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History of Present Illness

Patient is a 45 year old Asian female with history of two miscarriages (G5P3), who presents for an evaluation of rhythmical nocturnal seizure-like activity coinciding with spikes in her blood pressure.

The patient was in excellent health until October 2010, pre-partum of her 3rd child's birth (her 4th pregnancy) when she was noted to be hypertensive in the 150s/90s and deemed to have pre-eclampsia (hypertension and mild proteinuria, 406 mg in 24 hr urine). Delivery was induced at 37 weeks and was treated with IV magnesium.

One week post-partum, patient received TdaP vaccination. She had a strong initial reaction to the TdaP vaccination, in which she developed a fever, muscle aches, and multiple days of jaw pain and partial lockjaw.

Following delivery, elevated blood pressures in the 150/90s persisted at home, where she tracked her BP daily. For two weeks post-partum, she was treated with Nifidical XL 30 mg q D, which controlled her blood pressure to the 110s/80s.

Six weeks post-partum, she returned to work and normal activities. She again experienced episodes of elevated BP while not on medication.

On November 23rd (7 weeks post-partum) at 5:15 a.m. she experienced a secondary generalized seizure while sleeping in bed. The seizure lasted for approximately one minute and resolved spontaneously. Paramedics measured her BP at 200/128. She was hospitalized for 24 hours and diagnosed with eclampsia. Patient was discharged on Labetolol 100 mg bid and Nifidical CL 30 mg q D.

She has continued to have these seizures since, at regular intervals, each followed by a week of almost nightly episodes of lip smacking. This seizure-like activity was recorded on video by the patient's husband and shown to a neurologist, who felt it was typical for partial seizures.

Her initial seizure work up was by Dr. Kevin Graber at Stanford Medical Center. Her first EEG showed disorganized background and mild slowing, interpreted to be from either nonspecific cephalopathy or drowsiness.

A 24-hour EEG was completed in January of 2011. During that recording two lip smacking events occurred, but the EEG did not reveal any epileptiform activity.
Patient had an MRI in February 2011. There were no significant abnormalities that would account for the patient’s seizures.
The typical pattern for her seizures is to see a rise in diastolic blood pressure >90 (recordings taken daily) about 48 hours prior to the start. In addition, there are some subtle mood changes in the patient that she is unaware of but her husband notices. The first seizure is usually a generalized seizure with tonic-clonic activity. The next night or night after that there are multiple short episodes of lip-smacking usually, 1-6 per night, occurring approximately every hour or two. They can start within 10 minutes of the patient falling asleep. The seizure events occur most often between 23:00 and 1:00.

In the beginning, she would sometime have a gag-like movement versus the lip-smacking. For the first 6 months there would be an abdominal noise that was very loud that occurred after the lip smacking. Pulse and pulse ox have been measured during some seizures. During an event the patient's pulse rises to approximately 100 from a resting HR of 50, followed by O2 saturation declining from 98-99% to as low as 80%.

Of the three events that are available for review, the first showed O2 at approximately 90%, the second at 80%, and the third 80%. If the patient has a generalized seizure then she is disoriented for about 20-30 minutes afterwards and can't speak for the first 15 seconds. In the morning, she will feel sore all over her body like she participated in a very vigorous workout. If it was just the lip-smacking then she will wake at her baseline in the morning. Patient is unaware of the seizure events when they are occurring, as they always happen in her sleep, including napping during the daytime hours. She has never experienced any incontinence with the seizure events but has had tongue biting which resulted in significant bleeding.

Anti-seizure medications were recommended. As the patient wished to breastfeed her baby, she instead tried a ketogenic diet because patient wished to breastfeed her baby. She remained on the diet for 6-7 months. Her seizure activity continued every 49 days, nocturnally and clustering with a secondary generalized seizure followed by a week of nocturnal partial episodes.

Subsequently, she was evaluated by neuroendocrinologist Dr. Alan Jacobs to see if her cyclic seizure pattern might be hormonally related. Hormone panel acutely during seizure cluster did not demonstrate hormonal influence.

She was able to wean herself off of her antihypertensive medications after 6 months, and her baseline BPs seemed to normalize, but her seizures continued cyclically with the blood pressure rise to the 150s/90s for 48 hours prior.

In April 2012 she had her second miscarriage of an 11 week pregnancy. During this brief pregnancy, she did not have any tonic clonic seizures but did continue to have the lip-smacking episodes. In June 2012 her baseline blood pressure again rose to the 150s/90s and her tonic clonic seizures returned, at which point she was started on benazepril 20mg qAM. She also takes her labetalol 100mg tablets left over from before, as needed for diastolic blood pressure of >90.

Applied Lab Services grew Borrelia Burgdorferi after 2 months even though her previous Lyme testing in 2011was negative (IgG IgM) . IGeneX testing suggested that the spirochete had been present at least a year. She has had no symptoms of Lyme disease. In April 2012, she began treatment with Amoxicillin 1000 mg BID and Azithromycin 500 mg QD in April 2012 and continues on it presently, followed by ILAD physician.

