Dissociative Seizures

Introduction

Psychogenic Non-Epileptiform Seizures is a real disease. These events probably represent a subconscious dissociative physical response to distressing internal emotional stimuli. These attacks may look like epileptic seizures but are not truly caused by altered electrical activity in the brain but happen due to a reaction to adverse life experiences, trauma, loss or bereavement. Patients with PNES do not have a focal lesion, but rather have dysfunction that is distributed across a wide array of limbic and cortical substrates modulated by several key endocrine signals. The production of seizure-like symptoms is not under voluntary control, meaning that the person is not faking. Interestingly, about 5-20% of people with PNES also have epilepsy.

Other Terminologies

• Pseudoseizures - Use of this particular terminology is discouraged
• Functional Seizures
• Dissociative Seizures
• Non-Epileptiform Attack Disorder 

No single historical feature or combination of features is diagnostic of PNES. PNES are distinct from Epileptiform seizures as they do not show any abnormal electric discharge from the brain. The definitive test to diagnose PNES is Video EEG. Features that may suggest PNES are:

• Same frequency but variable amplitude throughout the seizure
• Recall of events
• Pelvic thursting
• Forced eye closure
• Episodes >2min

Treatment of PNES

1. Adequate communication and education with the patient/family
2. Continued neurological follow-up to safely withdraw anticonvulsant medications
3. Address comorbid psychiatric diagnoses - CBT/Antidepressants/Antipsychotics (Haloperidol/Olanzapine)

Further Reading
https://www.epilepsy.com/article/2014/3/truth-about-psychogenic-nonepileptic-seizures

Posted by:

              

     Lakshay Chanana

     

     ST4 Trainee

     Royal Infirmary of Edinburgh

     Department of Emergency Medicine

     Edinburgh

     Scotland

     @EMDidactic

Gullian Barre Syndrome

Guillain-Barré syndrome (GBS) is a heterogeneous group of immune-mediated peripheral neuropathies which presents as evolving polyradiculoneuropathy preceded by a triggering event (often an infection). Cross-reactivity between the pathogen and the nerve tissue sets up the autoimmune response. It typically manifests as a symmetric motor paralysis with or without sensory and autonomic disturbances. The progressive phase of this syndrome is followed by a plateau phase of persistent, unchanging symptoms. Improvement begins within days of the plateau and the time to resolution of symptoms varies among patients.

Patients commonly report a respiratory tract infection or gastroenteritis that resolved when the neuropathy began. Campylobacteriosis is the most common precipitant in GBS.  Other antecedent infections include cytomegalovirus, HIV, Epstein-Barr virus, and varicella-zoster virus.

Typical Presentation

• Weakness (Proximal>Distal, Legs>Arms) accompanied by tingling dysesthesias in the extremities 
• Areflexia few days of symptom onset.
• Cranial nerve involvement may affect airway maintenance, facial muscles, eye move- ments, and swallowing. 
• Shoulder, Back, Thigh Pain (severe, occurring with even the slightest of movements)

The patient with Guillain-Barré syndrome typically presents with weakness accompanied by tingling dysesthesias in the extremities.

Types of GBS
GBS has five distinct subtypes which can be distinguished electrodiagnostically and pathologically. 

1. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

• Autoimmune disorder, antibody mediated
• Triggered by antecedent viral or bacterial infection Electrophysiologic findings demonstrate demyelination. Inflammatory demyelination may be accompanied by axonal
• nerve loss.
• Remyelination occurs after the immune reaction stops.

2. Acute motor axonal neuropathy (AMAN)

• Pure motor axonal form of neuropathy 
• 2/3rd of patients are seropositive for campylobacteriosis.
• Electrophysiologic studies are normal in sensory nerves
• Reduced or absent in motor nerves. 
• Recovery is typically more rapid. High proportion of pediatric patients

3. Acute motor sensory axonal neuropathy (AMSAN)

• Wallerian-like degeneration of myelinated motor and sensory fibers 
• Minimal inflammation and demyelination
• Similar to AMAN except AMSAN affects sensory nerves and roots 
• Typically affects adults

4. Miller Fisher syndrome

• Rare disorder
• Rapidly evolving ataxia, areflexia, mild limb weakness, and ophthalmoplegia
• Sensory loss unusual, but proprioception may be impaired. Demyelination and inflammation of cranial nerve III and VI, spinal ganglia, and peripheral nerves
• Reduced or absent sensory nerve action potentials, tibial H reflex is usually absent.
• Resolution occurs in one to three months.

