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Basics

• Most common in pre-menopausal obese females
• Most common symptom is headache
• An important preventable cause of blindness
• Normal imaging studies and a lumbar puncture only demonstrates increased pressure
• Most cases are treatable with medication though refractory cases often require an operation

Symptoms and Signs

• Headache
• Decreased visual acuity, double vision, and blindness
• Nausea
• Other symptoms: stiff neck, ringing ears, difficulty walking, eye pain on movement, dizziness
• Worsening of symptoms with bending over or valsalva

Demographics

• Female : Male ratio 2-8:1
• Increased in obesity and women of childbearing age: Peaks in 30s

Diagnosis

• Diagnosis is made on the combination of normal imaging studies (CT and MRI) with increased opening pressure during a lumbar puncture
• Ophthalmologic exam

Treatment

• Weight loss
• Stop offending drugs
• Fluid and salt restriction
• Diuretics- Lasix, Diamox or Neptazane
• Surgery- Optic nerve sheath fenestration or a lumboperitoneal/ventriculoperitoneal shunt

Prognosis

• Spontaneous resolution is common within 1 year
• Permanent visual loss occurs in 2-24%
• Recurs in about 10%

Differential Diagnosis

• Drugs: Keprone, lindane, excess vitamin A, oral contraceptives
• Tumor
• Dural sinus thrombosis
• Chiari malformation
• Infection
• Neurosarcoidosis
• Pseudopapilledema
• Malignant hypertension
• Guillain-Barre syndrome
• head trauma

Basics

• A term normally used to describe a certain type of headache with a suspected vascular cause

Symptoms and Signs

• Initially one sided (unilateral) and throbbing headache that builds up over the course of several hours
• Patients prefer dark, quiet rooms
• Nausea and vomiting
• Normally subsides within 24hours
• Auras- can be visual (shimmering lights), sensory (paresthesias), or motor (heaviness of hte limbs)
• May see neurological deficit

Demographics

• 18% of females and 6% of males are affected
• 75% of individuals with migraines are adult women
• In children: Boys and girls are equally affected
• More common in whites than African/Asian Americans

Diagnosis

• Based on clinical history and exam: Additional studies to rule out other causes-
• CT / MRI
• Lumbar puncture
• EEG

Treatment

• Medication options have two main goals: Abortive therapy (for an ongoing migraine) and Preventive therapy (to prevent the future occurence of migraines)
• Abortive: Ibuprofen, Triptans (Imitrex, Maxalt, Axert), Opioids, DHE IV medication
• Abortive medication overuse (>2 days per week) can lead to analgesic rebound headaches
• Preventive: Methysergide, Calcium channel blockers, Beta blockers, antidepressants, anticonvulsants

Prognosis

• Not all severe headaches are due to a migraine and often they are a warning sign to a more severe condition (such as a ruptured aneurysm)
• In terms of migraines:

Differential Diagnosis

• Arteriovenous malformation
• aneurysm
• Cluster headache
• Tumor
• Infection
• Lupus
• Temporal arteritis
• Tolosa-Hunt syndrome

Basics

• Occurs when the intervertebral disk between 2 vertebrae (bones of the spine) bulges outward thus putting pressure on the spinal cord or nerve roots
• The disk will often cause radiating pain, weakness, and sensory changes.
• A large percentage of protruding disk will resolve spontaneously with the use of anti-inflammatory medications
• A large number (though small percentage overall) of patients will require surgery thus making the lumbar discectomy the most common surgical procedure performed in the US
• Disc herniations can occur in the cervical, thoracic, and lumbar spine (Upper, Middle, and Lower back)

Symptoms and Signs

• Symptoms depend on the location and direction of the herniation
• Back pain that is often aggrevated by weight bearing or movement
• Pain that radiates down the leg/arm (aka radicular pain)
• The pain often worsens with coughing or sneezing
• Weakness or decreased sensation
• May have bladder or other neurological problems

What is sciatica?

