Your brain is a complex system that controls your body, guides your thinking, and filters emotional response. When something like superficial siderosis damages neural function questions arise on how exactly a person’s thoughts, feelings, and cognitive function will be affected. If you or those close to you notice changes in memory, thinking or behavior it may be time to visit a neuropsychologist.
The MRI scans of some superficial siderosis cases may note the presence of cerebellar atrophy. Cerebellar atrophy is wasting that affects the brain, brainstem, and in some cases the spinal cord. You can blame gravity for how hemosiderin settles around the cerebellum, slowly damaging it, and bringing symptoms many in the superficial siderosis community are familiar with.
Even though your cerebellum is less than 10% your brain volume, it is home to 80% of your brain’s neurons. It’s common knowledge the cerebellum was linked with your motor function, but studies have now mapped how cerebellar damage can also result in a mix of cognitive problems such as executive function, spatial cognition, language, and behavior.
Neuropsychologists are licensed psychologists who concentrate on brain functioning. A neuropsychologist will focus not only on brain-behavior relationships of cognitive function: thinking, memory, language skills, attention but also how brain injuries or damage affect your emotions and behavior.
Your visit will likely start with questionnaires filled out by you and to someone who is familiar with your day-to-day activities. After these are reviewed, you will continue into a clinical interview. Your neuropsychologist will ask a variety of questions designed to help them decide what testing is appropriate for you. If you give your permission, they will also interview someone who can provide an outsiders point of view about your daily life. Often those closest to us are the first to notice when something is not quite right.
Testing may take anywhere from two to six hours with rest breaks between segments. Depending what your individual interview reveals you may be tested on your motor, cognitive, and perceptual skills. Cognitive function is often evaluated by the administration of either the Mini-Mental State Examination (MMSE) or the Saint Louis University Mental Status Examination (SLUMS), manipulative tasks will test motor skills, puzzles or drawing for spatial cognition, problem-solving, speaking, listening, and memory exercises. This testing is different than your MRI in that it helps determine how well your brain is functioning.
If your testing shows deficiencies in motor skill or language, your doctor should be able to give you referrals for speech, rehabilitation services, occupational or physical therapy. Help with adapting your home will make your daily living activities less stressful.
Often short-term memory loss can be traced to age, stress, depression or cerebellar difficulties besides atrophy. Memory loss alone does not necessarily mean you will progress into a more severe stage. Your neuropsychologist will be able to address the stressors and depression that battling a severe condition often bring.
Superficial siderosis patients with cerebellar atrophy may have impaired recall of newly learned information or difficulty with simple executive functions such as making plans or keeping thoughts in proper sequence. The majority of patients exhibiting problems will be diagnosed with mild cognitive impairment. Statistically, only 25% of those diagnosed with Superficial Siderosis will ever progress into dementia.
Dementia is classified as a group of symptoms affecting memory, thinking and social behavior enough to interfere with daily functioning. Behavioral indications include personality changes, depression, anxiety, inappropriate behavior, paranoia, agitation, hallucinations, and decreased verbal fluency. Patients will show difficulty in finding words, reasoning or problem-solving, handling complex tasks, planning, organizing, coordination, motor functions, confusion, and disorientation.∗
No matter if you experience simple memory problems or something more serious it’s important to have a baseline test recorded in your medical record. This will help your physician determine if you’re holding steady or testing needs to be revisited at a future date. Stress and depression are just as dangerous as loss of neural function.
∗I have only read one case study where the patient progressed into dementia. It is critical to note beside superficial siderosis, he had a past medical history of hypertension, cerebellar strokes, left ventricular ejection fraction of 46%, dyslipidemia, prostatic hyperplasia, and gastroesophageal reflux disease. From a non-medical point of view, it stands to reason there may have been other contributing factors besides superficial siderosis.
Xavier Guell, Jeremy D. Schmahmann, John D.E. Gabrieli, Satrajit S. Ghosh. “Functional Gradients of the Cerebellum.” eLife (First published: August 14, 2018) DOI: 10.7554/eLife.36652
Xavier Guell, John Gabrieli, and Jeremy Schmahmann. “Triple Representation of Language, Working Memory, Social and Emotion Processing in the Cerebellum: Convergent Evidence from Task and Seed-Based Resting-State fMRI Analyses in a Single Large Cohort.” NeuroImage (First published online: February 2, 2018 ) DOI: 10.1016/j.neuroimage.2018.01.082
Jeremy D. Schmahmann and Janet C. Sherman. “The Cerebellar Cognitive Affective Syndrome.” Brain: A Journal of Neurology (1998) DOI: 10.1093/brain/121.4.561
Jeremy D. Schmahmann. “Dysmetria of Thought: Clinical Consequences of Cerebellar Dysfunction on Cognition and Affect.” Trends in Cognitive Sciences (1998) DOI: 10.1016/S1364-6613(98)01218-2
