FRIDAY NOON CONFERENCELets continue the culture……

NARESH MULLAGURIRESIDENT PHYSICIAN PGY4

DEPARTMENT OF NEUROLOGYUNIVERISTY OF MISSOURI

Chief Complaint Emergency Room Consultation – Evaluate the patient for possible Seizure 50 Year old Caucasian male presented to the ER with the spells of right sided

head and gaze deviation, stretching out of the right upper extremity and flexion of the left upper extremity with clonic movements of the right upper and lower extremities with labored breathing lasting for almost 2 to 2 ½ minutes with no loss of consciousness, no bowel or bladder incontinence, tongue biting.

The spell was followed by lethargy for about 3-5min to become completely oriented with amnesia of the event. This is his fourth episode in the past 24hrs but the first seizure started exactly a week ago with none in the past 6 days until yesterday. For the past week, he had been experiencing right sided pins and needles sensation in the face starting from eye brow and marching towards the leg.

No HA, Nausea or aura prior to the spells like Neck pain, epigastric rising sensation, automatisms, altered smell or taste or visual phenomenon. Most seizures happened overnight but he had one seizure in the ER when the resident was evaluating the patient.

History continued First Seizure in 2003 which is a generalized tonic clonic seizure was worked

up and was prescribed Dilatin. He was on it in the hospital but thought he was cured and stopped taking it after discharge and remained spell free for the last 13 years. History of partial status epilepticus at that time and was in ICU.

He had known history of Cerebral palsy with mild right sided weakness but was on the high functioning side in terms of cognitive abilities and independent of all the ADLs.

He had history of right wrist extensor weakness but after the seizures he developed wrist drop and some right lower extremity weakness.

ROS Denied any trauma, fever, chills or rigors, neck pain or stiffness, cough,

SOB, diarrhea or constipation, joint pain or swelling, double vision, loss of vision. No recent infections, vaccinations or changes in any medications.

Other Pertinent histories PMH: HTN, IDDM, Cerebral Palsy, Seizures PSH: None FH: No significant Medical problems like Seizures, Stroke or cancer. SH: Lives with his wife, married for 13 years. Denied smoking, drinking

or usage of any recreational drugs in the past or currently. Medications: Insulin NPH, Glipizide, Lisinopril, Lovastatin, Metformin

HISOTORICAL DIFFERENTAL DIAGNOSIS Complex Partial Seizures – Frontal lobe given the Semiology of Seizure (SMA) Possible Etiology: Structural lesion given the h/o Cerebral Palsy, Hyperglycemia

triggering partial seizures given uncontrolled DM, Less likely infection, Autoimmune or Neoplastic causes.

The structural lesion can be Stroke from birth injury, Neuronal migration disorder with cortical dysplasia, vascular abnormalities like Cavernous malformations or AVF, Tumors like Meningioma or low grade glioma,

Vascular reasons like Cortical venous thrombosis or embolic stroke in the ACA/MCA territory given the risk factors like HTN, DM.

Physical Examination General: Moderately obese gentleman in no distress Eyes: Normal conjunctiva, No pallor, Fundus showed clear disc margins HEENT: No tongue bite, moist, dentition is poor with no gingivitis, No tonsillar

hypertrophy, no congestion of the posterior pharyngeal wall. No typical syndromic facies.

RS: Non labored breathing, clear to auscultation bilaterally CVS: RRR, S1 and S2 were heard, no murmurs, No carotid bruits, No Pedal edema or

JVD GI: Soft with no organomegaly. MSK: No dislocations or tenderness over the joints, Wasting of the intrinsic hand

muscles in the right hand Skin: no bruises or lesions, No neurocutaneous markers like Café au lait spots, ash leaf

macules, NF, port wine stain, telangiectasia. Psychiatric: appropriate mood and judgement

Neurological Examination HMF: Alert, awake and oriented to name, place, person and situation. Speech is clear,

naming, fluency, repetition, comprehension is intact. Able to read the sentences presented. No extinction. No loss of graphesthesia or astereognosis. No frontal release signs. No extinction or neglect.

CN: Visual fields are full to confrontation, Pupils were 4mm, round, reactive to light bilaterally for direct and consensual reflex, accommodation reflex is present. No gaze preference. EOM were intact, Sensations were intact to light touch, temperature and in both sides of the face in all the trigeminal distribution. No facial asymmetry, able to close the eyes tightly, able to smile symmetrically. Soft palate is elevated normally, shoulder shrug is strong bilaterally, tongue protrudes to midline.