• Sciatica is a term often used in conjunction with a herniated disc and refers to the pain produced by compression of the sciatic nerve roots or of the nerve itself.
• Sciatica is a way to describe a particular set of symptoms and does not necessarily mean that the patient has a herniated disc (this is where a lot of the confussion surrounding this term is derived)
• A characteristic pain that radiates down the lower back, into the buttock, and down the leg
• Just a few causes of sciatica include:

1. Herniated disc
2. Spinal stenosis
3. Piriformis syndrome
4. Tumors
5. or even pregnancy

Demographics

• More common in men

Diagnosis

• Clinical exam by a neurologist
• MRI, CT scan
• EMG or nerve conduction studies

Treatment

• Medical management: Medication and physical therapy will lead to pain resolution in a large percentage of patients
• Surgery- most surgeons will wait 5-8 weeks unless the patient has experienced cauda equina syndrome, worsening motor weakness, or intolerable pain
• Multiple surgical options from a standard removal to a minimally invasive operation
• Complications: Occur in about 10% of operations and consist of bleeding, infection, dural tear, nerve root injury, spinal stenosis, etc
• Disc recurrence after a discectomy occurs in about 5-15% of patients
• SPORT Trial- large study in the New England Journal of Medicine that demonstrated patients who chose to have an operation for lumbar disc herniation reported a greater improvement than patients who elected for nonoperative care (but this does not necessarily mean surgery is better than conservative management)

Prognosis

• 85% of acute disc herniations will improve without surgery in an average of 6 weeks

Differential Diagnosis

• Numerous types of spinal pathology
• Tumor
• Infection
• Inflammation
• Spinal stenosis
• Subarachnoid hemorrhage
• Dural AV Fistula

References

• NEJM SPORT Trial
• Handbook of Neurosurgery: Greenberg

Basics

• Characterized by repetitive, involuntary contractions of the face (supplied by the Facial Nerve- Cranial Nerve VII)
• Normally starts with the muscles around the eye and then progresses to the cheek and lips
• Brief twitches but increase in duration as the disease progresses
• Often caused by a abnormal blood vessel compressing the facial nerve inside the skull
• Treatment consist of medication and surgery in refractory cases

Symptoms and Signs

• Involuntary muscle contractions on one side of the face
• Contractions may be initiated by voluntary movement of the muscles of facial expression
• Muscle weakness in the face
• Normally painless

Demographics

• 8 (men) to 15 (women) out of 100,000
• Affects Women > Men, normally in the 50s and 60s
• slightly more common on the left than the right

Diagnosis

• EMG
• MRI / CT
• Clinical exam

Treatment

• Medications: Carbamazepine (Tegretol), baclofen, gabapentin (Neurontin), and Phenytoin (Dilantin)
• Injection of Botox– works for about 5 months and then the injections need to be repeated
• Surgery: Microvascular decompression (aka MVD) with insertion of a pledget (like a piece of felt) between the nerve and the abnormal blood vessel- the definitive treatment in medically refractory cases

Prognosis

• According to several studies, 2/3 of patients will achieve some relief with medication but the traditional thinking still views oral medications as being generally ineffective
• Botox injections- typically provide relief for 5months
• Surgery offers the definitive treatment of hemifacial spasm caused by an aberrant blood vessel: 86% of patients are spasm free at 10 years

Differential Diagnosis

• Basilar artery aneurysm
• Acoustic nerve tumor
• Meningioma
• Multiple sclerosis
• Bell’s palsy- can actually later result in hemifacial spasm

References

• Adam’s and Victor Neurology
• Handbook of Neurosurgery: Greenberg
• Harper, RL et al. “Microvascular decompression for hemifacial spasm: long term results of 114 operations” Journal of Neurosurgery

Basics

• Seizure- abnormal electrical activity in the brain that results in an alteration in sensation, motor function, behavior, or consciousness
• Epilepsy- A seizure disorder characterized by recurrent, unprovoked seizures
• It is possible to have a seizure without having epilepsy
• An exact description of the seizure is important because the different treatment options often depend on the type of seizures that you or family member are experiencing.

