Juvenile myoclonic epilepsy

Starting with a 19 yo patient with first ever generalized tonic clonic seizure in bed we discuss the management of first seizure, why it is necessary to perform an EEG early even if a possible trigger can be found and why idiopathic generalized epilepsy can still manifest first in more adult patients. It is actually quite hard to find good data about adults with generalized epilepsy, but some original research points out that quite a few of them can first manifest in higher ages – up to 75 years in small series.

Treatment is slowly changing, now that Levetiracetam is becoming a first line drug in idiopathic generalized epilepsy and there is also Zonisamide which proved efficacy in small series; otherwise valproate, lamotrigine and topiramate remain.

As for reviews, I found this, yet any good book on epilepsy does the job.


2 scenarios:

  • A stroke/tumor/meningitis patient with new hiccup – is it a sign of impending danger (such as yawning)? yes – ami! otherwise no! It mainly appears with medullary strokes and temporal lesions.
  • A patient referred for permanent hiccup since 2 years, already evaluated by ENT and GI specialists.


  • History (drugs, GI problems, etc.)
  • Physical examination (oral, external ear canal!)
  • EGD
  • Ultrasound abdomen
  • Chest CT including upper abdomen
  • Cervical US (thyroid)
  • Cranial MRI

As for treatment, there are some interesting household remedies (cold water, Valsalva, etc., mainly aiming for a vagal stimulation) and other mechanical ways, such as

  • catheter insertion into the pharynx, larynx, esophagus
  • external ear stimulation
  • bulbar pressure

Next, drugs: MCP/Haloperidol, Baclofen, Gabapentin, Propofol, Ketamine (these are my favorites). Important: try them with high doses (at least the first four) then taper the dose rapidly to the effective least dose.

Finally, for chronic cases: Vagal stimulator.

Here is the best reference (in german): ¬†Singultus. HNO 1999 – note that nothing much happened in singultus in the last years; quite surprising…


Hypertrophic pachymeningitis

An MRI with thick dura and lots of contrast enhancement all around the head Рthis is the opening to a broad differential. Generally speaking, you have to differentiate between low pressure and inflammatory changes Рso a spinal tap helps a lot and should be done soon. If inflammatory you face on of the various secondary causes of hypertrophic pachymeningitis or the idiopathic form (as in our case). There is good german article on the subject from 2006 and not much has been published on the subject apart from case reports.

For locals: in my folder there is a ppt presentation of a case of granulomatous idiopathic hypertrophic pachymeningitis…

Psychosocial history

With 5-7 new patients a day it becomes hard to remember all their stories, if you only recap their biological data. Furthermore, those who only remember the “medically pertinent” mumbo-jumbo tend to deliver worse care than those that capture the psychological and social background of their patients.
We read the article “Complicated lives” in the NEJM this year together in today’s session and concluded that learning, knowing and remembering more about our patients will make us better doctors. In handovers it really makes much sense to add some critical psychosocial details – it leads to better recall.

CSF protein, blood brain barriers and Reiber’s hypotheses

A patient with GBS-like symptoms and elevated protein in the CSF – why is the protein high? What do you need to distinguish between local inflammation, reduced blood-csf-barrier-function and reduced resorption?

We follow Reiber in one of his many articles in CSF books or the lab bible (Lothar Thomas. Labor und Diagnose) and discuss the intricacies of CSF production, dynamics and resorption. To be honest, I still don’t really understand where CSF is generated, but Reiber says it’s not by filtration and gives proof for it. He also reduces most of the elevated protein to reduced CSF flow – panta rhei. It all looks quite convincing, so we test the theory against the problem of elevated protein in the one most important differential for GBS, namely cervical myelopathy due to decompensated degenerative cervical myelopathy with blocked CSF passage (“Stop-Liquor”): do you really understand why the protein is elevated in this case?