Motor: Tone is slightly decreased on the right side, Hypothenar eminence atrophy in the right hand, decreased strength in the right hand grip 3/5, Wrist extension 2/5 with ulnar deviation, hip flexors, extensors, knee flexors and extensors were 4+/5. Left side is 5/5 in all the muscle groups. DTRs are 2+/4 in bilateral upper and lower extremities except for decreased right brachioradialis. No Hoffman’s sign, Plantars were downgoing bilaterally.

Examination continued… Sensory: Intact in all the four extremities to light touch, pain, temperature and vibration. No

extinction. Coordination: FTN is intact on the left side and HTS is intact bilaterally Gait: Not assessed.

Differential Diagnosis Complex partial seizure - Frontal Lobe seizure with SMA involvement– Structural

lesion including congenital malformations to tumor or new infarct. Todd’s paralysis in the Right upper extremity

Hyperglycemia induced Complex partial Seizure. Vascular malformations, CVT and Embolic stroke still prevails.

Investigations WBC : 7.4 HGB: 15.9 PLT: 189 Normal BMP Glucose – 336 CT Head: Irregular sulcation pattern with in the Left Frontal Lobe with focal parenchymal

atrophy and scattered left frontal lobe dystrophic calcifications.

MRI Brain with and without contrast

Types of Frontal Lobe Seizures Supplementary Motor Area Seizures Cingulate Seizures Anterior Frontopolar seizures Orbitofrontal Seizures Dorsolateral Seizures Opercular Seizures Motor Cortex Seizures

General Characteristics of FLE Prominence of Motor manifestations – automatisms as well as tonic – clonic activity. Rapid secondary generalization - little or no evidence of focal onset Focal clonic activity with or without march Prominent asymmetric tonic posturing Complex, bizarre motor automatisms Explosive onset and sudden ending with minimal postictal confusion Frequent, brief seizures often in clusters

The frontal lobe is the largest lobe and gives rise to seizures with distinctive features depending on the area of the frontal lobe involved. Motor features are prominent and range from hypermotor thrashing attacks with pelvic thrusting and bipedal automatisms to asymmetric tonic posturing. Frontal lobe seizures may begin with a brief aura, even when seizures occur from sleep. Seizures are typically brief, and can have prominent vocalization, bizarre behavior, urinary incontinence, and head and eye deviation. Frontal lobe seizures may be exclusively nocturnal and often cluster. The ictal EEG may not show ictal patterns or may be obscured by movement artifact.

CAUTION When consciousness is impaired, frontal dyscognitive seizures can be difficult to distinguish from absence seizures.

CAUTION Nocturnal frontal lobe seizures can be mistaken for parasomnias, however:

Frontal lobe seizures are usually brief events (< 2 minutes), with stereotyped features seen from seizure to seizure and preserved awareness. Parasomnias are usually longer in duration (> 10 minutes), have variable features from event to event and are characterized by a confusional state with the patient having no memory of the event afterwards.

In parasomnias, clustering is rare and the common non-REM parasomnias typically occur 1-2 hours after falling asleep, in the first cycle of deep slow wave sleep. Nocturnal frontal lobe seizures typically occur throughout the night, and more frequently within half an hour of falling asleep or awakening.

CAUTION Frontal lobe seizures may be mis-diagnosed as non-epileptic seizures as there may be bilateral motor phenomena with preserved awareness, and the ictal EEG can be normal.

SMA seizures

Frequent, brief episodes occuring in clusters Explosive onset and offset with no post ictal confusion Nocturnal preponderence Prominent tonic posturing usually of the contralateral upper extremity Contraversive head and eye deviation Fencing or M2e posture Preservation of consciousness in some Postictal Todd’s palsy (mild)

Motor cortex Seizures Mainly simple motor seizures Focal motor tonic-clonic, clonic movements with or without Jacksonian march Localized, rhythmix or arrhythmic, clonic movements contralateral to the focus Distal>proximal Hand(thumb) and face(Lips) preferentially affected(Homunculus of penfield) Remains highly localized or march to neighboring motor regions Myoclonic Seizures: Predominantly facial or distal in the limbs Tonic Postural motor seizures

Frontal lobe CPS Frequent, brief seizures occurring in clusters Sudden onset and offset with little or no postictal confusion,

Consciousness partially retained but commonly amnestic for episodes Nocturnal preponderance Complex motor automatisms-semi purposeful motor activity often in

combination with tonic clonic motor manifestations Vocalization from simple humming to shouted expletives Prominent mood changes Sexual automatisms Overall bizarre hysterical appearance ”Pseudo voluntary nature” Commonly misdiagnosed as Psychogenic attacks.

Difference between FLE and TLE