He Struggled With Walking and Talking … But Why? Articulation problems led this patient to a speech consultation—which helped reveal a rare and serious disease. Case Puzzler
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Case Puzzler  |   February 01, 2018
He Struggled With Walking and Talking … But Why?
Author Notes
  • Joseph R. Duffy, PhD, CCC-SLP, is an emeritus consultant in the Division of Speech Pathology in the Department of Neurology and professor of speech pathology at the Mayo Clinic in Rochester, Minnesota. He is an affiliate of ASHA Special Interest Groups 2, Neurogenic Communication Disorders; and 19, Speech Science. jduffy@mayo.edu
    Joseph R. Duffy, PhD, CCC-SLP, is an emeritus consultant in the Division of Speech Pathology in the Department of Neurology and professor of speech pathology at the Mayo Clinic in Rochester, Minnesota. He is an affiliate of ASHA Special Interest Groups 2, Neurogenic Communication Disorders; and 19, Speech Science. jduffy@mayo.edu×
Article Information
Speech, Voice & Prosodic Disorders / Professional Issues & Training / Case Puzzler
Case Puzzler   |   February 01, 2018
He Struggled With Walking and Talking … But Why?
The ASHA Leader, February 2018, Vol. 23, 44-45. doi:10.1044/leader.CP.23022018.44
The ASHA Leader, February 2018, Vol. 23, 44-45. doi:10.1044/leader.CP.23022018.44
For four months, his speech and balance had deteriorated. The first sign for “Mark,” 44, had been mild slurring of speech. Then came balance problems and falls, and now he was using a walker.
Mark’s only other complaint was a four-year history of erectile dysfunction and reduced testosterone levels. A neurologist colleague of mine had evaluated him and noted speech difficulty, clumsiness and an unusual, stiff-legged, awkward gait. But there were no other abnormalities—the pattern of problems did not fit with any common neurological disease.
Puzzled, the neurologist referred Mark to me for a speech evaluation.
Before the evaluation, the neurologist stopped by to let me know he thought that Mark’s problems might be psychogenic. He wanted my opinion to see if he was on the right track in organizing an appropriate workup.
If I felt the speech problem was indeed psychogenic, the neurologist would request more consultations to determine if all or most of the patient’s symptoms were nonorganic. If I thought the problem was organic and could point to its localization, he’d order a different set of consultations and tests. I told the neurologist I’d get back to him, recognizing that this was a case in which the speech diagnosis might be important to medical diagnosis.
Speech held the clue
When I saw Mark later that day, he told me the changes in his speech weren’t evident to others initially, but were now. His speech problem and gait difficulty now left him unable to work as a plant manager—a position he’d held for many years. He felt that drinking small amounts of alcohol substantially worsened his speech and that he had to work hard to position food in his mouth. He had no complaints about swallowing.
Mark’s speech rate was moderately slow, with a scanning prosodic pattern, and pitch and loudness fluctuations that varied from reduced to abnormally variable. He had irregular breakdowns in articulation. His alternating motion rates (for example, rapid repetition of “puhpuhpuh … ”) were moderately irregular and slow. In open-ended conversation, his speech intelligibility was mildly reduced.

I told the neurologist I’d get back to him, recognizing that this was a case in which the speech diagnosis might be important to medical diagnosis.

His oral mechanism was normal in size, strength, symmetry and range of motion, and oral praxis was normal.
Together these findings were consistent with a moderately severe ataxic dysarthria that was strongly suggestive of cerebellar dysfunction. I told Mark and his wife that his speech difficulty was almost certainly neurologic, and that it was probably due to abnormal functioning of the cerebellum. I conveyed that I thought a psychological cause was unlikely, but said final conclusions should await completion of his workup.
I reviewed this with his neurologist, and we talked about the next steps for testing in light of the high probability of ataxic dysarthria. We wondered if Mark might have a paraneoplastic syndrome, which can affect various parts of the nervous system, often the cerebellum. These very uncommon syndromes (affecting less than 1 percent of the cancer population) derive from cancer present elsewhere in the body, such as in the lung or ovary.
Paraneoplastic syndromes reflect an immunologic response that attacks cells in locations beyond the primary tumor. When cells in the cerebellum are the target, the disorder is called paraneoplastic cerebellar degeneration. Its signs and symptoms tend to emerge subacutely, often weeks or even years before the primary tumor is identified. Dysarthria can be a prominent early sign.
It was an uncommon possibility, but one with significant implications for Mark’s health.

