CLINICAL REPORT

Focal Cortical Resection for Complex Partial Status
Epilepticus Due to a Paraneoplastic Encephalitis
Fadi Nahab, MD, Adam Heller, MD, and Suzette M. LaRoche, MD

Abstract: We report a 57-year-old female who presented with
epilepsia partialis continua and rapidly progressed to refractory
complex partial status epilepticus (CPSE) with brain magnetic
resonance imaging revealing a focal cortical lesion on T2 sequences
corresponding to the seizure focus on ictal electroencephalographic
recordings. The patient underwent focal cortical resection of the
seizure focus. Though clinical and electrographic seizure activity
ceased, the patient remained unresponsive with repeat neuroimaging
showing diffuse limbic and brainstem involvement. Serological tests
revealed anti-Hu antibodies suggesting a paraneoplastic encephalitis. Chest computed tomography showed a 5-mm pulmonary nodule
and resection of the pulmonary nodule confirmed the diagnosis of
small cell lung cancer. Plasmapheresis was performed without
clinical improvement. Focal resection can be effective in terminating
refractory CPSE but evaluation for a paraneoplastic syndrome must
be considered early in the diagnosis of epilepsia partialis continua
and CPSE as these patients have a poor prognosis.
Key Words: epilepsia partialis continua, paraneoplastic
encephalitis, anti-Hu antibodies, complex partial status epilepticus,
focal resection
(The Neurologist 2008;14: 56 –59)

E

pilepsia partialis continua (EPC) is a clinical syndrome
defined by continuous focal motor seizures of cortical
origin often with minimal or no electrographic changes seen
on scalp electroencephalograph (EEG). EPC in adults is most
often caused by focal structural lesions as a result of vascular
disease, neoplasm, trauma, or infection.1 We present an adult
with EPC who progressed to medically refractory complex
partial status epilepticus. The patient underwent successful focal
cortical resection of the seizure focus with cessation of clinical
and electrographic seizure activity. The presence of anti-Hu
antibodies and pathologic confirmation of small cell lung cancer
led to the diagnosis of paraneoplastic encephalitis.

From the Department of Neurology, Emory University, Atlanta, GA.
Reprints: Fadi Nahab, MD, 1001 Garden View Drive NE, #711, Atlanta, GA
30319. E-mail: fnahab@emory.edu or fadinahab@rocketmail.com.
Copyright © 2008 by Lippincott Williams & Wilkins
ISSN: 1074-7931/08/1401-0056
DOI: 10.1097/NRL.0b013e3181578952

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CASE PRESENTATION
A 57-year-old female was evaluated for a 2 month
history of refractory simple partial seizures. Her first seizure
consisted of right upper extremity clonic movements that
progressed to a generalized tonic-clonic seizure. These developed into frequent episodes of clonic right face, arm, and
occasionally leg movements without alteration of awareness.
She had no significant medical history other than an 80
pack-year smoking history. Despite trials of phenytoin, valproic acid, and levetiracetam titrated to toxic doses, she
continued to have these persistent movements. Our clinical
diagnosis was EPC of unclear etiology. Previous evaluation
had included 2 normal brain magnetic resonance imaging
(MRIs) and an ambulatory electroencephalograph (EEG) that
demonstrated frequent epileptiform discharges over the left
fronto-central region correlating with clinical seizure activity
(Fig. 1). Inpatient admission for further evaluation was declined by the patient and topiramate was added to her AED
regimen of phenytoin, valproic acid, and levetiracetam.
Four days later the patient was brought to the hospital
by family after they noticed some speech difficulty. Examination was notable for expressive aphasia with persistent
clonic movements of the right face and arm. The patient was
admitted to the hospital and on hospital day 1, scalp EEG
showed brief runs of epileptiform discharges over the left
fronto-central region similar to those seen on ambulatory
EEG, which correlated with clinical seizures. Brain MRI on
day 1 revealed a T2 hyperintensity in the left fronto-parietal
region (Fig. 2A–C). The seizures continued despite titration
of topiramate and the addition of clonazepam. On hospital
day 2 a computed tomography (CT) scan of her chest,
abdomen, and pelvis was done to evaluate for possible malignancy and revealed a single 5-mm noncalcified nodule in
the left lower lobe of the lung. The pulmonary team was
consulted and given its small size serial imaging was recommended. Lumbar puncture was performed with cerebrospinal
fluid (CSF) showing an elevated protein of 70 mg/dL but
normal WBC count (2 cells/uL) and glucose (47 mg/dL). CSF
cytology was negative for malignant cells. A serum and CSF
paraneoplastic panel was sent.
On hospital day 8, the neurosurgery team performed a
brain biopsy of the lesion seen on MRI. Pathology revealed
reactive gliosis and no evidence of metastatic or primary
brain neoplasm. The patient’s seizures continued despite
multiple antiepileptic medications and progressed to complex
partial status epilepticus. She was placed in a burst-suppresThe Neurologist • Volume 14, Number 1, January 2008

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Focal Resection in Paraneoplastic Encephalitis

FIGURE 1. Ambulatory EEG showing seizure onset over the left fronto-central region.