Symptoms and Signs

• There are many different types of seizures and the most common is called a generalized tonic-clonic (aka grand mal) seizure
• Seizures can range from a blank stare (Absence seizure) to a rhythmic jerking of the arms and legs (grand mal seizure)

Demographics

• Occur in all age groups but a new, unprovoked seizure in an adult should be worked up for an underlying cause
• Most commonly occur in kids and young adults

Diagnosis

• Based on the clinical description
• Electroencephalogram (EEG) or long term Video-EEG
• MRI, PET scan, SPECT scan, Functional MRI, etc

Treatment

• Anticonvulsants- there are many different medications used to treat seizures/epilepsy and often multiple drugs/combinations are required in order to maintain adequate control
• Surgery- there are many different surgical options for intractable epilepsy (the cases in which medication does not control).

Prognosis

• Dependent on the type and underlying cause of the seizures
• Surgical treatment of poorly controlled epilepsy has improved seizure frequency in a subset of epilepsy patients

Differential Diagnosis

• Metabolic abnormality
• Non-epileptic seizures- a psychiatric condition

Basics

• Defined as a loss in previously attained intellectual abilities that is severe enough to affect everyday life.
• Memory problems are the most common sign
• Affects up to 11% of adults >65%
• Most commonly caused by Alzheimer disease, dementia with Lewy bodies, and vascular dementia

Symptoms and Signs

• Memory loss plus impairment in one of the following areas: language, perception, visuospatial function, calculation, judgement, abstraction or problem solving skills

Demographics

• Increased in people older than 65

Diagnosis

• A clinical diagnosis in most cases
• Blood test to rule out reversible causes of dementia (such as B12 def)
• Brain biopsy- normally reserved for a chronic progressive disorder with an unusual course

Treatment

• Dependent on underlying cause

Prognosis

• Dependent on underlying cause

Differential Diagnosis

• Alzheimers
• Creutzfeldt-Jakob disease, AIDS encephalopathy
• Brain tumor such as a low grade astrocytoma
• Parkinson disease
• B12, niacin, thiamine deficiency
• Depression

Basics

• CMT Disease is part of a larger spectrum of inherited diseases affecting the peripheral nerves
• Most common inherited disorder of peripheral nerves
• Usually inherited in an autosomal dominant fashion (consecutive generations affected) though some cases are X-linked recessive
• Normally presents in childhood/early adulthood with foot deformities or gait disturbances
• Slowly progressive disease that results in weakness and atrophy beginning in the legs.
• Sensory loss is common

Symptoms and Signs

• Onset in late childhood or adolescense though Type 2 may present in middle-aged
• Foot deformities- pes cavus with hammer toes
• Foot drop from lower extremity weakness
• Frequently sprained ankles, difficulty running, slapping of the feet
• Progressive lower and upper extremity weakness
• Progressive sensory loss
• Difficulty walking is the main disability

Demographics

• Type 1- 1st decade of life
• Type 2- 2nd decade of life
• Prevalence of 1 in 2,500

Diagnosis

• Nerve conduction velocity and EMG– helps differentiate between different types of peripheral neuropathy

Treatment

• No known treatment though NSAIDS (Ibuprofen), anticonvulsants and antidepressants are used to help control pain
• Bracing and attempting to minimize the disability are the main focus of treatment options

Prognosis

• Normal life expectancy
• Difficult to predict disability even among siblings

Differential Diagnosis

• HMSN non-type 1,2

Basics

• Most common entrapment neuropathy in the upper extremity
• Usually occurs in middle-aged patients that have performed repetitive hand motions
• Commonly presents as a painful, numb feeling in the hand or hands
• Weakness is common
• Can be managed with medication or surgically

Symptoms and Signs

• Dysethesias- patients are normally awoken with a painful, numb feeling in the hands (feels like your hands are asleep) of a specific distribution
• Weakness of the grip- may see some atrophy
• Clumsiness and changes in handwriting or difficulty buttoning buttons (more a result of numbness)
• Positive Phalen’s and Tinel’s signs

