Paroxysmal speech disorder as the initial symptom in a young adult with anti-N-methyl-D-aspartate receptor encephalitis: A case report

Table 1 Comparing the present case with that from Finke et al[18]

Item	The present case	Finke et al[18]
Age (yr)	39	67
Gender	Male	Male
History of past illness	No	Migraine with aura
Vascular risk factors	No	No
Initial paroxysmal symptoms	Nonfluent aphasia	Right homonymous hemianopia, global aphasia and right hemiparesis
Accompanying symptoms	Generalized tonic-clonic seizures	Throbbing bilateral headaches, confusion and agitation
CSF analysis	Mild pleocytosis (28 cells/μL) dominated by lymphocytes (85%) and elevated protein (662 mg/L)	Lymphocytic pleocytosis (95 cells/mL) with few activated lymphocytes and plasma cells and elevated protein (96 mg/dL)
Brain MRI	No lesions	Mild frontoparietal microangiopathic leucoencephalopathy
EEG	No epileptic discharges	First: Moderate generalized slowing; r: Normal
Tumor screening	Negative	Negative
Testing for anti-NMDAR antibodies	IgG NMDAR antibodies in both CSF (titer, 1:10) and serum (titer, 1:32)	IgG NMDAR antibodies in CSF (titer, 1:32), but not serum
Treatment	Intravenous immunoglobulin and methylprednisolone, followed by oral methylprednisolone	Oral corticosteroids and plasma exchange, followed by azathioprine
Outcome	Asymptomatic	No further episodes occurred, but verbal long-term memory deficit persisted

MRI: Magnetic resonance imaging; CSF: Cerebrospinal fluid; EEG: Electroencephalogram; NMDAR: N-methyl-D-aspartate receptor.

Table 2 Reported cases of anti-N-methyl-D-aspartate receptor encephalitis with aphasia as the sole or dominant manifestation

Item	Constantinides et al[19]	Hébert et al[17]	Deiva et al[20]
Age (yr)	29	29	4
Gender	Female	Female	Female
Presenting symptoms	Isolated, abrupt-onset aphasia	A progressive nonfluent aphasia; simple partial seizures; confusion and emotional lability	Fever; repeated right partial motor seizures; sudden and isolated Broca's aphasia
Description of language difficulties	With a 6-mo history of aphasia; her prominent impairment, namely, non-fluent aphasic disturbances (effortful, halting speech with sound errors), had progressed rapidly and reached a peak in 72 h, at which point she was unable to speak and had difficulties in writing, but her ability to perceive verbal stimuli was relatively preserved	6-d history of progressive word-finding difficulties	The patient suddenly presented isolated speech difficulties; speech evaluation showed that her receptive language was preserved but that expressive language was affected associated with anomia, and anarthria suggestive of Broca's aphasia
EEG	Paroxysmal left temporal theta and delta waves	Abundant intermittent polymorphic slow wave activity over the left lateral frontotemporal area	Waking EEG was characterized by unilateral left hemispheric slowing, and sleep EEG showed a repetitive pattern of focal theta rhythms over 10-15 s in the postero-temporal region which then spread to the whole left hemisphere for 45-60 s
Brain MRI	Normal	Normal	Normal
CSF analysis	Within normal limits (3 white blood cells × 106/L, protein 420 g/L), with negative cytology	Within normal limits (2 white blood cells × 106/L, 95% lymphocytes, protein 0.20 g/L, glucose 3.7 mmol/L) with normal cytology	19 leukocytes, with 0.22 g/L of protein and no oligoclonal bands
Testing for anti-NMDAR antibodies	Positive in both serum and CSF	Positive in CSF	Positive (1:100) in both serum and CSF
Screening for ovarian teratoma	Negative	A 5.3 cm right adnexal cystic teratoma (confirmed by pathology)	Negative
Immunotherapy	A 5-d course of intravenous methylprednisolone 1 g/d, followed by slowly tapered oral methylprednisolone 1 mg/kg per day; six courses of plasmapheresis; azathioprine 50 mg bid	A 2d course of 2 mg/kg intravenous immunoglobulin	Intravenous rituximab (375 mg/m2)
Prognosis	Aphasia eventually resolved at the 1 yr follow-up	10 mo after symptom onset, her language impairments completely resolved, but she had impaired recollection of the events surrounding her hospitalization	After 20 mo of follow-up, the child had completely recovered and was free of seizures

EEG: Electroencephalogram; MRI: Magnetic resonance imaging; CSF: Cerebrospinal fluid; NMDAR: N-methyl-D-aspartate receptor.