Functional disorders of the nervous system and epilepsy

The treatment of neuropsychiatric disorders such as depression, obsessive–compulsive disorder (OCD) (recurrent compulsive behaviors and intrusive thoughts), and Tourette syndrome (motor and vocal tics) requires a multidisciplinary therapeutic approach consisting of psychotherapy, behavioral therapy, and pharmacological treatment with antidepressants. Affected patients are often severely limited in their daily lives, as depression and compulsions can dominate their behavior.

Tremor, Parkinson’s disease, and dystonia (involuntary, sustained contractions of skeletal muscles) belong to the extrapyramidal motor disorders of the central nervous system.

Tremor (shaking) manifests as regular, involuntary, rhythmic muscle contractions. However, it is not an independent neurosurgical functional disorder, but rather a symptom that can occur in association with other underlying conditions. The diagnosis of tremor is complex and should be made by an interdisciplinary team of neurologists and neurosurgeons.

Five main types of tremor are distinguished: Essential tremor (ET), Parkinsonian tremor (PT), Dystonic tremor (DT), Midbrain tremor, Tremor associated with multiple sclerosis (MST). For therapeutic decision-making, the tremor frequency and the distinction between resting tremor, postural tremor, and action tremor are also important.

Over time, pharmacological therapy for tremor has proven to be only moderately effective, whereas deep brain stimulation (DBS) has become an increasingly effective treatment option.

Many patients suffering from psycho-motor neurochirurgical disorders cannot be adequately helped with conventional pharmacological treatment, physiotherapy, psychotherapy, or even microsurgical interventions. In these cases, an option is electroconvulsive therapy—or more specifically, Deep Brain Stimulation (DBS).

DBS is a reversible, minimally invasive neurosurgical procedure that patients themselves can partially control. It delivers mild electrical stimulation as a treatment alternative for patients who are resistant to classical therapies. DBS is currently the only neurosurgical method that allows for ongoing therapy control.

The DBS system consists of four main components:

• Stereotactic electrodes – thin, flexible titanium wires with metal contacts at the tip. These are implanted in specific brain regions via a burr-hole trepanation to modulate or inhibit abnormal brain activity.
• Extension cable – a thin, insulated wire running under the skin that connects the electrodes in the brain to the pulse generator (neurostimulator).
• Pulse generator (neurostimulator) – allows programming of stimulation parameters. About 5 × 4 cm in size, it is implanted under the skin of the upper chest, generating the electrical impulses required for stimulation and targeting precisely selected brain areas.
• Programmer device – used externally to check and adjust the neurostimulator’s settings and functions.

Trigeminal Neuralgia
Trigeminal neuralgia is a severe facial pain caused by a dysfunction of the fifth cranial nerve (trigeminal nerve). This nerve transmits sensory information from the face to the brain and controls the muscles involved in chewing.

If conservative pharmacological treatment is unsuccessful, several invasive options are available, including: vascular microvascular decompression (Jannetta procedure), where the trigeminal nerve is surgically exposed, non-surgical balloon compression or stereotactic radiosurgery/Gamma Knife.

Migraine
Migraine is characterized by typically unilateral, episodic, pulsating headaches, often accompanied by autonomic symptoms such as nausea, vomiting, and light sensitivity, lasting from several hours up to three days. Usually, migraines are successfully treated with medication. For chronic, therapy-resistant cases, functional neurosurgical treatments such as occipital nerve stimulation may be applied.

Other complex pain syndromes include post-stroke or post-spinal surgery pain, cluster headaches, phantom limb pain, and stump pain.

Hemifacial spasm is a movement disorder of the facial muscles, characterized by repeated, involuntary, unilateral contractions that are often disfiguring and painful. In most cases, the cause is vascular compression at the exit site of the facial nerve (cranial nerve VII).
Mild cases can be treated conservatively with antiepileptic drugs (e.g., gabapentin or carbamazepine). For persistent spasms, Botox injections into the facial muscles can suppress contractions. Severe cases may require neurosurgical interventions, such as microvascular decompression.

Epilepsy, one of the most common chronic disorders of the central nervous system, is characterized by excessive, synchronous electrical activity in the entire brain or in specific brain regions. This leads to temporary disruption of brain function, resulting in epileptic (seizure) events.

Many forms of epilepsy begin in childhood. Possible causes include brain malformations (focal cortical dysplasias), brain tumors, pre- or perinatal brain injuries, meningitis, or severe head trauma, although in some cases the cause remains unknown.

Epilepsies are classified as: focal epilepsies, where the seizure originates from a specific, clearly defined brain region, also called the epileptogenic focus and generalized epilepsies, where the seizure involves both hemispheres simultaneously.

A multidisciplinary team of specialists evaluates each individual case to determine the optimal treatment for each child. About two-thirds of patients can achieve seizure freedom with medication, but in roughly 30%, the epilepsy is therapy-resistant (pharmacoresistant). Depending on the cause, location, and extent of the diseased tissue, different surgical techniques may be used. These include microsurgical procedures as well as minimally invasive methods, such as robot-assisted and MRI-guided laser ablation.

Epilepsy surgery represents a highly effective treatment option and offers an additional chance of achieving seizure freedom. The earlier children are evaluated and, if appropriate, referred for surgery, the better the chances for normal brain development. One of the key prerequisites for a successful operation is the most precise possible localization of the epileptic focus. This is achieved through imaging procedures, seizure monitoring, or occasionally by the direct implantation of electrodes. During a several-hour surgical procedure, the precisely defined diseased brain tissue (the lesion) is removed or disconnected from the brain without damaging adjacent brain areas.

Due to scientific and medical-technological advances in neurosurgery, anesthesia, and intensive care medicine, epilepsy surgery has become very safe and can now be performed even in infants during the first year of life.

In addition to the targeted neurosurgical removal of epileptic foci, other treatment options include the minimally invasive implantation of vagus nerve stimulators (VNS systems) and deep brain stimulation (DBS). Vagus nerve stimulation makes it possible to successfully treat severe forms of epilepsy without direct brain surgery by stimulating the vagus nerve using a special “nerve pacemaker.”

Our goals are:

• Comprehensive information for patients and their families about the disease, diagnosis, treatment, and, if necessary, follow-up care
• Seizure freedom or reduction of seizure frequency
• Reduction of medication use and medication-related side effects
• High quality of life and support for the cognitive, motor, and social development of our patients with epilepsy

Our clinic works closely with the Epilepsy Center of the University Department of Neurology and the Outpatient Clinic for Advanced Epilepsy Diagnostics of the University Department of Pediatrics and Adolescent Medicine.

The diagnosis of hydrocephalus is most commonly made in a pediatric context, but it is also frequently seen in adult patients. It occurs due to excess accumulation of cerebrospinal fluid (CSF) and dilation of the brain’s internal or external CSF spaces, resulting in either a circulatory disturbance or a production/resorption disorder of CSF, with or without increased intracranial pressure. Hydrocephalus can be congenital or acquired.

In adults, secondary (acquired) forms of hydrocephalus are more common, typically resulting from blockages or outflow obstructions. These may occur after hemorrhages, infections, tumors, or neurosurgical procedures.

Malresorptive hydrocephalus arises from impaired CSF absorption in the arachnoid villi of the subarachnoid space. This can occur when blood enters the CSF due to subarachnoid or ventricular hemorrhage, meningitis, or tumor disease.

Occlusive hydrocephalus (hydrocephalus occlusus) is often caused by a tumor obstructing the CSF pathways or by a narrowing of the cerebral aqueduct, the channel connecting the third and fourth ventricles.

Hydrocephalus ex vacuo results from brain atrophy, either as a natural aging process or due to neurodegenerative diseases such as multiple system atrophy, Parkinson’s disease, Alzheimer’s disease, or cerebrovascular disorders. In this case, there is a passive enlargement of CSF volume, but intracranial pressure is not increased.

Normal Pressure Hydrocephalus (NPH) is a special form of hydrocephalus that primarily affects older adults (>65 years). On imaging, it is characterized by enlarged internal CSF spaces with normal or narrow external CSF spaces. The measured intracranial pressure is only temporarily elevated and is otherwise relatively normal, which gives the condition its name.

The classic symptom triad of normal pressure hydrocephalus, which closely resembles Alzheimer’s or Parkinson’s disease, is known as the Hakim triad, named after the Colombian neurosurgeon Salomon Hakim. It includes: cognitive impairment ranging from memory and concentration problems to dementia, gait disturbance, characterized by small, shuffling steps, a magnetic quality, and a broad-based stance (ataxia) and urinary incontinence.

A diagnostically decisive test is the CSF drainage test (spinal tap test), in which 30–50 ml of cerebrospinal fluid is removed once or repeatedly via lumbar puncture. If this leads to improvement in neurological symptoms, surgical treatment is indicated. The therapeutic goal is permanent CSF diversion from the lateral ventricles, typically achieved neurosurgically using a ventriculoperitoneal or ventriculoatrial shunt.

From a dynamic perspective, hydrocephalus is classified as chronic or acute, the latter requiring urgent intervention.