NOROL BIL D 18: 3 , 2001
Ayse SAGDUYU, Nese CELEBISOY
Ege University Faculty of Medicine, Department of Neurology, Bornova, Izmir, 35100, Turkey
A 38-year-old male patient who was initially followed up as relapsing remitting multiple sclerosis (MS) subsequently developed transitional diffuse sclerosis with typical MRI findings and frontal syndrome as a very unusual clinical presentation.
Transitional diffuse sclerosis (TDS) is a distinct and quite rare form of multiple sclerosis(MS). It differs from Schilder’s disease (SD) with the presence of smaller unrelated plaques of myelinoclasia in addition to large hemispheric plaques (6). Its age of onset and the chronic relapsing course resembles MS more than SD (1,6,8). This paper is about a patient initially diagnosed as relapsing-remitting MS who subsequently developed transitional diffuse sclerosis, presenting with symptoms of frontal syndrome.
A 38 year-old-man was admitted to hospital because of behavioral abnormalities and urinary incontinence, impotence as well as gait and speech disorders that had progressed for the last three months.
His wife reported that he was less talkative, usually sitting on his own with loss of initiative and interest. He had bursts of anger and violence. He had urinary and fecal incontinence which he was unaware and emotionless. He had difficulty in gait initiation with slow and short steps. He was on follow up in our MS outpatients clinic.
His medical records revealed that his first attack was in 1986 with loss of vision in his right eye which responded to corticotherapy. In 1991, he was admitted for the second time with left sided hypoestesia which resolved completely in a month with ACTH. His third admission was in 1995 with a right sided weakness treated with pulse methylprednisolon and resolved in approximately 3 weeks. His medical history was otherwise normal. His family history was unremarkable.
On examination, he was apathetic and exhibited emotional lability. He had difficulty in initiating conversation, continued with brief, common words and long pauses. He had a prominent loss of interest and was poorly cooperated. His physical examination was normal.
The neurological examination showed a minimal right sided hemiparesis with bilateral plantar extensor responses and urinary and fecal incontinence. The optic fundi revealed bilateral temporal pallor. His gait was slow with difficulty in initiation and short steps. Motor and mental impersistence was prominent.
Benton Visual Retention Test-Revised F and Mini Mental State Examination demonstrated cognitive decline and loss of attention with short and long term memory deficits. His complete blood count and biochemical analysis were normal.
CSF protein, Ig G and glucose concentrations were normal and no oligoclonal bands were present. The visual evoked potentials (VEP) with stimulation of both eyes showed P100 potentials with increased latency and decreased amplitude. Brain stem auditory evoked potentials (BAEP) were normal.
Somatosensory evoked potentials were abnormal on both sides. Adrenal function tests were normal. The MRI revealed bilateral large, confluent fronto-parietal white matter lesions which were hyperintense on T2-weighted (Fig 1) and hypointense on T1-weighted images with surrounding edema and marginal contrast enhancement (Fig 2).
His previous MRI gathered from his medical records was in 1991 and showed bilateral parieto-occipital hyperintense lesions in close relation with the posterior horns of the lateral ventricles on T2-weighted images (Fig 3). He was treated with pulse MP 1 gr/day, for seven days, followed by tapering oral doses.
After treatment, his fecal and urinary incontinence and gait disorder recovered,. his emotional lability disappeared, the spontaneity and verbal output improved. Control MRI, performed 2 weeks later, showed only a mild regression.
Myelinoclastic diffuse sclerosis or SD is a rare, acute or subacute demyelinating disorder affecting mostly children but which also affects the adults (3,5).
It results in the formation of one or more commonly two, roughly symmetrical, bilateral , large plaques involving the centrum semiovale of the cerebral hemispheres (2). These must be only lesions that can be demonstrated on the basis of clinical, paraclinical (e.g. evoked potential) or imaging studies. There must be no involvement of the peripheral nervous system (7).
The fact is that in many such cases, additional smaller, unrelated plaques of myelinoclasia are found elsewhere in the central nervous system. The later situation is than called “transitional” or “diffuse-disseminated sclerosis (4-7). It occurs most often in adults and these cases are similar to that of chronic relapsing MS and frequently the illness runs a protracted and remitting course (1,8) Poser’s review of the literature (7) uncovered 105 cases that could be designed as the Schilder type of diffuse sclerosis. In 72 of them, isolated demyelinative plaques were found in other parts of the central nervous system in addition to the large foci in the cerebral white matter.
Diffuse cerebral sclerosis of this type must be closely related to MS and may indeed be a variant of it as was originally proposed by Schilder (1). SD and TDS represent rare topographical and aggressive variants within the spectrum of MS.
Our patient had two interesting points: First, he had a very unusual clinical presentation for MS and the second is that, he was initially diagnosed as MS and was followed up for five years when he developed large bilateral hemispheric white matter lesions which were typical for Schilder’s disease according to Poser’s description. While his previous clinical, paraclinical and radiological findings were on the behalf of MS, the last MRI was consistent with SD, so he was diagnosed as TDS.
(c) Journal of Neurological Sciences