Opercular Syndrome: A Rare Presentation of Schilder's Variant of Multiple Sclerosis
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.344611
Source of Support: None, Conflict of Interest: None
Schilder's disease (SD), also known as Myelinoclastic diffuse sclerosis (MDS), is a very rare demyelinating disorder and traditionally considered as a variant of multiple sclerosis (MS). However, in the recent past, few studies reported clinical as well as immunopathological disparity between the two entities.,, Opercular syndrome also known as Foix–Chavany–Marie syndrome (FCMS), facio–labio–glosso–pharyngolaryngo–brachial paralysis, and cortical type of pseudobulbar paralysis, is a rare occurrence due to bilateral lesions of opercular cortex surrounding the insula. Manifestations include central type of facial paresis, difficulty in opening mouth, chewing food, deglutition, protruding tongue, and inability to vocalize. Here we are reporting a rare case of Schilder's variant of MS presenting as Opercular syndrome.
A 24-year-old right-handed woman presented with insidious onset gradually progressive weakness of right upper limb for 1 month followed by right lower limb for 15 days. Over the next 5 days, patient also noticed mild weakness in left upper and lower extremities. She was unable to vocalize for last 5 days, though she was able to comprehend. Two episodes of focal seizure with bilateral tonic-clonic movements occurred since the onset of illness and patient had persistent mild to moderate headache. There was no previous history of fever, ear or nasal discharge, trauma and surgery.
On examination, she was conscious, alert, cooperative and afebrile. Her blood pressure was 110/78 mm of Hg and pulse rate 80/min. Power in right upper and lower limb was 1/5 and 3/5 respectively while on left side it was 4/5 in upper and lower limbs. Deep tendon reflexes were brisk in all 4 limbs with bilateral extensor plantars. There was mild spasticity in the tongue with brisk jaw jerk reflex. She had pure motor aphasia with preserved comprehension. She was unable to open her mouth or protrude tongue on commands but do so while laughing or yawning. Fundus examination was normal. There were no involuntary movements. Sensory and cerebellar examinations were normal. Cardiorespiratory and abdominal examinations were within normal limit. Hematological, kidney and liver function test, thyroid function test, electrocardiography and 2D echocardiography revealed normal study. Cerebrospinal fluid (CSF) analysis showed <5 cells/mm3, protein 32.5 mg/dL and sugar 77 mg/dL. Oligoclonal band was negative in CSF. CSF study for gram and acid-fast bacilli staining, India ink staining, Gene-Xpert, bacterial and fungal cultures, PCR for HSV, CMV, JE, and malignant cytology were negative. MRI brain revealed large lesions involving bilateral cerebral hemispheres [Figure 1]. Typical bilateral subcortical location involving the centrum semiovale with other radiological features favors the diagnosis of SD.
Patient was started on injection methylprednisolone 1 gm IV once daily for 5 days, along with injection levetiracetam 500 mg twice daily. There was partial improvement in in her weakness as well as speech at the end of IV therapy. Later, she was switched to oral prednisone 1 mg/kg body weight as maintenance dose. At 6 weeks follow up, power in right upper limb was 3/5, right lower limb 4/5 with normal power in left upper and lower limbs. Her spontaneous speech improved but fluency was poor.
SD typically presents with cognitive as well as focal neurological deficit, while the present case had classical features of Opercular syndrome. On imaging, the radiological findings were typical and she responded dramatically to steroids with no relapse in further follow up. MDS, also termed as SD was first reported by the Austrian psychiatrist Paul Ferdinand Schilder in 1912. Since the histopathological features of these diseases were similar to MS, they were classified as MS variants, although their clinical features vary from MS to some degree., Contrary to MS, lesions in MDS are often bilateral, symmetrical, demyelinating involving the centrum semiovale, have monophasic course, low recurrence rates, spare cortex and spinal cord, and commonly affect children. Jarius et al. also observed absent oligoclonal bands (77%), lack of pleocytosis (79%), and elevated CSF proteins (56%) in MDS and reported MDS as immune-pathologically distinct entities from MS.
The clinical course of SD is very diverse. There will be subacute or chronic cognitive deterioration, spastic paresis, seizure, and involvement of vision and hearing. Features of raised intracranial pressure, headache, and vomiting may suggest a mass lesion. The cerebrospinal fluid study can be normal or with slight elevation of cell count or protein. Close differential diagnosis is primary brain tumor, metastasis, cerebral abscess, acute disseminated encephalomyelitis (ADEM) or inherited metabolic disorders of myelin, particularly adrenoleukodystrophy.
FCMS or Opercular syndrome is a rare entity, described as lip-facio-laryngo-glosso-masticatory diplegia along with voluntary-autonomic dissociation, without mental alteration. The etiology is mostly vascular (thrombosis or embolism) followed by tumor, developmental abnormality, infection, or demyelinating disorder. Among demyelinating disorder, there is no available literature of Opercular syndrome with SD.
Poser et al. proposed the diagnostic criteria for SD: (a) clinical symptoms and signs often atypical for the early course of MS; (b) CSF normal or atypical for MS; (c) bilateral large areas of demyelination of cerebral white matter measuring at least 3 × 2 cm; (d) no history of fever, viral or mycoplasma infection or vaccination preceding the symptom onset; and (e) normal serum concentrations of very-long-chain fatty acids. Bacigaluppi et al. suggested the neuroradiologic features to diagnose SD if Poser's criteria are fulfilled: No signs of raised intracranial pressure, presence of one or two subcortical cyst-like lesions with open ring sign on CT or MRI, exclusion of ischemia, primary tumor, metastasis and abscess by typical radiological findings and perfusion MRI, and consideration of an abnormal glutamate peak on short-echo time MRS.
Corticosteroid remains the mainstay of treatment of SD. As such, there are no guidelines about the use of corticosteroids, their doses and duration or other immunomodulators for the treatment of SD. However, based on the available literature, SD should be treated initially like an MS attack, with pulse steroids, with further tapering of oral doses as the neurological symptoms stabilize.
To conclude, SD itself is a very rare condition and its association with Opercular syndrome is not available in the published literature. To the best of our knowledge, this is the first case report of SD with Opercular syndrome.
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