What is gerstmann syndrome?

Gerstmann Syndrome (GS) is an uncommon neurological disorder characterized by cognitive alterations that correspond to four symptoms: acalculia, agraphia, right-left disorientation, and finger agnosia. Certain brain injuries, particularly those affecting the angular gyrus (a part of the brain with roles in language processing and reading) and surrounding areas, have been reported to produce similar outcomes. In addition, the possibility of disconnection syndrome, a group of neurological disorders, should be considered in specific cases.^1,3

• Agnosia: incapacity to identify, name, and differentiate between the fingers
• Agraphia: developed disruption in the capacity for writing
• Acalculia: lack of the capacity to employ numerical ideas and execute mathematical tasks
• Right-left disorientation:  difficulty in right-left determination¹¹

 Although this disease has a similar name to the Gerstmann-Sträussler-Scheinker disease, , it must be noted that they are totally different.⁴

Information about case reports

There are rare cases of the syndrome in youngsters, sometimes known as developing Gerstmann's syndrome, but the cause remains unknown. The majority of instances are identified when children reach school age and are required to complete writing and arithmetic activities. Typically, children with Gerstmann syndrome exhibit poor handwriting, spelling skills and difficulty with math functions such as addition, subtraction, multiplication, and division. They may also be unable to discriminate between right and left hands, as well as specific fingers. Many children also suffer from constructional apraxia, which is the inability to reproduce simple designs and reading skills are usually affected. It may impact both youngsters with good intellectual functioning and those who have undergone brain damage.⁴

A unique case

A rare and complex presentation was discovered as an ischemic stroke, which occurs when blood supply to the brain is cut off by a blockage. This led to a hemorrhagic conversion (bleeding in the brain), resulting in Gerstmann syndrome symptoms. Subsequently, it was realised that this individual’s condition was brought on by secondary polycythemia (overproduction of blood cells) after long-term use of anabolic steroids that are generally taken to improve athletic performance.⁵

Treatment

There is no treatment option for Gerstmann Syndrome; thus, the therapeutic approach can only be supportive. Patients experiencing symptoms can choose to participate in clinical research as volunteers to advance research in fast detection and treatment for symptoms.⁴

Causes

There are potential causes for this syndrome, such as ischemic stroke, carotid artery dissection due to aneurysm or stenosis, several cancer types, cerebral aneurysm located in the middle artery, chronic subdural hematoma, multiple sclerosis, and cortical atrophy.⁶

Cerebrovascular disease, which is defined by decreased blood flow to the brain after a stroke or other brain trauma, can cause the syndrome in adults. The parietal lobes, or top side lobes, of the brain are affected by Gerstmann syndrome. The parietal lobes mediate processes related to sensation, perception, and the understanding of sensory information. Rarely, a brain tumor or severe brain injury in the same region of the brain might cause the many symptoms associated with Gerstmann syndrome. The origin of children's Gerstmann syndrome is often unknown, but children without brain damage can also be affected.⁷

The pathophysiology

The pathogenesis (development of a disease) of Gerstmann syndrome is mostly dependent on the involvement of the supramarginal and angular gyri (plural for gyrus) in the dominant parietal lobe, particularly the loss of white fibers in that area.⁸ These nerve fibres are extremely crucial, as they enable different parts of the brain to communicate for various functions, and this includes language and speech for this specific area of the brain.  The pathophysiology of Gerstmann syndrome is similar to that of Parkinson's disease, and the disconnection syndrome is thought to be caused by injuries to association fibers connecting various cortical and subcortical regions.^9,10 Therefore, this suggests that deep brain stimulation, the surgical procedure that improves movement in Parkinson’s, may also play a part in treating Gerstmann syndrome.⁸

Natalizumab use

A research report describes a thirty-year-old woman with Gerstmann's disease. An MRI of the left cerebral hemisphere revealed a large lesion (damage to the relevant tissue). The usual treatment, intravenous corticosteroids,  did not reverse the disease's progression.  Initially,  the signs pointed to a tumour or cerebral lymphoma, but the brain biopsy confirmed inflammatory demyelination, which is when the immune system mistakenly attacks the myelin sheath surrounding the nerve fibres and damages them. Following the identification of tumefactive multiple sclerosis, treatment with natalizumab resulted in a considerable reduction in disease activity.¹² Natalizumab is taken as an infusion or injection by people with multiple sclerosis, and it works by attaching itself to the T cells in your immune system, which prevents them from reaching the brain and attacking the myelin sheath. 