The patient states that prior to her 4th pregnancy (with her 3rd child) she was in excellent health, and never needed to see a physician. On the rare occasion she did have a regular check-up she was never told of any high blood pressures, so she assumes it had been normal previously. She denies any episodic spells of diaphoresis, palpitations, racing heart, headaches, or anxiety at any point. She has had a marked decrease in energy level, however, since her 4th pregnancy. The patient also denies any headaches, abnormal movements (except for during seizure events), changes to her vision, hearing, smell, taste or ability to swallow and no changes to coordination or gait. She has never had a head injury.

Patient is a participant with the Andrews/Reiter Epilepsy Research Program that helps to control seizure activity through biofeedback, relaxation/meditation, exercise and nutritional support.

An abdominal contrast CT performed in July of 2012 revealed a 2.3 x 2.2 x 2.4 cm well circumscribed left adrenal mass measuring with a postcontrast density of 93HU. In combination with the hypertension, pheochromocytoma was the principle concern. The patient underwent a full evaluation with Dr. Karel Pacak, director of the pheochromocytoma research program at the National Institute of Health, including FDG Pet scan, FDOPA PET scan, MRI, serum metanephrine and catecholamines tests.
The final results and report from the NIH is not currently available. Based on the preliminary results (negative plasma metanephrines) they feel that they have ruled out pheochromocytoma.

A repeat MRI of the brain on 7/23/12 at NIH was interpreted as showing a subtle signal abnormality in right hippocampus on flair sequencing. This finding is suggestive of hippocampal sclerosis, the most common cause of complex partial seizures. A neurology consult at the NIH suggested 72-96 hour video EEG monitoring to capture nocturnal events to document partial seizure correlation.

Seizure Pattern
To summarize, the patient has a distinct and unusual seizure pattern. Her seizures are:
  • exclusively nocturnal
  • cluster
  • first cluster of partial seizures becomes a secondary generalized seizure
  • followed by six to seven days of partial seizures (lipsmacking or less frequently a single gagging reflex)
  • rhythmic: cycle repeats every 49-days: secondary generalized and then one week of partials
  • do not appear to be hormonally influenced by progesterone/estrogen/LH/FSH/prolactin
  • Have the features of hypoxia and tachycardia: specifically, there is a jump in heart rate before a pronounced drop in O2 levels.)
  • BP during partial episodes is only mildly elevated. On a number of occasions, patient’s husband has put the BP cuff on the patient within a minute or two of a partial seizure and recorded pressures typically below 150/90. More importantly, he has never recorded a BP during an actual partial seizure and the tachycardia. If the diastolic is above 90, he will have the patient take a Labetolol, something that he’s done maybe a dozen times over the course of approximately 200 partial seizures.

Past Medical History

Unremarkable prior to the onset of conditions noted in the HPI.

Social History

Patient lives with her husband and 3 children in Sonoma County, CA. She is an ophthalmologist by profession but has been retired since 1999, prior to the birth of her first child. She currently works part time as a life coach to physicians and other health care professionals.
She is a lifetime nonsmoker, never drinks alcohol, and has never used any illicit drugs. Prior to her 4th pregnancy, she was highly physically active. She practiced martial arts as a black belt in Tae Kwon Do, was an avid swimmer, and was a yoga instructor.
She currently has a Mirena IUD in place since her last miscarriage in April 2012.

Family History

Mother - miscarriage, essential hypertension, still alive, age 69
Father - CAD, S/P CABG at the age of 68, still alive, age 73
Sister #1 - 42 yo, healthy
Sister #2 - 35 yo, miscarriage, otherwise healthy
Sister #3 - 31 yo, healthy

Current Medications

Amoxicillin 1000 mg bid
Azithromycin 500 mg q D
Labetalol 50 mg po bid



Review of Systems

All systems reviewed and are negative except as noted per HPI.

Physical Examination

(Notes from the NIH examination, July 2012)
Patient is a pleasant right-handed woman in no apparent distress, who is attentive and interactive for questions and fully oriented. She is a good historian. Her speech is fluent and appropriate. Affect is full range. Her head is normocephalic without tenderness on palpation. Her neck is supple without thyromegaly or carotid bruits. She denies any pain on percussion of her back.
Cranial nerve testing reveals:
I: Not tested
II: Bilateral fundi with intact optic disc margins. The pupils are equally round and reactive to light from 5mm to 3mm.
III, IV, VI: Extraocular movements intact without nystagmus.
V, VII: Face symmetric with intact sensation and movement.
VIII: Hearing intact to finger rub bilaterally, Webber test, L=R
IX, X: Uvula midline, soft palate rises symmetrically
XI: SCM, Trap intact 5/5
XII: Tongue midline without fasciculations

Muscle tone and bulk are normal throughout the extremities. Strength is 5/5 in all extremities. Her reflexes at brachioradialis, biceps, triceps, patella and ankle jerks are 2 bilaterally. Plantar responses are down going toes. There is no clonus at the ankles. There is no pronator drift or abnormal movements. She has a normal gait with normal station and arm swing. Toe, heal and tandem gait are intact. Romberg is absent. Patient can stand on one foot. Finger to nose, RAMs, and heel to shin are intact bilaterally. Her sensory examination reveals light touch, temperature, pinprick, vibration, and proprioception intact. There is no hemineglect. She had negative Lhermitte's signs.

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