantly less on imaging.

Clinical presentation

Most patients present with complex partial temporal lobe epilepsy.

Febrile seizures

The relationship, if any, of mesial temporal sclerosis with febrile seizures is controversial, made all the more difficult due to the relative insensitivity of imaging and the difficulty in establishing whether a particular seizure was truly febrile. Up to a third of patients with established refractory temporal lobe epilepsy have a history of seizures in childhood at the time of fever 3. Follow up of children with febrile seizures does not demonstrate significant increased incidence of temporal lobe epilepsy 3.

Pathology

The hippocampal formation is not uniformly affected, with the dentate gyrus and the CA1, CA4 and to a lesser degree CA3 sections of the hippocampus being primarily involved 4. Histologically there is neuronal cell loss, gliosis and sclerosis.

Aetiology

Controversy exists as to the causative mechanism: is mesial temporal sclerosis a result of temporal lobe epilepsy or visa versa 5. In children with newly diagnosed epilepsy, only approximately 1% have evidence of MTS on imaging 3. Furthermore, in adults 3-10% of cases of mesial temporal sclerosis demonstrate bilateral changes 5 even though symptoms may be unilateral.

Radiographic features

MRI

MRI is the modality of choice to evaluate the hippocampus, however dedicated TLE protocol needs to be performed if good sensitivity and specificity is to be achieved 5. Thin section angled coronal sequences at right angles to the longitudinal axis of the hippocampus are required, to minimize volume averaging.

Coronal volume and coronal high resolution T2WI/FLAIR  are best to diagnose MTS.

Findings include 4:

reduced hippocampal volume: hippocampal atrophy

increased T2 signal

abnormal morphology: loss of internal architecture (interdigitations of hippocampus)

Although comparing left to right side is easiest, it must be remembered that up to 10% of cases are bilateral, and thus if symmetry is the only feature being evaluated, many cases may be misinterpreted as normal.

Often mentioned, but probably one of the least specific findings, is enlargement of the temporal horn of the lateral ventricle 5. If anything, care must be taken to not allow an enlarged horn to trick you into thinking the hippocampus is reduced in size.

When severe and long standing, additional associated findings include  4:

atrophy of the ipsilateral fornix and mamillary body

increased signal and or atrophy of the anterior thalamic nucleus

atrophy of the cingulate gyrus

increased signal and/or reduction in volume of the amygdala

reduction in volume of the subiculum

dilatation of temporal horn and temporal lobe atrophy

collateral white matter and entorhinal cortex atrophy

thalamic and caudate atrophy

ipsilateral cerebral hypertrophy

contralateral cerebellar hemiatrophy

loss of grey-white matter interface in the anterior temporal lobe 5

reduced white matter volume in the parahippocampal gyrus 5

Additional 3D volumetric studies can be performed, and although time consuming to post-process may be more sensitive to subtle hippocampal volume loss. Gadolinium is not required 5.

T2 relaxometry may also be useful in detecting cases of hippocampal sclerosis 5.

Diffusion MRI

As a result of neuronal loss, the extra cellular space is enlarged and thus diffusion of water molecules is greater on the affected side, resulting in increased values on the affected side (higher signal on ADC).

Conversely, due to neuronal dysfunction and swelling, diffusion is restricted following a seizure, and thus vales are lower 5.

MR spectroscopy

MR spectroscopy findings typically represent neuronal dysfunction 5:

decreased NAA and decreased NAA/Cho and NAA/Cr ratios

decreased MI in ipsilateral temporal lobe

increased lipid  and lactate soon after as seizure

MR perfusion

MR perfusion demonstrates similar changes to SPECT (see below) with blood perfusion depending on when the scan is obtained.

During the peri-ictal phases, perfusion is increased, not only in the mesial temporal lobe but often in large parts of temporal lobe and hemisphere.  In interictal periods, in contrast, perfusion is reduced 5.

Nuclear medicine

SPECT and PET imaging are also a useful adjuncts, with both ictal and inter-ictal scans demonstrating abnormalities

ictal scan: hyper perfusion

interictal scan: hypo perfusion

Other causes of temporal lobe epilepsy (TLE) should be considered, especially as small temproal lobe cortical tumours can have similar appearances.

Treatment and prognosis

TLE is initially managed medically with anti-epileptic agents. In patients who are refractory to medical management temporal lobectomy or selective amygdalohippocampectomy may be performed. Anterior temporal lobectomy is successful in 75-90% of patients with MTS.