5. Acute panautonomic neuropathy

• Rarest of all the variants
• Sympathetic, parasympathetic nervous systems are involved. Cardiovascular involvement is common (postural hypotension, tachycardia, hypertension, dysrhythmias). 
• Blurry vision, dry eyes, anhidrosis, gastrointestinal pseudo-obstruction, and urinary retention 
• Recovery is gradual and often incomplete. 
• Often combined with sensory features

Diagnosis
GBS is the most common type of rapidly evolving, generalized peripheral nervous system disorder. However, there are several mimics that must be considered.

Diagnostic Criteria for Typical Guillain-Barré Syndrome Features required for diagnosis

• Progressive weakness in both arms and legs Areflexia

Features strongly supporting the diagnosis

• Progression of symptoms over days, up to four weeks
• Relative symmetry of symptoms
• Mild sensory symptoms or signs
• Cranial nerve involvement, especially bilateral weakness of facial muscles
• Recovery beginning two to four weeks after progression ceases 
• Autonomic dysfunction
• Absence of fever at onset
• High concentration of protein in cerebrospinal fluid, with fewer than 10 cells per cubic millimeter 
• Typical electrodiagnostic features

Features excluding diagnosis

• Diagnosis of botulism, myasthenia, poliomyelitis, or toxic neuropathy Abnormal porphyrin metabolism
• Recent diphtheria
• Purely sensory syndrome, without weakness

Differential Diagnosis Guillain-Barré Syndrome

• Basilar artery occlusion (asymmetric limb paresis)
• Botulism (descending paralysis)
• Heavy metal intoxication (confusion, psychosis, organic brain syndrome)
• Hypophosphatemia (irritable, apprehensive, hyperventilation, normal cerebrospinal fluid)
• Metabolic myopathies (cerebral and cerebellar symptoms) 
• Myasthenia gravis (weakness and fatigue that improves with rest) 
• Neoplastic meningitis (asymmetric spastic paralysis)
• Neurotoxic fish poisoning (spontaneous recovery within 24 hours) 
• Paraneoplastic neuropathy (chronic)
• Poliomyelitis (purely motor disorder with meningitis)
• Polymyositis (chronic, affects proximal limb muscles)
• Spinal cord compression (asymmetric)
• Tick paralysis (sensory changes absent, normal cerebrospinal fluid) 
• Transverse myelitis (abrupt bilateral leg weakness, ascending, sensory)
• Vasculitic neuropathies (mononeuropathy)

The diagnosis of GBS is based on typical clinical features, electrodiagnostic examination and examination of the cerebrospinal fluid (CSF) can aid in the diagnosis. Characteristic CSF findings consist of elevated protein without pleocytosis (abnormal number of cells in the CSF). 

Treatment
1. Supportive care (Analgesia, Fluids/Electrolytes, Physical Therapy, Nutrition, DVT Prophylaxis, Ventilation)
2. Specific therapy (IVIg, Plasmapheresis)

If patients advance past the acute phase of illness, most will recover function. However, the neuropathy can advance so rapidly that endotracheal intubation and mechanical ventilation may be necessary within 24 hours of symptom onset. Therefore, all patients who have GBS should be admitted to a hospital for close observation for respiratory compromise, cranial nerve dysfunction, and autonomic instability. 

Indications to Consider Intubation

• Forced vital capacity <20 mL per kg
• Maximal inspiratory pressure <30 cm H2O
• Maximal expiratory pressure <40 cm H2O
• Progression is noted with a reduction of more than 30 per cent in vital capacity, maximal inspiratory pressure, or maximal expiratory pressure

Other predictors of subsequent mechanical ventilation:
(1) time from GBS onset to hospital admission of less than seven days
(2) inability to lift the elbows or head above the bed
(3) inability to stand
(4) ineffective coughing
(5) increased liver enzyme levels

Specific treatment should be initiated soon after diagnosis. High-dose intravenous immunoglobulin (IVIg; 400 mg per kg daily for five days) or plasmapheresis can be initiated. IVIg and plasmapheresis are considered as equally effective therapies. 

Disadvantages of plasmapheresis include its rare complications, such as sepsis, that are believed to be caused by depletion of immunoglobulins. If fresh frozen plasma is used as replacement fluid, there is a risk of acquiring viral infections such as hepatitis and HIV. IVIg treatment has advantages over plasmapheresis because it is easier to administer, has significantly fewer complications, and is more comfortable for the patient. 