Gerstmann’s syndrome in active relapsing-remitting multiple sclerosis with cognitive deficits

Gerstmann's syndrome has been observed when severely active relapsing multiple sclerosis is paired with significant cognitive deficits.^12,13

A patient with gerstmann syndrome & antidepressants

A patient who was temporarily laid off due to his depression, later came to the hospital presenting with Gerstmann's syndrome. The angiography (used to check blood vessels) showed a dural arteriovenous fistula (DAVF), which is an abnormal connection between the artery and a vein in the dura mater (protective membrane of the brain and spinal cord) that can lead to bleeding in the brain. Treatments for the DAVF included endovascular surgery and coil embolization, which improved his melancholy and Gerstmann's health. The patient's antidepressant dose was lowered during the therapy, and two years later, they resumed work without the need for antidepressants. As the patient fully ceased their use of antidepressants two years after starting treatment, DAVFs are believed to be a contributing factor in depression.¹⁴

Diagnosis process and procedure

Gerstmann's syndrome is associated with lesions or malfunctioning of the left angular gyrus in the parietal lobe.¹⁵ A tuberculous brain abscess in the left parietal lobe afflicted a patientand it was the source of both right-sided apraxia and Gerstmann's syndrome. It is difficult to diagnose Gerstmann's condition since aphasia is usually present.¹⁶ Communication and understanding fluency are usually impaired. The patient was able to explain the typical characteristics of Gerstmann's disease since they did not have aphasia,  resulting in a simple diagnosis.¹⁷

Steroid use correlation with gerstmann syndrome

Chronic usage of steroids has been connected to problems with the neurological system, musculoskeletal system, heart, and reproductive system. Cardiomyopathy (a group of heart conditions that reduce its ability to pump blood around the body) and secondary erythrocytosis (excess of red blood cells being produced) can raise the risk of stroke among users without any other risk factors, despite the fact that there aren't many recorded occurrences of strokes linked to anabolic steroid use. Among those who utilize anabolic steroids are bodybuilders and athletes.  Due to long-term usage of anabolic steroids,  a 49-year-old man had hemorrhagic conversion and hypercoagulability (increased risk for blood clot formation), which in turn caused the onset of Gerstmann syndrome.¹⁸

Transient gerstmann syndrome during DAVF treatment

In an assessment of DAVFs, it was noted that following a blood vessel rupture, one patient out of 157 procedures experienced temporary Gerstmann syndrome. Nonetheless, no further irreversible neurological deficits were seen, suggesting that endovascular (inside the vessel) treatment of DAVFs does not typically result in significant side effects similar to Gerstmann syndrome.¹⁹

Endovascular embolization for mild gerstmann syndrome

In this case, a large middle cerebral artery (MCA) serpentine aneurysm (named due to the shape) was the source of a 44-year-old man's migraines and seizures. The aneurysm was large, thrombosed (clot formed), and resembled a mass-like lesion that kept overflowing into the MCA's l surrounding branches. After completing a balloon occlusion test (BOT) to assess collateral circulation, an endovascular embolization was performed on the MCA artery. An endovascular embolization procedure blocks off blood flow from a specific blood vessel to prevent excessive bleeding or cut off abnormal connections between vessels. After the procedure, the patient experienced mild Gerstmann syndrome, but she was discharged in a cognizant state. On follow-up imaging, there was a reduced mass effect, and the patient's Gerstmann syndrome improved. Endovascular embolization is shown to be an effective therapy for a large serpentine aneurysm after a balloon occlusion test evaluation.²⁰

Management of gerstmann syndrome in a patient with MALT lymphoma after chemotherapy

The week after beginning MALT lymphoma (a type of cancer) therapy, a 60-year-old man was admitted to the hospital with abrupt speech problems, Gerstmann syndrome, and incomplete right hemiparesis (one-sided muscle weakness). An MRI of his brain showed left-sided bleeding, and additional testing revealed two middle cerebral artery (MCA) aneurysms that had grown bigger over the preceding two weeks. Using a blood artery from his head, medical professionals conducted bypass surgery after the aneurysms were removed. The aneurysm walls' histological analysis revealed no evidence of malignant or infected cells, which was most likely the cause of the aneurysms Considering how effective the treatment was, the lymphoma cells might have vanished. Reconstructing the scalp blood artery is one surgical method for treating a ruptured MCA, although this form of aneurysm is extremely uncommon.²¹

Summary

Gerstmann Syndrome (GS) is a rare neurological disorder characterized by four key symptoms: acalculia (inability to perform math), agraphia (difficulty with writing), right-left disorientation, and finger agnosia (inability to identify fingers). It is typically caused by damage to the angular gyrus in the dominant parietal lobe, often due to cerebrovascular events like strokes, brain tumors, or trauma. Though there is no specific treatment for GS, supportive care is provided, and clinical research offers insights for future treatment options. The syndrome can also occur in children, sometimes with no clear brain injury. Certain medical conditions like multiple sclerosis and dural arteriovenous fistulas (DAVFs) can also be linked to GS. Treatment often involves managing the underlying cause, such as using natalizumab for multiple sclerosis or endovascular procedures for DAVFs. Additionally, long-term steroid use, especially anabolic steroids, has been associated with increased stroke risk, potentially leading to GS.