Mark’s speech problem and gait difficulty left him unable to work as a plant manager—a position he’d held for many years.

Diagnosis?
Over the next two weeks, Mark met with physicians in neuroimmunology, infectious disease and endocrinology. He underwent multiple tests. The findings were revealing: a brain MRI showed abnormalities in the cerebellar vermis and cerebellar folia, and a whole body FDG-PET scan showed abnormities in the cerebellar vermis and below the left kidney.
So, given these findings, what was the diagnosis? Mark indeed had a paraneoplastic cerebellar disorder in response to a primary seminoma—a type of cancerous tumor—in his lower abdomen. A seminoma is usually a testicular tumor, but can also occur in other locations; it is a treatable and often curable cancer if discovered early.
Mark began chemotherapy and shortly afterward began physical and occupational therapy and speech treatment. His speech at that time was a bit worse, perhaps because of increased fatigue. Speech treatment focused primarily on rate-reduction strategies to improve intelligibility.
After Mark completed three cycles of chemotherapy over three months, another FDG-PET scan showed near complete resolution of the seminoma. Unfortunately—but not uncommonly because the damage to the nervous system can be permanent—his neurologic symptoms had improved only minimally. His ataxic dysarthria was somewhat better but still moderately severe.
Two months later, his balance was a bit better. He could mow his lawn on a riding mower, and his handwriting had improved. He was uncertain if his speech had improved significantly, but he continued with speech treatment in his hometown. He had no major complications from his immunotherapy and his treatment-related nausea was gone. His neurologist arranged to reassess his neurologic problems in three months. I will see him for speech re-evaluation at that time.
This complex and unusual case illustrates the value of speech evaluation at or near the time of onset of difficult-to-diagnose neurologic diseases that include speech abnormalities. Although a psychogenic diagnosis was an initial consideration in this case, it was the speech evaluation that pointed to an organic problem—an ataxic dysarthria that likely involved the cerebellum. That diagnosis eventually led to confirmation of cerebellar involvement and identification and successful treatment of the primary tumor.
This case is also a powerful example of the importance of cross-disciplinary teamwork in medical diagnosis and management.
Duffy, J. R. (2013). Motor speech disorders: Substrates, differential diagnosis, and management (3rd Edition). St. Louis: Elsevier.
Duffy, J. R. (2013). Motor speech disorders: Substrates, differential diagnosis, and management (3rd Edition). St. Louis: Elsevier.×
Paslawski, T., Duffy, J. R., & Vernino, S. (2005). Speech and language findings associated with paraneoplastic cerebellar degeneration. American Journal of Speech-Language Pathology, 14, 200–207. [Article] [PubMed]
Paslawski, T., Duffy, J. R., & Vernino, S. (2005). Speech and language findings associated with paraneoplastic cerebellar degeneration. American Journal of Speech-Language Pathology, 14, 200–207. [Article] [PubMed]×
Vernino, S. (2012). Paraneoplastic cerebellar degeneration. Handbook of Clinical Neurology, 103, 318–322. doi: 10.4449/aib.v149i3.1232.
Vernino, S. (2012). Paraneoplastic cerebellar degeneration. Handbook of Clinical Neurology, 103, 318–322. doi: 10.4449/aib.v149i3.1232.×
1 Comment
February 13, 2018
Rebecca Kitchens
Wow.
A stirring reminder of the significance of a multi-disciplinary team approach to treatment. Thank you for sharing!
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February 2018
Volume 23, Issue 2