Despite its benefits, there are side effects from this IVIg therapy such as congestive heart failure and renal insufficiency. Patients may develop fever, myalgia, headache, nausea, and vomiting, but these “influenza-like” symptoms are self-limiting. A history of previous anaphylaxis to IVIg is a contraindication to repeat treatment. 

Corticosteroids were once believed to be useful in the treatment of GBS because of its immune-mediated inflammatory mechanism. However, a Cochrane Database Review of randomized trials, which included 195 patients treated with corticosteroids compared with controls, showed no difference in the outcome. Corticosteroids no longer have a role in GBS treatment.

Approximately 85 per cent of patients with GBS achieve a full and functional recovery within six to 12 months. 

Take Home:

• Guillain-Barré syndrome (GBS) is a heterogeneous group of immune-mediated peripheral neuropathies which presents as evolving polyradiculoneuropathy. It typically presents as Ascending Symmetrical Weakness (Proximal>Distal, Legs>Arms) accompanied by tingling dysesthesias in the extremities, Areflexia, Cranial nerve lesions and Shoulder, Back, Thigh Pain 
• Patients commonly report a respiratory tract infection or gastroenteritis that resolved when the neuropathy began. Campylobacteriosis is the most common precipitant in GBS.  
• Therefore, all patients who have GBS should be admitted to a hospital for close observation for respiratory compromise, cranial nerve dysfunction, and autonomic instability. 
• Treatment of GBS includes Supportive care (Analgesia, Fluids/Electrolytes, Physical Therapy, Nutrition, DVT Prophylaxis, Ventilation) and Specific therapy (IVIg, Plasmapheresis)

References:

• Lindenbaum Y, Kissel JT, Mendell JR. Treatment approaches for Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. Neuro Clin 2001;19:187-204.
• Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2003;61:736-40.

• Hughes RA, van Der Meche FGA. Corticosteroids for treating Guil- lain-Barré syndrome. Cochrane Database Syst Rev 2003;(4): CD001446. Review.
• Zochodne DW. Autonomic involvement in Guillain-Barré syndrome: a review. Muscle Nerve 1994;17:1145-55
• Newswanger DL, Warren CR. Guillain-Barré syndrome. American family physician. 2004 May 15;69(10).

Posted by:

              

     Lakshay Chanana

     

     ST4 Trainee

     Royal Infirmary of Edinburgh

     Department of Emergency Medicine

     Edinburgh

     Scotland

     @EMDidactic

Diplopia - ED evaluation

Primary eye disorders or systemic diseases presenting as ophthalmologic complaints may present to the Emergency Department especially when symptoms are acute in onset. Diplopia is one such complaint which we might come across in ED. 

Key Questions when evaluating diplopia:
1. Is it Monocular or Binocular Diplopia?

• Monocular Diplopia - Eye Problem (cataract irregularities, lens displacement, or primary problems with the corneal curvature such as keratoconus.)
• Binocular Diplopia (Common) - Likely Neuro Problem (3,4,6 Nerve lesions, Grave's eye Disease, Myasthenia gravis, Orbital Myositis)

2.  Does the degree of diplopia change with direction of gaze and/or head position?  (Determines whether deficit related to cranial nerve innervation)

3. Is the diplopia horizontal or vertical?  

(Horizontal diplopia suggests cranial nerve III or VI deficit (i.e. lateral gaze function); vertical diplopia suggests cranial nerve IV deficit (i.e. elevator/depressor gaze function).

4. Associated pain? (Suggests possible foreign body or extraocular muscle entrapment).

5. Associated trauma? (Blow-out fractures can be associated with diplopia).

6. Associated weakness, headache, confusion, or dizziness?  (Imaging usually indicated to rule out intracranial processes such as stroke or increased intracranial pressure).

3rd Nerve palsy

• Supplies most of the EOM
• Lesion results in Down and out deviation of eyeball, Ptosis due to levator palpebrae paralysis and Ptosis due to damage to parasympathetic pupil-constrictor fibers from the Edinger-Westphal nucleus

Most third nerve palsies are caused by ischemic events due to hypertension or diabetes. 

Pupillary involvement is a crucial diagnostic sign -- compressive lesions tend to involve the pupil, while vascular lesions might actually spare it! This is due to the fact that the parasympathetic nerves course along the surface of the oculomotor nerve making them susceptible to compressive lesions from the outside whereas ischemic lesions occur deeper within the oculomotor nerve and thus spare the superficial parasympathetic fibers.


