Question Title: If not CNS LYMPHOMA or VASCULITIS, then what?

Forum: Neurology Forum
Topic: Neurology - General

His initial symptoms were dizziness, poor coordination, slurred speech. Rufusing to see a physician, my husband attributed the symptoms to drug reaction, stress, etc. January of 1996 he had a severe headache and had a CTscan which showed nothing (he had always had a lot of headaches but this was by far the worst).

He began having problems with his eyes, double vision. The opthamologist denied that the above symptoms were related and his diagnosis was conjuncavitis. Within a few days he had difficulty speaking and went to the hospital where he was met by a neurologist. After many tests, MRI and CTscans, the doctors concluded he had a stroke area of his cerebellum. He was put on Ticlid. Aside from an episode of back pain and a severe case of hives, he made a remarkable recovery (Note: Two other pre-existing conditions were back pain and Barrett's Esophagus -- he had a super selective vagotomy in 1988 but the acid has never been under control. The hives were attributed to the Prevacid he was put on at that time for a severe case of esophagitis.)

That July the symptoms seemed to be returning and he had another MRI which showed evidence consistent with a stroke in the cerebellum. He refused to see a neurologist.

The slurred speech continued along with poor coordination. On October 31, 1996 31 he saw a neurologist at Lahey Clinic. After two days of testing they discovered a tumor mass in the cerebellum. Surgery was scheduled for 11/13 and he was put on a course of Decadron to be tapered off over the next two weeks. The surgery was performed without problems. The initial diagnosis was a Glioblastoma Multifore but the final pathology report on 11/18/96 was "consistent with malignant lymphoma. Chemotherapy and radiation were recommended.

We then saw a Lymphoma specialist at the Brigham and Women's in Boston for a second opinion. This doctor called this "a very interesting problem...a slow growing left cerebellar lesion and most likely non-Hodgkins lymphoma." He believed that the original "stroke" in February and all other symptoms were related and concluded that if may be a low grade Lymphoma which "could possibly be eradicated just by radiation therapy." With this second opinion we decided to wait for treatment until the next MRI in January. That MRI showed no changes so postopned a decision to treat.

The next few months (we were in Florida) Ray was extremely fatigued all the time and began frequently choking, having difficulty concentrating, reading, irritablility and depression. He developed an erratic breathing pattern and high blood pressure. He had difficulty with speech and very poor use of the left upper extremity. An MRI in late April showed new lesions in the thalllamic and temporal region.

He was admitted to Boston's Brigham and Women's Hospital for futher workup of "presumed central nervous system lymphoma versus vasculitis and the identification of the CNS lesions progressively observed on serial MRI scans. All blood and urine , lumbar puncture, spinal MRI and abdomen/pelvis CT scan were all normal. An angiography of carotids obtained 5/7 showed "no vascular lesions in the brain and no relation of the masses to the vascular structures." During the procedure, however, Ray suffered an embolic stroke. He was unable to speak and had "mild right sided hemiparesis." A branch of the middle cerebral artery was occluded (beyond the M2 segment).

He made steady improvements, however his ability to speak or swallow never returned. A J-tube for feed and a tracheostomy tube were placed.

His "....original biopsy samples were circulated with Hematology/Oncology at Dana-Farber Cancer Institue and the Brigham and Women's Hospital, and it was found to be non diagnostic becase of the Decadron treatment the patient received prior to the biopsy...". The NCI was also contacted and further work-up was done on the original sample but showed no new insight.

Ray spent another month at a rehab facility and came home July 1996, where he has remained (aside from frequent hospitalization with conditions related to the J-tube and Trach tube) in my care, 24 hours a day, 7 days a week. He still cannot speak or swallow nor communicate in any way other than nodding his head yes or no. He cannot be left alone due to his coordination and motor planning difficulties.

He has made remarkable, though slow, progress and there have been no new lesions of any sort on any of the MRI's to date. Even his neurologist told us he never expected to see him do so well. They have pretty much ruled our Lymphoma due to the length of time he has had the condition. The last word we had from the neurologist was "vacsculitis which is in remission or something that they may have overlooked.

The only medications he takes are 40mg Prilosec and 75mg Effexor daily. In addition, I daily give him 900mg Vitamin C, 1/2 tsp garlic extract, and either Echinechea or Astragulus. When he becomes anemic (due to esophageal bleeding) he gets iron. He uses a nebulizer daily with Ipatroprium Bromide daily. He has frequent bouts of respiratory infection when he then has Levaqun, Trovan, Cephalexin and Potasium Iodide to help with secretions. He is now on 10mg Ritilin to see if he can be more wakeful as he sleeps 16 hours or more each day. He was always a very physically active person but, though he can do nothing, he is not depressed and has a very positive outlook.

I've often wondered if there is something in his family history or his past experiences that exposed him to some chemical or other element that may be responsible for his current condition. I know I am stretching afar, but following is a little history.

He was raised on a farm. Of his seven older siblings, one died of uterine cancer, a second had a malignant melanoma removed and treated, a third had breast cancer, a fourth was diagnosed with Takayasu's Areteritis, a fifth had a "subarachnoid hemorrhage" at the base of the brain. his father died in 1953 after brain surgery after what was considered some kind of stroke. Ray, two sisters and his father and son all have a condition causing severe acid secretion. Ray and his son both have Barrett's Esophagus.

Since 1958 Ray, who is a pilot, flew corporate jets. He has been exposed to many chemicals, including the "dope" used in the construction of fabric airplanes. For years, as a hobby, he rebuilt automobiles, using body putty and paints and all the chemicals necessary. He did this in our garage which was attached to our home. All of us would suffer the effects whenever he painted or worked on a car.

I know I am grasping for straws, but I hope that someone out there has a suggestion for the "something they may have overlooked."

Joanne Ridinger

Dear Joanne:

A few thoughts regarding the information supplied in your letter:

-I agree that as more time passes, the diagnosis of Lymphoma becomes
less likely given its traditionally progressive course.
-The use of steroids prior to biopsy may alter the results of standard
pathologic studies. However, special immunostaining techniques can
still detect the cellular traits of lymphoma (?were these done at
Dana Farber)
- It is hard to remark upon potential conditions overlooked without
knowing what other studies were done during your husbands workup
(i.e. the specific tests run on the spinal fluid and blood, etc.)
Your care was likely exemplary, given the reputation of the institutions
mentioned in your letter. Some of the other conditions which were
likely tested for (and could cause diffuse lesions responsive to
steroids) include MS, rare MS varients, primary or secondary CNS
vasculitis (incl. Lupus and Sjogrens disease), HIV, HTLV-1, and
ADEM.
I hope this has been helpful. Good luck to you and your husband.

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