sion coma with intravenous diprivan and phenobarbital while
continuing phenytoin, valproic acid, levetiracetam, and topiramate. The patient failed attempts to wean off the anesthetic coma
with regression back to electrographic status epilepticus.
The patient was evaluated for surgical resection of the
seizure focus though functional imaging of the presumed
focus was limited by the patient now being intubated and
therefore ineligible for SPECT and PET scans. On day 16,
intracranial grids were placed over the left fronto-parietal
region for detailed localization; prolonged seizures occurred
over the left frontal region. Motor mapping was completed on
day 18 and the patient was taken for surgical resection on day
22. Pathology on the resected area showed mild-moderate
chronic inflammatory infiltrate involving the leptomeninges,
perivascular spaces of the cortex, and white matter blood
vessels. The pathologist’s differential diagnosis included autoimmune disease, paraneoplastic disease, and Rasmussen’s
encephalitis.
Postoperatively, the patient’s seizures resolved both
clinically and electrographically. Despite resolution of seizures and weaning of anesthetic agents, the patient remained
comatose and ventilator dependent. A follow-up brain MRI
© 2008 Lippincott Williams & Wilkins

was performed on day 29 (Figs. 2D–F). Diffuse T2 hyperintensities were seen throughout the subcortical white matter
and brainstem, consistent with limbic encephalitis. The following day, results of previously obtained serum testing for
anti-Hu antibodies were positive. The patient was taken for
resection of the 5-mm lung nodule and pathologic diagnosis
was consistent with small cell lung carcinoma.
The patient underwent a 5-day course of plasmapheresis
in an attempt to treat the paraneoplastic limbic encephalitis. The
patient remained comatose without recurrence of seizures. Repeat MRI after plasmapheresis remained unchanged. The patient
developed a ventilator-acquired pneumonia in the ICU and the
family agreed on comfort care measures. The patient died on
hospital day 54.

DISCUSSION
The present case highlights an adult with paraneoplastic
encephalitis (PE) presenting as EPC who underwent cortical
resection to treat medically refractory complex partial status
epilepticus (CPSE). Our patient clearly had medically intractable CPSE with failure of multiple medical therapies, includ-

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Nahab et al

The Neurologist • Volume 14, Number 1, January 2008

FIGURE 2. (A,B,C) Brain MRI on hospital day 1, with FLAIR images showing an area of T2 hyperintensity in the left frontoparietal, peri-rolandic distribution, which involves the postcentral gyrus to a greater extent than the precentral gyrus with minimal
mass-effect. (D,E,F) Brain MRI on hospital day 29 with FLAIR images showing diffuse T2 hyperintensity of the bilateral occipital
and temporal lobes, corpus callosum, middle cerebellar peduncles, pons, and left cerebellar hemisphere.

ing anesthetic-induced coma. The patient provided a unique
situation in which the rapidly declining neurologic status after
admission warranted aggressive treatment before the diagnosis of PE was definitive.
EPC has been previously reported as a rare manifestation of an anti-Hu-associated PE.2– 6 EPC was the initial
presentation of an underlying small cell lung cancer in 5 of 7
reported cases. Our patient was unique in that at presentation
there was no evidence of limbic involvement by clinical,
EEG, or radiographic testing. Initial limbic involvement was
seen in all but one previous report of patients with an
anti-Hu-associated PE.2
Our patient demonstrated an atypical evolution of MRI
findings from a focal nonenhancing lesion on T2-weighted
imaging in the sensorimotor area to multifocal involvement
of both limbic and brainstem structures on repeat imaging
after surgical and immunologic therapy. Previous studies of
PE have suggested that MRI represents an important tool in
the diagnosis. The presence of a nonenhancing T2-weighted
abnormality on our patient’s initial MRI is consistent with
previous reports of PE in which up to 83% of patients had
T2-weighted abnormalities.7 However, in our patient, the T2
abnormality may represent the sequelae of recurrent focal
seizures rather than a specific paraneoplastic phenomenon
given that similar T2 abnormalities have been reported in
EPC secondary to nonketotic hyperglycemia.8 Evolution
from focal to multifocal involvement of both limbic and
brainstem structures on our patient’s repeat imaging has not

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been reported previously though patients with PE who undergo repeat neuroimaging often have new abnormalities.9
Our patient’s repeat imaging is similar to that seen in the
majority of paraneoplastic limbic and brainstem encephalitides, including those associated with anti-Hu, anti-Ri, and
anti-Ma2 antibodies.10
Only one other case report has described a patient with
anti-Hu-associated PE who progressed from EPC to a medically refractory CPSE and this patient succumbed to medical
complications as a result of anesthetic coma.4 Our patient
underwent successful focal cortical resection with complete
cessation of clinical and electrographic seizures as in previous reports of successful focal resective surgery of patients
with medically refractory status epilepticus.11,12
There is no definitive treatment for anti-Hu-associated
PE and the prognosis of these patients is poor. The median
survival was 11.8 months in an analysis of 200 patients with
anti-Hu-associated PE.13 Previous treatments have included
the use of antineoplastic therapy with or without immunotherapy and immunotherapy alone, with some evidence of
improvement or stabilization of the PE, though 3-year survival remains 20%.13
EPC that evolves into CPSE can be difficult to treat and
patients with medically refractory CPSE should be considered
for focal resective surgery when a seizure focus can be found.
Given the poor prognosis of patients with anti-Hu-associated PE,
aggressive surgical intervention in these patients is likely to
increase short-term morbidity without extending survival. Thus,
© 2008 Lippincott Williams & Wilkins

The Neurologist • Volume 14, Number 1, January 2008

evaluation for a paraneoplastic syndrome must be considered
early in the diagnosis of EPC and CPSE.
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Focal Resection in Paraneoplastic Encephalitis

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