4th Nerve Palsy

• Difficult to diagnose
• Innervates Superior Oblique muscle
• These patients have an upward deviation of the affected eye with a tilt their head away from the lesion
• Possible Causes include Trauma, ischemia, congenital lesions, malignancy

6th Nerve Palsy

• Supplies the lateral rectus muscle
• Loss of function renders the eye unable to abduct (turn out). 
• Patients go cross-eyed, so to compensate they may turn their head to avoid double vision.
• Susceptible to high intracranial pressures.e.g. pseudotumor cerebri 

Table from Rosen's EM Textbook - Causes of Diplopia

Take Home

• Differentiate betweekn monocular and binocular diplopia is the key
• Think compressive causes e.g. P Comm Aneurysm with a dilated pupil (3N palsy)

Posted by:

              

     Lakshay Chanana

     

     ST4 Trainee

     Royal Infirmary of Edinburgh

     Department of Emergency Medicine

     Edinburgh

     Scotland

     @EMDidactic

Non-specific back pain

Back pain is a common ED presentation. Usually, the non-specific pain is mild to moderate and is aggravated by movement and relieved with rest. There are no risk factors for a serious disease on the history and physical examination. 

Pain lasting >6 weeks is an indicator of more serious disease, since most episodes of nonspecific back pain (80% to 90%) resolve within 6 weeks. 

Red Flags

• Trauma (Fracture)
• Unexplained Weight Loss (Malignancy, Metastasis)
• Neurologic Symptoms (Coed COmpression)
• Age <18 years old and >50 years old (BOny abnormalities, Spinal Stenosis, AAA)
• Fever (Infection - Systemic, Osteomyelitis, Epidural Abscess)
• IVDU (Spinal Infection)
• Steroid Use, Immunocompromised (Infection)
• H/O Cancer (Metastasis)

Injection drug user + Back Pain = Spinal infection (Osteomyelitis, Epidural Abscess)

Elderly + Back Pain - Think Aortic Pathology

Bowel or bladder incontinence + Back Pain = Cauda equina syndrome

Back Pain - Critical documentation components

• Fever
• Localised warmth/tenderness
• Abdominal Masses
• Renal Angle Tenderness
• Sensory-Motor Exam (Perianal/Saddle Anesthesia)
• Reflexes
• Rectal Exam (Tone, Masses) - Must be performed in those with neurologic complaints or findings or other red flags for back pain
• SLRT (screening examination for a herniated disk) - With the patient lying supine, lift each leg separately to approximately 70 degrees in an attempt to produce radicular pain. A positive straight leg raise test causes a radicular pain radiating below the knee of the affected leg. This pain is worsened by ankle dorsiflexion and improved with ankle plantar flexion or decreasing leg elevation. 

Hip Flexion – L2 
Knee Extension – L3
Ankle Dorsiflexion – L4 
Great toe flexor – L5 

Ankle Plantarflexion – S1 

ED Management

• FBC, ESR/CRP, Urine Analysis, ALP, Ca (Only if clinically indicated)
• LS Spine X-Ray (with h/o trauma)
• For most patients, no testing is required
• MRI/CT Myelogram in suspected Epidural Abscess

Patients who resume their normal activities to the furthest extent tolerable recover more rapidly than those bed rest. Management options include:

• Analgesics (Paracetamol + NSAIDs with PPI cover if concerned about GI bleed). 
• Muscle relaxants (Diazepam, 5 to 10 milligrams) 
• Local application of heat 
• Manipulative therapy - Controversial treatment 
• Physical modalities (traction, diathermy, exercise, US treatment, TENS) - Questionable benefit

Take Home

• Watchful waiting (6-8 weeks) ensures appropriate utilisation of resorces and reduces unnecessary radiation exposure. 
• Set expectations and arrange follow up - Patient may expect some sort of imaging. Clarify this and let them know what symptoms concern you.

Further Reading

https://www.rcemlearning.co.uk/foamed/back-pain-advice-know-say/

https://www.rcemlearning.co.uk/references/lower-back-pain/

Posted by:

              

     Lakshay Chanana

     

     ST4 Trainee

     Royal Infirmary of Edinburgh

     Department of Emergency Medicine

     Edinburgh

     Scotland

     @EMDidactic

Headache in children - ED management

Headache is a rare chief complaint in children but more frequently seen in adolescents. ED evaluation should answer the following the questions:

• Primary or Secondary Headache?
• Needs Investigations or Imaging?
• Need for follow up with GP?