Demographics

• 4:1 Female to Male normally in middle-aged patients

Diagnosis

• EMG and nerve conduction velocity
• If the diagnosis is unclear blood test may be required: Thyroid function testing (to rule out myxedema), CBC and 24hr urine (multiple myeloma), electrolytes and BUN/Cr (uremic neuropathy)
• Carpal tunnel is also common in pregnancy and tuberculosis

Treatment

• Medications: NSAIDS and steroid injections (1/3 will relapse)
• Splints- 50% will improve with splinting alone
• Surgery- the definitive treatment though not all patients are surgical candidates

Prognosis

• 90% of patients treated surgically will have improvement

Differential Diagnosis

• Cervical radiculopathy
• Thoracic outlet syndrome
• Pronator teres syndrome- entrapment of the median nerve in the upper forarm
• de Quervain’s syndrome- also common in pregnancy
• Reflex sympathetic dystrophy
• Tenosynovitis

Basics

• The most common cause of peripheral (the forehead is involved) facial paralysis
• The cause is thought to be secondary to a viral infection usually herpes simplex virus or Lyme disease
• Often treated with steroids. All cases show some recovery and if none is seen by 6 months another cause should be pursued
• 80% recovery fully

Symptoms and Signs

• Normally a viral illness precedes the onset of partial facial paralysis
• Partial paralysis often progresses to complete paralysis within 1 week
• Loss of taste and salivation (due to an affected chorda tympani)
• Increased volume of sound (due to an affected stapedial branch of the facial nerve)
• Decreased tearing of the eyes (due to an affected geniculate ganglion)

Demographics

• Incidence 200/1million people per year

Diagnosis

• Patients should seek prompt medical attention because of available treatment options
• Based mostly on clinical exam demonstrating a facial paralysis with involvement of the forehead
• Facial paralysis is graded using the House & Brackman grading scale
• EMG and nerve conduction may be used

Treatment

• Steroids: Prednisone- reduce both the pain associated with Bell’s palsy and the number of patients with complete denervation.
• Should be instituted promptly
• Eye protection with artificial tears/ointment and avoiding bright light
• Surgical decompression- very controversial and rarely used

Prognosis

• Most cases demonstrate some improvement
• 80% fully recover
• 10% have partial recovery
• 10% have a poor recovery

Differential Diagnosis

• Other types of infection
• Tumor
• Trauma
• Side effects of diabetes, fevers, or hypertension

References

• Handbook of Neurosurgery: Greenberg
• eMedicine

Basics

• Second most common reason for visiting a physician in the US and the number one cause of disability in those less than 45 years-old
• There are many causes of back and neck pain that range from processes affecting the muscles, vertebrae, spinal cord, nerve roots, and other surrounding structures.
• Do to the wide variety of causes related to back pain making generalizations about the underlying cause is difficult unless performed by a neurologist or neurosurgeon on an individual case basis.
• Common causes include: Degenerative changes, trauma, sprains and strains, congenital abnormalities, tumors, autoimmune disorders, infection, inflammation, and referred pain from organ sites.
• Acute low back pain- pain of less than 3 months duration that is aggrevated with motion and relieved by rest: 85% have full recovery

Symptoms and Signs

• The character of the pain and the history of the patient direct the diagnostic work-up and provide an indicator as to the underlying cause.
• The specific symptoms of each type of back pain are covered in other sections.
• Some important findings that warrant further investigation include: Pain worse at rest or at night, prior history of cancer, chronic infections, prior trauma, incontinence, IV drug use, steroid use, rapid worsening of motor or sensory function, fever, weight loss, pain directly over the spine to hard pressure

Demographics

• Affects all age groups
• Varies depending on specific cause

Diagnosis

• X-ray, CT scan, MRI
• Laboratory blood test
• EMG and nerve conduction studies

Treatment

• Dependent on the underlying cause- Ranging from Reassurance to Surgery
• Back pain of less than 3 months duration without any other risk factors is treated with reassurance and pain relief in necessary (NSAIDS)

Prognosis

• Acute low back pain of less than 3 month duration- 85% fully recovery
• Otherwise, the prognosis is dependent on the underlying cause

Differential Diagnosis

• Hundreds of Causes