Rüzgar Miroğlu

Neurology 2

Thoracic Outlet Syndrome

Thoracic outlet syndrome (TOS) is a syndrome involving compression at the superior thoracic outlet involving compression of a neurovascular bundle passing between the anterior scalene and middle scalene.It can affect the brachial plexus , and/or the subclavian artery or vein.The compression may be positional (caused by movement of the clavicle (collarbone) and shoulder girdle on arm movement) or static (caused by abnormalities or enlargement of the various muscles surrounding the arteries, veins, and brachial plexus).
It is also possible to classify TOS by the location of the obstruction:
* Scalenus anticus syndrome (compression on brachial plexus and/or subclavian artery caused by muscle growth) – diagnosed by using Adson’s sign with patient’s head turned outward.
* Cervical rib syndrome (compression on brachial plexus and/or subclavian artery caused by bone growth) – diagnosed by using Adson’s sign with patient’s head turned inward.
* Costoclavicular syndrome (narrowing between the clavicle and the first rib) – diagnosed with the costoclavicular maneuver.

 

 

Facial nerve paralysis is a common problem that involves the paralysis of any structures innervated by the facial nerve.The most common is Bell’s palsy, an idiopathic disease that may only be diagnosed by exclusion.
Bell’s palsy is the most common cause of acute facial nerve paralysis . Previously considered idiopathic, it has been recently linked to herpes simplex infection. Another more severe form of facial palsy, called Ramsay-Hunt syndrome, is linked to herpes zoster infection of the facial nerve. Other, less common, etiologies are Lyme disease polio, TB.
Bell’s palsy is an exclusion diagnosis. Some factors that tend to rule out Bell’s palsy include:
   1. Recurrent paralysis
   2. Slowly progressive paralysis (The onset of Bell’s palsy is very sudden, usually within 24 hours all the symptoms have been manifested)
   3. Twitching
   4. Associated symptoms (either cochlear or neurologic)
Bell’s palsy is believed in the most recent studies to be due to herpes virus. Other proposed etiologies include vascular problems in the inner ear. Treatment includes steroids and antivirals.
Ramsay Hunt syndrome type II or Herpes zoster oticus is essentially a herpes zoster infection that affects cranial nerves VII (facial nerve) and VIII (vestibulocochlear nerve). Patients present with facial paralysis, ear pain, vesicles, sensorineural hearing loss, and vertigo. Management includes Antiviral drugs and oral steroids.
Physical trauma, especially fractures of the temporal bone, may also cause acute facial nerve paralysis.Transverse fractures in the horizontal plane present the highest likelihood of facial paralysis (40-50%). Patients may also present with hemotympanum,sensory deafness, and vertigo.

 

A vestibular schwannoma(acoustic neuroma),is a benign primary intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve (CN VIII).It arises from Schwann cells, which are responsible for the myelin sheath in the peripheral nervous system.
The earliest symptoms of acoustic neuromas include ipsilateral sensorineural hearing loss/deafness, disturbed sense of balance and altered gait, vertigo with associated nausea and vomiting, and pressure in the ear, all of which can be attributed to the disruption of normal vestibulocochlear nerve function. Additionally more than 80% of patients have reported tinnitus.Large tumors that compress the adjacent brainstem may affect other local cranial nerves. Paradoxically, the 7th cranial nerves are rarely involved pre-operatively; involvement of the trigeminal nerve (CN V) may lead to loss of sensation in the involved side’s face and mouth. The glossopharyngeal and vagus nerves are uncommonly involved, but their involvement may lead to altered gag or swallowing reflexes.
Larger tumors may lead to increased intracranial pressure, with its associated symptoms such as headache, vomiting, and altered consciousness.
Acoustic neuromas may occur sporadically, or in some cases occur as part of von Recklinhausen neurofibromatosis.
Contrast-enhanced CT will detect almost all acoustic neuromas that are greater than 2.0 cm in diameter and project further than 1.5 cm into the cerebellopontine angle. Those tumors that are smaller may be detected by MRI with gadolinium enhancement.Indicated treatments for acoustic neuroma include surgical removal and radiotherapy.

 

Alzheimer’s disease (AD) is the most common form of dementia.Most often, it is diagnosed in people over 65 years of age,although the less-prevalent early-onset Alzheimer’s can occur much earlier. As the disease advances, symptoms include confusion, irritability and aggression, mood swings, language breakdown, long-term memory loss, and the general withdrawal of the sufferer as their senses decline.Gradually, bodily functions are lost, ultimately leading to death.Individual prognosis is difficult to assess, as the duration of the disease varies.Alzheimer’s disease develops for an indeterminate period of time before becoming fully apparent, and it can progress undiagnosed for years. The mean life expectancy following diagnosis is approximately seven years.
The cause and progression of Alzheimer’s disease are not well understood. Research indicates that the disease is associated with plaques and tangles in the brain.Currently used treatments offer a small symptomatic benefit; no treatments to delay or halt the progression of the disease are as yet available.
Because AD cannot be cured and is degenerative, management of patients is essential. The role of the main caregiver is often taken by the spouse or a close relative.Alzheimer’s disease is known for placing a great burden on caregivers; the pressures can be wide-ranging, involving social, psychological, physical, and economic elements of the caregiver’s life.
 

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Hypotonia is a state of low muscle tone, often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but a potential manifestation of many different diseases and disorders that affect motor nerve control by the brain or muscle strength. Recognizing hypotonia, even in early infancy, is usually relatively straightforward, but diagnosing the underlying cause can be difficult and often unsuccessful. The long-term effects of hypotonia on a child’s development and later life depend primarily on the severity of the muscle weakness and the nature of the cause.

 
Some causes of hypotonia
Congenital
Genetic disorders are the most common cause
Down syndrome – most common
Achondroplasia
Marfan’s syndrome
Infantile spinal muscular atrophy such as Werdnig-Hoffman disease
Prader-Willi syndrome
Spinal Muscular Atrophy

Developmental disability
Cerebellar ataxia (congenital)
Sensory integration dysfunction
Developmental dyspraxia
Hypothyroidism (congenital)
Hypotonic cerebral palsy

Acquired
Genetic – Muscular dystrophy (including Myotonic dystrophy) – most common,Rett Syndrome,Spinal Muscular Atrophy
Infections – Encephalitis,Guillain-Barré syndrome,Infant botulism,Poliomyelitis
Autoimmunity disorders – Myasthenia gravis

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Idiopathic intracranial hypertension or benign intracranial hypertension(pseudotumor cerebri),is a neurological disorder that is characterized by increased intracranial pressure in the absence of a tumor or other diseases.
The most common symptom of BIH is headache, which occurs in almost all cases.Many have pulsatile tinnitus, a whooshing sensation in one or both ears (64–87%); this sound is synchronous with the pulseVarious other symptoms, such as numbness of the extremities, generalized weakness, loss of smell, and incoordination, are reported more rarely; none are specific for BIH.In children, numerous nonspecific signs and symptoms may be present.
The increased pressure leads to compression and traction of the cranial nerves, a group of nerves that arise from the brain stem and supply the face and neck. Most commonly, the abducens nerve (sixth nerve) is involved. This nerve supplies the muscle that pulls the eye outward. Those with sixth nerve palsy therefore experience horizontal double vision which is worse when looking towards the affected side. More rarely, the oculomotor nerve and trochlear nerve are affected; both play a role in eye movements.The facial nerve is affected occasionally.
The increased pressure leads to papilledema, which is swelling of the optic disc.This occurs in practically all cases of IIH, but not everyone experiences symptoms from this. Those who do experience symptoms typically report “transient visual obscurations”, episodes of difficulty seeing that occur in both eyes but not necessarily at the same time. Long-term untreated papilledema leads to visual loss, initially in the periphery but progressively towards the center of vision.
BIH is diagnosed with a brain scan and a lumbar puncture; lumbar puncture may also provide temporary and sometimes permanent relief from the symptoms. Some respond to medication (with the drug acetazolamide), but others require surgery to relieve the pressure. The condition may occur in all age groups, but is most common in young women, especially those with obesity.

 

Neurosarcoidosis refers to sarcoidosis, a condition of unknown cause featuring granulomas in various tissues, involving the central nervous system.It can have many manifestations, but abnormalities of the cranial nerves are the most common. It may develop acutely, subacutely, and chronically. Approximately 5-10% of people with sarcoidosis of other organs (e.g. lung) develop central nervous system involvement. Only 1% of people with sarcoidosis will have neurosarcoidosis alone without involvement of any other organs.
Abnormalities of the cranial nerves are present 50-70% of cases.The most common abnormality is involvement of the facial nerve, which may lead to reduced power on one or both sides of the face, followed by reduction in visual perception due to optic nerve involvement. Rarer symptoms are double vision,decreased sensation of the face,hearing loss or vertigo, swallowing problems and weakness of the shoulder muscles or the tongue.Seizures (mostly of the tonic-clonic type) are present in about 15%.Meningitis occurs in 3-26% of cases. Symptoms may include headache and nuchal rigidity.
Other investigations that may be performed in any of the symptoms mentioned above are computed tomography (CT) or magnetic resonance imaging (MRI) of the brain, lumbar puncture, electroencephalography (EEG) and evoked potential (EP) studies. If the diagnosis of sarcoidosis is suspected, typical X-ray or CT appearances of the chest may make the diagnosis more likely; elevations in angiotensin-converting enzyme and calcium in the blood, too, make sarcoidosis more likely.Treatment is with immunosuppression.

 

Transverse myelitis is a neurological disorder caused by an inflammatory process of the spinal cord, and can cause axonal demyelination.This demyelination arises idiopathically following infections or vaccination, or due to multiple sclerosis. One major theory posits that immune-mediated inflammation is present as the result of exposure to a viral antigen.The lesions are inflammatory, and involve the spinal cord typically on both sides. With acute transverse myelitis, the onset is sudden and progresses rapidly in hours and days. 
Symptoms include weakness and numbness of the limbs as well as motor, sensory, and sphincter deficits. Severe back pain may occur in some patients at the onset of the disease. The symptoms and signs depend upon the level of the spinal cord involved and the extent of the involvement of the various long tracts. In some cases, there is almost total paralysis and sensory loss below the level of the lesion. In other cases, such loss is only partial. 
The three main conditions to be considered in the differential diagnosis are: acute spinal cord trauma, acute compressive lesions of the spinal cord such as epidural metastatic tumour, and infarction of the spinal cord, usually due to insufficiency of the anterior spinal artery. Lyme disease serology is indicated in patients with transverse myelitis keeping in mind that dissociation in Lyme antibody titers between the blood and the CSF is possible.

 

Herpes zoster is a viral disease characterized by a painful skin rash with blisters in a limited area on one side of the body, often in a stripe. The initial infection with varicella zoster virus (VZV) causes the acute (short-lived) illness chickenpox  which generally occurs in children and young people. Once an episode of chickenpox has resolved, Varicella zoster virus can become latent in the nerve cell bodies and less frequently in non-neuronal satellite cells of dorsal root, cranial nerve or autonomic ganglion,without causing any symptoms.Years or decades after a chickenpox infection, the virus may break out of nerve cell bodies and travel down nerve axons  to cause viral infection of the skin in the region of the nerve. The virus may spread from one or more ganglia along nerves of an affected segment and infect the corresponding dermatome causing a painful rash.Although the rash usually heals within two to four weeks, some sufferers experience residual nerve pain for months or years, a condition called postherpetic neuralgia.Typically, the neuralgia is confined to a dermatomic area of the skin and follows an outbreak of herpes zoster  in that same dermatomic area.Treatment options for PHN include antidepressants, anticonvulsants (such as gabapentin or pregabalin) and topical agents such as lidocaine patches or capsaicin lotion. Opioid analgesics may also be appropriate in many situations. 
Ramsay Hunt syndrome type 2 also known as herpes zoster oticus is a disorder that is caused by the reactivation of pre-existing herpes zoster virus in a nerve cell bundle in the head (the geniculate ganglion).The neurons in this ganglion are responsible for the movements of facial muscles, the touch sensation of a part of ear and ear canal, the taste function of the frontal two-thirds of the tongue, and the moisturization of the eyes and the mouth.The symptoms and signs include acute facial nerve paralysis, pain in the ear, taste loss in the front two-thirds of the tongue, dry mouth and eyes, and eruption of a erythematous vesicular rash in the ear canal, the tongue, and/or hard palate.

 

Arnold–Chiari malformation is a malformation of the brain. It consists of a downward displacement of the cerebellar tonsils through the foramen magnum,sometimes causing hydrocephalus as a result of obstruction of cerebrospinal fluid outflow.The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in the vasculature of the brain. It can cause headaches, fatigue, muscle weakness in the head and face, difficulty swallowing, dizziness, nausea, impaired coordination, and, in severe cases, paralysis.The conditions sometimes associated with Chiari Malformation include hydrocephalus, syringomyelia, spinal curvature, tethered spinal cord syndrome, and connective tissue disorders such as Ehlers-Danlos syndrome and Marfan Syndrome.
Dandy-Walker syndrome is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it.The key features of this syndrome are an enlargement of the fourth ventricle; a partial or complete absence of the cerebellar vermis, the posterior midline area of cerebellar cortex responsible for coordination of the axial musculature; and cyst formation near the internal base of the skull. Symptoms, which often occur in early infancy, include slower motor development and progressive enlargement of the skull. In older children, symptoms of increased intracranial pressure such as irritability, vomiting and convulsions and signs of cerebellar dysfunction such as unsteadiness and lack of muscle coordination or jerky movements of the eyes may occur.

 

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