Primary headaches (Disabling): Migraine, Tension Headache, Cluster headache (and other trigeminal autonomic cephalalgias); and other primary headaches (such as cough or exertional headache). They are thought to be due to a primary malfunction of neurones. Primary headaches are typically self-limited.

Secondary headaches (a/w Morbidity and Mortality): May show up symptomats of some underlying pathology, e.g. from raised intracranial pressure and space-occupying lesions. Headaches due to a SOL are worse when lying down and morning vomiting is characteristic. There is often a change in mood, personality or educational performance. Causes of secondary headache include brain tumors, vascular malformations, and intracranial abscesses; craniofacial problems, such as sinusitis, dental abscesses, or otitis; systemic disorders, such as lupus cerebritis; and exposure to toxic substances, such as carbon monoxide, lead, or cocaine. Other features suggestive of a space-occupying lesion are i.e Red Flags for Headache:

• Visual field defects – from lesions pressing on the optic pathways, e.g. craniopharyngioma (a pituitary tumour)
• Cranial nerve abnormalities causing diplopia, new-onset squint or facial nerve palsy. Abducens nerve has a long intracranial course and is often affected when there is raised pressure, resulting in a squint with diplopia and inability to abduct the eye beyond the midline. It is a false localising sign. Other nerves are affected depending on the site of lesion, e.g. pontine lesions may affect the VIIth (facial) cranial nerve and cause a facial nerve palsy.
• Abnormal gait (Cerebellar Lesions)
• Torticollis 
• Bradycardia
• Confusion, Seizures
• Growth failure, e.g. craniopharyngioma or hypothalamic lesion
• Papilloedema 
• Cranial bruits – may be heard in arteriovenous malformations 
• Recent change in personality, behaviour or educational performance
• Headache that wakes up a child (different from headache on awakening, not uncommon in migraine)

Headache coinciding with the onset of fever suggests inflammation - sinusitis, pharyngitis, otitis, or meningitis), or may be associated with a more general viral syndrome. The severity of a headache is not helpful to determine the cause. Patients with tension headaches can com- plain of terrible pain, whereas a child with a brain tumor may complain of mild to moderate pain. Nonetheless, complaints of very intense pain should be taken seriously and assessed in context with other historical elements.

The most common causes of headache in chidren are viral and respiratory illnesses and migraneous headaches. A migraine that lasts >72 hours is known as status migrainosus. 

• The mainstay of management is a thorough history and examination. Imaging is unnecessary in the absence of any Red Flag features.
• Among children with intracranial diseases, most are unable to indicate the location of the pain or had an occipital headache. 
• Positional preferences --> space- occupying lesions
• Abdominal pain, nausea, or vomiting--> May be seen in migraine 
• Cluster headaches can be associated with multiple ipsilateral symptoms
• Headache with effortless vomiting but no GI complaints --> suggests elevated ICP
• Family history is commonly seen in migraneous headaches

Examination findings may be normal even if the history suggests a secondary headache. A careful neurologic examination with attention to cranial nerves, gait, strength, and mental status is essential to exclude secondary headache. There is a very low incidence of positive findings on neuroimaging studies in children with headache who have normal findings on physical examination.

Imaging CT or MRI - NCCT is sufficent for most cases that require imaging. CT with contrast is needed when you suspect brain abscess but a normal head CT does not exclude an intracranial mass because the posterior fossa is not well visualized. The posterior fossa is the most common location of brain tumors in children, and MRI of the brain provides better visualization of the posterior fossa. MRA/MRV are useful when vascular malformations or dural sinus thrombosis is suspected. 

Treatment

Do not use narcotics for primary headaches. Long-term use of narcotics can change the pain-modulatory system leading to more intense pain. 

Options: 

• Primary Headaches - Fluid bolus/NSAIDs, Stemetil or Metoclopermide. Metocloperamide and Stemetil can cause extrapyramidal reactions, which are relieved by diphenhydramin, Triptans High Flow Oxygen can also be used to treate Cluster HA.  
• Secondary Headaches need symtom control with Analgesics and further evaluation. 

Take Home:

The mainstay of management is a thorough history and examination. Imaging is unnecessary in the absence of any Red Flag features.

Posted by:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic